Only $2.99/month

Terms in this set (255)

Clinical criteria is also known as the Modified Duke criteria and is widely utilized to establish the diagnosis of endocarditis. The criteria are classified as either Major criteria (two positive blood cultures for a microorganism that is typical to cause endocarditis; evident of endocardial involvement via an echocardiogram ((vegetation, abscess)); development of a new regurgitant murmur. Minor criteria include: vascular phenomena (skin hemorrhages, emboli, aneurysms, or pulmonary infarction), fever >100.4 degrees Fahrenheit (38 degrees Celsius), immunologic phenomenon (glomerulonephritis, Osler's nodes, Roth spots, rheumatoid factor), and positive blood cultures that do not meet the specifics of the major criteria.

The correct answer is 2 positive blood cultures with Staphylococcus aureus and development of a new regurgitant murmur. Since two Major criteria are identified, a definitive diagnosis of infective endocarditis can be made with 80% accuracy. The presence of one major criterion and three minor criteria or even if there are five minor criteria listed can also qualify in this 80% accuracy diagnosis range.

The diagnosis is possible but not highly likely to be infective endocarditis if the patient displays the following: one major and one minor criterion or three minor criterions are met. Any less than these should lead a healthcare provider to suspect a different diagnosis.

Choice A only has one minor criterion; Choice B has one major and only two minor criteria; Choice C has only one major and one minor criterion and finally Choice D only has two minor criteria.
The patient should avoid using oral contraceptives.

This patient's presentation and diagnostic testing most likely reveals Eisenmenger's syndrome with a ventricular septal defect. Eisenmenger syndrome is a general term applied to pulmonary hypertension and shunt reversal in the presence of a congenital defect, including VSD, ostium primum ASD, AV canal defect, aortopulmonary window, or PDA.

In the Eisenmenger VSD patient, chronic hypoxemia in cyanotic CHD results in secondary erythrocytosis due to increased erythropoietin production. Normal white cell counts with normal to decreased platelet counts are typical. Patients with decompensated erythrocytosis fail to establish equilibrium with unstable, rising hematocrits, and recurrent hyperviscosity symptoms. Iron-deficiency occurs from erythrocytosis and may be exacerbated by phlebotomy. Progressive symptoms after recurrent phlebotomy in decompensated patients with Eisenmenger's syndrome are usually due to iron depletion with hypochromic microcytosis. Iron depletion results in a larger number of smaller (microcytic) hypochromic red cells that are less capable of carrying oxygen and less deformable in the microcirculation; as such, iron-depleted erythrocytosis results in increasing symptoms due to decreased oxygen delivery to the tissues. Thus, this patient will benefit from increased iron supplementation.

Higher RBCs counts relative to plasma volume will increase viscosity versus an equivalent hematocrit with fewer, larger, iron-replete, deformable cells. Owing to the risk of clot formation and vascular thrombosis due to hyperviscosity, oral contraceptives are often contraindicated for cyanotic women.
Blowing apical diastolic murmur
This patient's EKG finding suggests a primary atrioventrucular heart block. First-degree AV block is the most common conduction disturbance and is characterized by a PR interval that is prolonged for greater than 0.2 seconds. In general, the PR interval is constant, and each atrial impulse is conducted to the ventricles; a regular cardiac rhythm is expected.
First-degree AV block can be a normal variant in young or athletic individuals due to excessive vagal tone. It also occurs in elderly patients without underlying heart disease. It is also associated with myocarditis, mild digoxin toxicity, and inferior wall myocardial infarction secondary to AV nodal ischemia. The intensity of the first heart sound (S1) is decreased in patients with first-degree AV block. Patients with first-degree AV block may have a short, soft, blowing, diastolic murmur heard at the cardiac apex. This diastolic murmur is not caused by diastolic mitral regurgitation, because it reaches its peak before the onset of regurgitation. The diastolic murmur is thought to be related to antegrade flow through closing mitral valve leaflets that are stiffer than normal.
A harsh, medium-pitched systolic murmur at the 3rd and 4th interspaces that is reduced with squatting and increased with strain from Valsalva or standing is consistent with a diagnosis of hypertrophic cardiomyopathy. Delayed carotid upstrokes characterized by a small and slow rise of amplitude suggests underlying aortic stenosis.
Correct answer:
Ventricular tachycardia

Explanation The observation of a wide-complex tachyarrhythmia at a rate of 160 beats per minute associated with P waves at a rate of 75 beats per minute represents a total dissociation (AV Dissociation) between the atria (upper portions of the heart) and the ventricles (lower portions of the heart). A prudent provider will recognize that, in the presence of a rapid, wide complex tachyarrhythmia, AV Dissociation is the classic criterion of ventricular tachycardia (V.T.).
Having diagnosed ventricular tachycardia, you will endeavor to obtain a more complete history and reasonably complete medical physical examination. It is essential that you provide timely placement of this patient in a medical intensive care unit or a coronary care unit with placement on the critically ill list and advise out-of-town family to travel to visit him. Should the patient survive, then his healthcare providers will need to document his verbalized understanding of their medical professional advice in his chronological record of medical care, which includes the recommenadation that he stop smoking cigarettes immediately and abstains from smoking cigars and pipes as well as from chewing tobacco and dipping snuff.

Paroxysmal supraventricular tachycardia (PSVT) is a narrow-complex tachyarrhythmia. Sinus tachycardia is also a narrow-complex tachyarrhythmia. Ventricular fibrillation (V.F.) is a very chaotic tachyarrhythmia with no readily discernible P waves and no readily discernible QRS complexes. Asystole represents a total absence of cardiac contractions and is seen as a flat line on the monitor. Atrial fibrillation is both irregular and narrow-complex. These answers, therefore, are incorrect.
Polyarteritis nodosa is a rare autoimmune disease that can affect medium-sized arteries in any organ of the body. The cause is unknown. It commonly affects nerves, skin, intestines, muscles, and joints. It is seen more in middle-aged people and results in decreased blood supply to affected body parts. Men and women are affected approximately equally. There has been some association made with hepatitis B. 3 criteria need to be met out a list of possible symptoms before the diagnosis of polyarteritis nodosa can be made. These include: muscle pain, a positive hepatitis B surface antigen or antibody test, elevated BUN or creatinine, livedo reticularis (a mottled purplish skin discoloration over the extremities or torso), an elevated diastolic blood pressure (greater than 90 mm Hg), weight loss of 4kg or more, a biopsy positive for vasculitis, dilated arteries or constricted blood vessels (seen by arteriogram), testicular pain or tenderness, or nerve disease.

It is diagnosed by examining the tissues. This is usually achieved through a biopsy. Histological analysis usually shows focal necrotizing arteritis and a mixed cellular infiltrate within the walls of the affected blood vessels. Testing for an elevated sedimentation rate and C-reactive protein will also help with the diagnosis. Patients often have an elevated white blood cell count and a decreased red blood cell count. Urinalysis may show proteinuria and hematuria.

Polyarteritis nodosa is usually treated with high doses of cortisone and immunosuppressive drugs such as cyclophosphamide or azathioprine. If the patient also has hepatitis B, antiviral medications such as interferon-alpha may be included in treatment.
Acute mesenteric ischemia due to superior mesenteric artery thromboemboli presents as sudden onset of severe periumbilical pain, disproportionate to findings on abdominal examination. Superior mesenteric artery thrombosis usually occurs at anatomic points of narrowing. Thrombi are typically observed within 2cm of the take-off of the superior mesenteric artery and may be associated with atherosclerotic disease.
Superior mesenteric artery embolism usually has a cardiac source. Typically, there is also clinical evidence of other embolic events, such as to the kidneys, and the extremities. Non-cardiac sources include arterio-arterial emboli from atherosclerosis, and aortic aneurysms and iatrogenic from intra-arterial procedures.

CT scan is non-specific for acute mesenteric ischemia, however, it may demonstrate intestinal wall thickening, intestinal distension, intraabdominal fluid, pneumatosis, portal venous air, or intraperitoneal free air. If intravenous contrast is utilized, arterial occlusion may be identified. Arteriography is the gold standard to diagnose acute mesenteric ischemia due to a superior mesenteric artery embolism. Also, if surgical treatment is required, knowledge of the extent and nature of the arterial occlusion may aid in performing a mesenteric bypass.

Patients with a superior mesenteric artery embolism have successfully been treated with thrombolytics; however, this is usually combined with stenting, when bowel infarction is not suspected. This method has also been combined with laparoscopy to assess bowel viability. In this particular patient, bowel infarction is probable, and requires emergent surgical exploration. An embolectomy is performed via an arteriotomy in the superior mesenteric artery, allowing thrombectomy of the proximal and distal superior mesenteric artery. The thrombosis should also be milked out by sequentially compressing the artery up to the arteriotomy. If revascularization is necessary because of poor inflow from the proximal superior mesenteric artery, then prosthetic graft or saphenous vein, in the case of infarcted bowel, should be used for bypass.
This patient's diagnosis is mitral valve prolapse (MVP). It most commonly occurs in young women and in patients with heritable disorders of connective tissue, including Marfan's syndrome, osteogenesis imperfecta, and Ehlers-Danlos syndrome. Rarely, it occurs as a sequel to acute rheumatic fever, in ischemic heart disease, in various cardiomyopathies, as well as in 20% of patients with ostium secundum atrial septal defect.

MVP varies in its clinical expression, ranging from only a systolic click and murmur with mild prolapse of the posterior leaflet to severe mitral regurgitation due to chordal rupture and leaflet flail; in North America, MVP is now the most common cause of isolated severe MR requiring surgical treatment.

The most important finding is the mid- or late- (non-ejection) systolic click following S1, and it is thought to be generated by the sudden tensing of slack, elongated chordae tendineae or by the prolapsing mitral leaflet when it reaches its maximum excursion. Systolic clicks may be multiple, and representing mitral valve regurgitation, they may be followed by a high-pitched, late systolic crescendo-decrescendo murmur, occasionally 'whooping' or 'honking' and heard best at the apex. The click and murmur occur earlier with standing, during the strain phase of the Valsalva maneuver, and with any intervention that decreases LV volume, exaggerating the propensity of mitral leaflet prolapse. Conversely, squatting and isometric exercises, which increase LV volume, diminish MVP; the click-murmur complex is delayed, moves away from S1, and may even disappear.

Patients with MVP most frequently have symptoms of autonomic dysfunction, including easy fatigability, dizziness, and atypical chest pain. Further symptoms include palpitations, light-headedness, and syncope.
Correct answer:
Acute coccidioidomycosis

Explanation
Acute coccidioidomycosis is a disease caused by breathing in a fungus found in the soil in certain parts of the southwestern United States, Mexico, and Central and South America. Dark-skinned people and people with a weak immune system will have more serious infections. Infection is caused by breathing in spores of a fungus (Coccidioides immitis) found in desert regions. About 60% of infections cause no symptoms and are only recognized by a positive coccidioidin skin test. Symptoms include cough, chest pain (mild pain to severe constriction), fever, chills, night sweats, headache, muscle aches and stiffness, joint stiffness, and rash on the lower legs (erythema nodosum).

Acute pulmonary eosinophilia is a self-limiting inflammation of the lungs associated with infiltration of eosinophils in the lungs and blood. The etiology includes exposure to various drugs, parasitic infestation (especially ascariasis in children), nickel exposure, recent blood transfusion, or lymphangiogram. Symptoms may include general malaise, loss of appetite, fever (greater than 2 to 3 days), productive cough (mucoid sputum), chest pain, shortness of breath, wheezing, rapid respiratory rate, headache, and muscle pain.

Histoplasmosis is a chronic respiratory infection caused by inhaling the spores of the fungus Histoplasma capsulatum, which is found in bird and bat droppings common along river valleys. Most cases are mild or asymptomatic. Risk factors include travel or residence in central/eastern United States or South America, environmental or occupational exposure to droppings of chickens, bats, or blackbirds, and pre-existing COPD (chronic obstructive pulmonary disease). Immunocompromised people are also at risk. Symptoms include fever, chills, cough (with mucus or pus), skin lesions, and joint stiffness. The associated skin lesion usually presents as a lesion on the mouth or inner cheek as a papule; it may ulcerate.

Pulmonary actinomycosis is an infection caused by Actinomyces israelii or actinomycete bacteria that causes disease of the chest, mouth, jaw, and pelvis. The bacterium is found in the normal flora of the mouth and gastrointestinal tract of humans. Symptoms include lethargy, weight loss, fever, productive cough, draining sinuses, night sweats, shortness of breath, and chest pain. In the chest, it results in cavities in the lung and pleural effusion, which may spread through the chest wall and produce sinuses.

Pulmonary asbestosis is a respiratory disease caused by inhaling asbestos fibers, which results in pulmonary fibrosis. Asbestos-related disease includes pleural plaques (calcification), malignant (cancerous) tumors called mesotheliomas, and pleural effusion. Cigarette smoking increases the risk of developing the disease. Symptoms include shortness of breath on exertion, cough, tightness in the chest, chest pain, nail abnormalities, or clubbing of fingers.
Correct answer:
Abdominal sonogram

Explanation
The correct response is abdominal sonogram. Cancer, tuberculosis, and uremia are causes of exudative effusions; since his is a transudate, they are unlikely causes of this patient's effusion. Cirrhosis typically causes bilateral effusions. Urinothorax, on the other hand, causes a unilateral effusion and is associated with obstructive uropathy. In addition to the transudative picture, a fluid to serum creatinine ratio of 1 also supports the diagnosis. An abdominal sonogram will help in identifying this as the cause.

Pleural effusion occurs because of accumulation of excess amount of fluid in the pleural space. It could be transudative or exudative. Transudates have fluid protein less than 50% of the serum protein, and LDH less than 60% of serum LDH. Exudates, on the other hand, have protein more than 3g/dL and LDH greater than 200, or 50 and 60% of the serum respectively. Causes of transudative effusions include congestive heart failure, cirrhosis, nephritic syndrome, superior vena cava obstruction, pulmonary embolism, and urinothorax. Causes of exudative include cancer, tuberculosis, esophageal rupture, pulmonary embolism, connective tissue disorders, pancreatitis, and pneumonias.

Pleural fluid culture and biopsy are unnecessary in transudative effusion. Chest CT will show the effusion and underlying causes such as cancer and pneumonia, which is not the case here. Therefore, it will not provide additional diagnostic benefit.

Sputum AFB (acid-fast bacilli) will identify tuberculosis if the patient has active pulmonary TB. However, there is no reason to suspect tuberculosis based on this patient's symptoms.
Correct answer:
Bronchiectasis

Explanation
The clinical picture along with clubbing and the honeycomb appearance on chest X-ray is diagnostic of bronchiectasis.

Bronchiectasis is a disease caused by irreversible dilatation of the bronchial tree. Pathogenesis can be obstruction, congenital disorder, and infection. It can develop as a result of severe bacterial infections in childhood, often as a complication of whooping cough or measles.

It can be cylindrical, varicose, or cystic, and it corresponds to the severity of degree of bronchiectasis. Typically, the patient has recurrent respiratory infections, usually cough with lots of sputum production. There will be clubbing of fingers and cyanosis (depending on the severity), and it presents at a later age compared to cystic fibrosis. Chest X-ray shows a typical honeycomb appearance. Diagnosis can be confirmed by CT scan or bronchography.

Treatment is by antibiotics and assisting postural drainage.

Cystic fibrosis presents with associated symptoms of malabsorption and infertility.

COPD is associated with a history of smoking. It will usually have a normal X-ray, as in bronchitis, or be seen with associated features of emphysema (e.g., bullae and hyper-translucent lung field with loss of peripheral vascular markings).

Pneumonia will have an acute history, and recurring infections are not characteristic. X-ray will be diagnostic. Tuberculosis will also be diagnosed on the X-ray. Bronchiectasis may develop secondary to a tuberculous hilar lymph node obstructing a major bronchus.
Correct answer:
Tracheomalacia

Explanation
Tracheomalacia is a common cause of persistent wheezing in early infancy, with male preponderance in the ratio of 2:1. In primary tracheomalacia, there is insufficient cartilage to maintain the patency of the airway throughout the respiratory cycle. It commonly occurs in premature infants. Secondary tracheomalacia occurs when trachea is compressed by structures like vascular rings or deficient cartilage due to tracheoesophageal fistula.

The dominant finding is a monophonic, low-pitched wheeze most prominent over central airways. There is persistent wheeze even in the absence of any viral respiratory infection, and it is loudest over the trachea. Subcostal retractions are absent unless there is asthma or some other cause of small airway obstructions. Wheeze does not respond to bronchodilators. Diagnosis is confirmed by flexible or rigid bronchoscopy.

Bronchiolitis is usually caused by viral infection. More than 50% of cases are caused by respiratory syncytial virus (RSV). Other viruses include parainfluenza virus, adenovirus, and mycoplasma. It commonly occurs in male infants who have not been breast-fed. The infant first develops a mild upper respiratory tract infection with sneezing and rhinorrhea, which is accompanied by fever up to 101 - 102 degrees Fahrenheit. Respiratory distress, cough, and wheezing appear gradually; symptoms may interfere with feeding. Apnea is more common in infants less than 2 months of age. The infant is tachypneic with nasal flaring and chest retractions. Auscultation may reveal fine crackles and wheeze. A trial with bronchodilators, albuterol, or epinephrine may be considered and continued only if there is a clinical improvement with epinephrine used in an inpatient setting.

Congenital subglottic stenosis is the 3rd most common congenital anomaly of the larynx causing stridor, which is usually biphasic or primarily inspiratory. Recurrent or persistent croup occurs in these infants. The 1st symptom often occurs during an episode of respiratory infection, as edema and thick secretions cause narrowing of the already compromised airway.

Esophageal atresia is a frequent congenital anomaly affecting 1/4000 neonates. Of these, over 90% have associated tracheoesophageal fistula. It typically manifests with frothing and bubbling at the mouth and nose and episodes of coughing, respiratory distress, choking, and cyanosis. These symptoms are exacerbated by feeding. Regurgitation of gastric contents through the distal fistula causes more damaging pneumonia than aspiration of pharyngeal secretions from the blind upper pouch of esophagus.

Vocal cord paralysis is the 2nd most common cause of neonatal stridor. It is often associated with central nervous system lesions like meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. Bilateral vocal cord paralysis typically produces airway obstruction manifested by high-pitched inspiratory stridor. Unilateral paralysis causes aspiration, coughing, and choking. Cry is weak. Other symptoms of airway obstruction are less common. Diagnosis of vocal cord paralysis is made by awake flexible laryngoscopy with ultrasonography, which is a useful adjunctive examination.
Correct answer:
Begin positive pressure ventilation

Explanation
The initial steps like providing warmth, positioning, clearing the airway, drying, removing wet linen, and evaluating the infant takes about 30 seconds. If the infant is still apneic and the heart rate is less than 100/minute, the infant's breathing should be assisted by providing positive pressure ventilation with a bag and mask.

When a newborn or fetus is deprived of oxygen, there is an initial period of rapid breathing followed by a period of primary apnea during which stimulation like drying or flicking the sole will cause resumption of breathing. If oxygen deprivation continues, the infant will have gasping irregular breathing efforts and will then go into secondary apnea. At this point, stimulation will not restart the infant's breathing; therefore, positive pressure ventilation must be provided for the respiration to start.

This infant will require positive pressure ventilation because he is in secondary apnea. He will not respond to stimulation by flicking the soles or free flow of oxygen.

After providing PPV for 30 seconds, evaluate the heart rate. If it is below 60 beats per minute, you should support circulation by providing chest compressions (CC). Since the heart rate is 80/min, chest compressions are not required at present.

Epinephrine is administered if the heart rate remains below 60/min after giving PPV with bag and mask/endotracheal tube and bag for 30 seconds, followed by PPV and CC for another 30 seconds. At present, it is not required in the above infant; however, effectiveness of PPV and chest compressions should be checked frequently.

Ventilation of the lungs is the single most important and most effective step in the cardiopulmonary resuscitation of the asphyxiated newborn. Once this is accomplished, heart rate, blood pressure, and pulmonary blood flow improve spontaneously. However if oxygen levels have become very low, cardiac output may have to be supported by chest compressions and by administering epinephrine.
Correct answer:
Pleural effusion

Explanation
The correct answer is pleural effusion. This patient has a history of congestive heart failure, which is one of the most common causes of transudative pleural effusions. Pleural effusions in the setting of heart failure are usually right-sided or bilateral; in this patient's case, physical exam findings point to bilateral pleural effusion. Dyspnea is a common symptom seen in patients with a pleural effusion. Based on his pitting edema on exam, it is clear that this patient has uncontrolled heart failure. The physical exam findings are also common with pleural effusion.

Lung malignancy is incorrect because he does not have a history of tobacco use, and because of his career as a post office worker, he has a low probability of being exposed to asbestos.

Tuberculosis is incorrect because pleural effusions caused by tuberculosis are typically unilateral. Typically, a patient with TB would have a productive cough, night sweats, and weight loss as presenting symptoms.

Empyema is incorrect because a patient would more than likely present with fever, productive cough, and pleuritic chest pain; however, a patient with empyema would have diminished breath sounds and decreased tactile fremitus. Empyema is more likely to cause a massive pleural effusion with mediastinal shift.

Spontaneous pneumothorax is incorrect because it is unilateral; also, the patient complains of dyspnea and chest pain localized to the side of the pneumothorax. It is also acute onset. Physical examination shows hyperresonant percussion and decreased tactile fremitus that is localized to the side of the pneumothorax.
Correct answer:
Cranial resection followed by lung resection

Explanation
Occasionally, patients present with resectable localized lung cancer and evidence of solitary metastasis. These patients should be considered for a combined approach in which the primary tumor and solitary metastasis are resected. Locations that are amenable to a combined approach include adrenal glands, brain, lung, and bone. Approximately 11% of asymptomatic and 33% of symptomatic patients with bronchoalveolar lung cancer have brain metastasis. The overall histologic incidence in order of decreasing frequency is adenocarcinoma, small cell carcinoma, squamous cell carcinoma, and large cell carcinoma. Cranial resection is done first and thoracotomy thereafter, unless symptoms from the primary tumor site dominate the clinical picture. The overall survival with this combined approach is 37.5 at 24 months with complete resections.

PRIMARY TUMOR (T)

TIS: carcinoma in situ

T1: Tumor <3 cm in greatest dimensions, surrounded by visceral pleura, without bronchoscopic evidence of invasion more proximal than the lobar bronchus

T2: Tumor with any of the following features of size or extent: >3 cm, involves main bronchus >2 cm distal to the carina, invades the visceral pleura, associated with atelectasis or obstructive pneumonitis that extends to the hilar region but does not involve the entire lung

T3: Tumor of any size that directly invades any of the following: chest wall, tumor in the main bronchus <2 cm distal to the carina but without involvement of the carina, or associated atelectasis or obstructive pneumonitis of the entire lung

T4: Tumor of any size that invades any of the following: Mediastinum, heart, great vessels, trachea, esophagus, vertebral body, carina; or tumor with a malignant pleural effusion or pericardial effusion, or tumor with satellite tumor nodules within the ipsilateral primary-tumor lobe of the lung

REGIONAL LYMPH NODES

N0: No regional lymph node metastasis

N1: Metastasis to ipsilateral peribronchial or ipsilateral hilar lymph nodes and intrapulmonary lymph nodes involved by direct extension of the primary tumor

N2: Metastasis to ipsilateral mediastinal or subcarinal lymph node (s)

N3: Metastasis to contralateral mediastinal, contralateral hilar, ipsilateral or contralateral scalene or supraclavicular lymph node (s)

DISTANT METASTASIS

M0: No distant metastasis detected

M!: Distant metastasis present including a different lobe from primary-tumor lobe

STAGES

Stage IA T1N0MO
IB T2NOMO
Stage IIA T1N1MO
IIB T2N1MO
T3N0M0
Stage IIIA T3N1MO
T3N2MO
T2N2MO
T1N2M0
Stage IIIB T4NOMO
T4N3MO
Stage IV T1-4N1-3M1
Correct answer:
Amoxicillin and clavulanic acid

Explanation
The correct response is amoxicillin and clavulanic acid.

The history, along with the X-ray findings, suggests that the patient is suffering from lobar pneumonia.

In addition to S. pneumoniae and H. influenzae, the Gram-negative coccus Moraxella (Branhamella) catarrhalis is a common cause of exacerbation of chronic bronchitis and pneumonia in patients with moderately severe COPD. Lower respiratory tract infections due to M. catarrhalis have been shown to be associated with smoking. The symptoms are typical with chills, pain, and malaise, but sometimes low-grade fever and lack of leukocytosis can be seen. The X-ray appearance can be variable, and sometimes the clinical parameters do not determine the organism causing the illness in a heavy smoker with COPD.

The Gram stain in this case shows the abundant presence of Gram-negative cocci in pairs, which is typical of M. catarrhalis. M. catarrhalis is a Gram-negative, oxidase-positive diplococci, which is also called Branhamella catarrhalis. It causes bronchitis or pneumonia in children or adults with underlying lung pathology. It also occasionally causes bacteremia and meningitis, especially in immunocompromised individuals. As most strains produce beta-lactamase, penicillin, ampicillin, or amoxicillin will only be effective against the strains not producing beta-lactamase.

However, since resistance to both ciprofloxacin and tetracycline has been reported, this pneumonia will require treatment with the most effective agent.

Amoxicillin-clavulanic acid, 2nd and 3rd-generation oral cephalosporins, trimethoprim, and sulfamethoxazole are all effective. Therefore, the appropriate choice is the combination of amoxicillin and clavulanic acid, which will suppress the M. catarrhalis lactamase.
Correct answer:
High-resolution computed tomography (CT)

Explanation
This patient has a likely diagnosis of idiopathic pulmonary fibrosis (IPF). This is a relatively rare disease and can be difficult to diagnose. Patients typically have symptoms for 1 - 2 years prior to definitive diagnosis. IPF is a chronic, progressive restrictive pulmonary disease of the lung parenchyma. Unlike pneumoconiosis, there are no known occupational exposures (hence the term idiopathic). IPF presents with exertional dyspnea and a non-productive cough. A high-resolution computed tomography (CT) and/or a lung biopsy are the means for definitive diagnosis. The CT may show reticular opacities, honeycombing, and perhaps a 'ground glass' appearance in the lung tissue. There are heterogeneous areas of diseased lung interspersed with healthy tissue.

An echocardiogram is not helpful in diagnosing IPF. It can be assumed that evaluation by a cardiologist would have determined if an echocardiogram was necessary. This patient was cleared by a cardiologist and has only pulmonary complaints (dyspnea and cough, with findings of lung crackles and clubbing). The CT should be done next, and if not consistent with IPF, further testing with an echocardiogram should be considered.

The Mantoux test or purified protein derivative (PPD) is a skin test for tuberculosis. This patient's presentation is not consistent with tuberculosis (TB), so a PPD would not be recommended. Chronic cough, fever, night sweats, weight loss, along with TB risk factors (such as nursing home setting, foreign birth or travel, etc.) would indicate PPD.

Ultrasound of the thorax would not be helpful in evaluating possible IPF. In fact, ultrasound of the thoracic structures (outside of echocardiography) is not often done. A plain chest film (X-ray) is a good starting point for pulmonary complaints, followed by a CT as the next appropriate test.

A ventilation-perfusion scan is a test that is primarily used in the diagnosis of pulmonary embolism (PE). This patient is not presenting with a history consistent with a PE (which may include acute dyspnea and chest pain).
Explanation
This patient's most likely diagnosis is (IPAH). Calcium channel blockers, such as nifedipine, have been used to treat IPAH.

Calcium channel blockers (CCBs) are believed to act on the vascular smooth muscle, dilating the pulmonary resistance vessels and lowering the pulmonary artery pressure. Several studies report clinical and hemodynamic benefits from the use of long-term calcium channel blockade. Long-term treatment improves the quality of life and survival rate in patients who have a proven response to such therapy. In general, CCBs are used at high doses in patients with IPAH.

Cardiac glycosides are used for prevention and treatment of supraventricular arrhythmias associated with SPAH and for patients who have concomitant left-sided heart failure.

Digoxin is not useful in the treatment of right-sided ventricular failure.

Only patients with an acute vasodilator response to an intravenous or inhaled pulmonary vasodilator challenge (e.g., with inhaled nitric oxide at 10 to 20 parts per million, intravenous epoprostenol (2 to 12 ng/kg/min), intravenous adenosine (50 to 350 mg/min), or inhaled iloprost [5 mg]) derive any long-term benefit from CCBs.

Parenteral vasodilators such as epoprostenol are used for patients whose IPAH fails to respond to calcium channel blockers or who cannot tolerate these agents and who have New York Heart Association (NYHA) type III or IV right-sided heart failure.

Ambrisentan is an endothelin receptor antagonist indicated for pulmonary arterial hypertension in patients with WHO class II or III symptoms. It improves exercise ability and decreases progression of clinical symptoms. It inhibits vessel constriction and elevation of blood pressure by competitively binding to endothelin-1 receptors ETA and ETB in endothelium and vascular smooth muscle. Because of the risks of hepatic injury and teratogenic potential, this agent is available only through the Letairis Education and Access Program (LEAP).

Beta-blockers, such as metoprolol, have no established role in the treatment of IPAH.
Correct answer:
Prednisone

Explanation
This patient most likely has hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis. This is an inflammatory disorder of the lung involving alveolar walls and terminal airways that is induced by repeated inhalation of a variety of organic agents. The chronic form of HP typically results from low-grade or recurrent exposure over many months to years, and the lung disease may already be partially or completely irreversible. These patients are usually advised to avoid all possible contact with the offending agent.

In addition to identification of the causative agent and its avoidance, institution of glucocorticoid treatment is indicated. Prednisone at a dosage of 1 mg/kg per day or its equivalent is continued for 7 - 14 days. It is then tapered to 0.25-0.5 mg/kg and is maintained at this level for an additional 4 - 12 weeks at a rate that depends on the patient's clinical status. While patients with chronic HP may gradually recover without therapy following environmental control, a trial of prednisone may be useful to obtain maximal reversibility of the lung disease. Following initial prednisone therapy (1 mg/kg per day for 2 to 4 weeks), the drug is tapered to the lowest dosage that will maintain the functional status of the patient. Many patients will not require or benefit from long-term therapy if there is no further exposure to the antigen. Improvement of lung function may continue over a few months to years.

If the patient's condition continues to decline on glucocorticoids, a second agent should be introduced while lowering or maintaining the prednisone dose at 0.25 mg/kg per day.

Glucocorticoid therapy is recommended for symptomatic interstitial lung disease (ILD) patients with eosinophilic pneumonias, cryptogenic organizing pneumonia, connective tissue diseases, sarcoidosis, hypersensitivity pneumonitis, acute inorganic dust exposures, acute radiation pneumonitis, diffuse alveolar hemorrhage, and drug-induced ILD. In organic dust disease, glucocorticoids are recommended for both the acute and chronic stages.
Correct answer:
Malignancy

Explanation
Malignancy is the correct response.

A pleural effusion is an abnormal accumulation of fluid in the pleural space. Patients with pleural effusions most often report dyspnea, cough, and pleuritic chest pain. Large pleural effusions are more likely to be symptomatic than smaller effusions. Physical findings are usually absent in small effusions. Larger effusions may present with dullness to percussion and diminished or absent breath sounds over the affected area.

A diagnostic thoracentesis should be performed whenever there is a new pleural effusion and no clinically apparent cause, when there is an atypical presentation, or when an effusion fails to resolve as expected. Sampling allows visualization of the fluid in addition to chemical and microbiologic analyses to help identify the etiology of the effusion. Pleural samples should be sent for measurement of protein, glucose, and LDH, in addition to total and differential white blood cell counts. These chemistry tests are used to classify effusions as a transudate or an exudate. This distinction is important because the differential diagnosis for each is entirely different.

A pleural exudate is an effusion that has 1 or more of the following laboratory features: (1) ratio of pleural fluid protein to serum protein > 0.5; (2) ratio of pleural fluid LDH to serum LDH > 0.6; and (3) pleural fluid LDH greater than 2/3 the upper limit of normal serum LDH. Additionally, samples with low glucose levels usually indicate a bacterial or significant inflammatory etiology. In contrast, transudative effusions have none of these features. Transudates are also distinguished by fewer than 1000 white blood cells/microliter and a pleural glucose level equal to serum. These types of effusions occur in the setting of normal capillary integrity and also suggest the absence of local pleural disease.

The characteristics of the pleural fluid from the patient in the clinical scenario presented above indicate an exudative pleural effusion. Malignancy is the most likely etiology in this patient, as a bacterial cause would typically present with a decreased glucose level in the pleural fluid compared with the serum; there is an equal glucose level in this case.

Congestive heart failure accounts for >90% of transudative pleural effusions. Nephrotic syndrome and cirrhosis with ascites can also lead to transudative pleural effusions. Bacterial pneumonia and cancer are the most common causes of exudative effusions.
Correct answer:
Prescribe a diuretic, such as furosemide

This patient is presenting with a progression of a primary pulmonary disease (pulmonary hypertension) into cor pulmonale, which is also known as pulmonary heart disease. Cor pulmonale, when moderate to severe, will present with signs and symptoms of right heart failure, such as the severely fluid overloaded state. In addition to treatment of the underlying pulmonary disorder, cor pulmonale is treated much like right-sided heart failure is treat. It would be most appropriate to prescribe a diuretic, such as furosemide, at this time. If possible, this patient should also be referred to a cardiopulmonary specialist.

A calcium channel blocker, such as verapamil, can actually worsen right ventricular function, which is already diminished in this patient. The ECG findings indicate right ventricular hypertrophy.

A fluoroquinolone, such as levofloxacin, would be an appropriate choice if this patient's dyspnea were due to bacterial pneumonia. However, she is afebrile, and her fluid overload symptoms and ECG findings are not typical in cases of pneumonia.

A lipase inhibitor, such as orlistat, might be considered for weight loss in a patient with obesity and the inability to lose weight. However, this patient reports a relatively recent weight gain, which is attributable to fluids; therefore, orlistat would be inappropriate for this patient.

A thiazolidinedione, such as actose, is contraindicated in a patient with symptomatic heart failure. In essence, this patient has right-sided heart failure due to her pulmonary hypertension.
Correct answer:
Paraneoplastic

Explanation
Paraneoplastic syndrome (Lambert-Eaton syndrome), resembling myasthenia gravis, occurs in some people with small cell carcinoma of the lungs. It usually manifests as progressive weakness in the large muscles. Lambert-Eaton syndrome is caused by the inhibition of voltage-gated calcium channels on the presynaptic membrane of the neuromuscular junction; this prevents the release of acetylcholine. As the muscles continue to contract, acetylcholine can build up in sufficient quantities for the strength to get better; weakness improves after repetitive muscle contraction. Although the underlying mechanism is autoimmune, Lambert-Eaton syndrome in this patient is regarded as paraneoplastic because it is a consequence of a cancer, not due to a local presence of cancer cells.

Tumor infiltration is a local manifestation of a tumor associated with the production of various types of extracellular matrix-degrading enzymes. Local infiltration of the nerves excludes variegated symptoms of autonomic nervous system disturbance and fluctuating weakness in several muscle groups.

Secondary spinal cord tumors usually follow hematogenous spread to the vertebral bodies, epidural expansion, and/or intramedullary metastasis. Subsequent symptoms of compression (i.e., pain, radicular, or medullar symptoms) gradually worsen, not improve with exercise.

Nicotine poisoning is not likely; it is impossible to overdose on nicotine through smoking alone. Smoking causes vascular disease, cancer, lung disease, peptic ulcer, and reproductive disturbances (e.g., premature birth). Nicotine may contribute to tobacco-related disease, but direct causation has not been determined because nicotine is consumed simultaneously with a multitude of other potentially harmful substances that occur in tobacco smoke. The effects of nicotine on nerves and muscles are generally dose-dependent; they also occur in nicotine-tolerant individuals. Initially, nicotine has a short-lived stimulatory phase followed by a longer inhibitory phase which leads to a neuromuscular blockade. Neuromuscular symptoms include hypotonia, decreased deep tendon reflexes, weakness, fasciculations, and paralysis of muscles (including respiratory muscles). Cholinergic effects on the autonomic nervous system, often observed initially, include diaphoresis, salivation, lacrimation, increased bronchial secretions, miosis, and later mydriasis. Nicotine acts on the sympathetic ganglia, chemoreceptors of the aorta, and carotid bodies; it affects the adrenal medulla, releasing catecholamines.
Correct answer:
Reduced intravascular oncotic pressure

Explanation
Pleural effusions, an abnormal collection of fluid in the pleural space, is related to an underlying pathology causing excess fluid production or decreased absorption. Effusions are classified as transudative or exudative depending on the characteristics of the fluid.

Patients with liver disease/cirrhosis have decreased protein and albumin production, leading to decreased oncotic pressure of the intravascular fluid. This contributes to ascites and dependent edema. Peritoneal ascites may travel across the diaphragm and lead to pleural effusions (hepatic hydrothorax), most commonly on the right side. Pleural effusions caused by decreased oncotic pressure are transudative. Treatment is aimed at reducing the ascitic fluid using diuretics (loops and/or spironolactone). Severe cases may require treatment with a transjugular intrahepatic portosystemic shunt (TIPS) or liver transplant. Other causes of hypoalbuminemia, such as nephrotic syndrome, also cause pleural effusions due to reduced intravascular pressure.

Altered permeability of the pleural membrane causing a pleural effusion is seen with localized inflammation, malignancy, or pulmonary embolism. Decreased lymphatic drainage is commonly seen in malignancy (lung, breast, or lymphoma). The effusion is exudative and almost always unilateral on the side with the associated pathology.

Pleural effusions caused by congestive heart failure are due to increased hydrostatic pressure. They are usually bilateral and transudative.

Reduced pressure in the pleural space prevents full lung expansion, allowing pleural fluid accumulation. Causes of this include atelectasis (transudative) or mesothelioma (exudative).
Correct answer:
Physical exam findings of pulmonary hypertension commonly occur.

Explanation
This patient's most likely diagnosis is idiopathic pulmonary fibrosis. It is critical to obtain a complete history, including medication history, social history, occupational history, exposure history, and review of systems, to ensure other causes of interstitial lung disease are excluded. Amiodarone, bleomycin, and nitrofurantoin are notable medications associated with pulmonary fibrosis.

Most patients with idiopathic pulmonary fibrosis present with a gradual onset, often for longer than 6 months duration. Dyspnea, which is the most prominent symptom in idiopathic pulmonary fibrosis (IPF), usually begins insidiously and is often progressive.

Approximately 5% of patients have no presenting symptoms when idiopathic pulmonary fibrosis is diagnosed. The clinical symptoms of idiopathic pulmonary fibrosis are nonspecific; symptoms often precede the diagnosis by a median of 1-2 years.

Most patients present with exertional dyspnea and a nonproductive cough.

Pulmonary hypertension is a common comorbidity in patients with idiopathic pulmonary fibrosis, and an estimated 20-40% of patients with idiopathic pulmonary fibrosis who are evaluated or listed for lung transplantation have pulmonary hypertension at rest. Patients may have a loud P2 component of the second heart sound, a fixed split S2, a holosystolic tricuspid regurgitation murmur, and pedal edema. As right ventricular hypertrophy ensues, a right ventricular heave may be palpated at the lower left sternal border and increased right atrial pressure may cause elevation of the jugular venous pressure.

The chest radiograph lacks diagnostic specificity for idiopathic pulmonary fibrosis. In most patients with idiopathic pulmonary fibrosis, the physical examination reveals fine bibasilar inspiratory crackles (Velcro crackles). Additionally, digital clubbing is seen in 25-50% of patients with IPF.
Correct answer:
Cor pulmonale

Explanation
Myocardial infarctions occur at rest and most commonly in the early morning. The pain is similar to angina in location and radiation, but it may be more severe and builds up rapidly or in waves to maximum intensity over a few minutes or longer. Associated symptoms include diaphoresis, weakness, apprehension, and a feeling of impending doom; patients may move about, seeking a position of comfort, preferring not to lie quietly. Light-headedness, syncope, dyspnea, orthopnea, cough, wheezing, nausea and vomiting, or abdominal bloating may occur. Physical exam findings may include fever, anxiousness, diaphoresis, bradycardia or tachycardia, low cardiac output, or arrhythmia. There may be hypertension (in hypertensive patients) or low in patients with shock. Respiratory distress, jugular venous distention a Kussmaul sign, soft heart sounds, and atrial gallops (S4) or ventricular gallops (S3) usually indicate heart failure.

Primary biliary cirrhosis is most common in middle-aged females and is characterized by fatigue, pruritus, hepatosplenomegaly, xanthomatous lesions on the skin, eyelids, and tendons, jaundice, and steatorrhea. Signs of portal hypertension are late findings. Other findings include orthostatic hypotension and cognitive dysfunction.

Left-sided or forward failure may account for many of the clinical manifestations of heart failure, such as mental confusion from decreased cerebral perfusion, fatigue and weakness from decreased skeletal muscle perfusion, and sodium and water retention with secondary venous congestion from decreased renal perfusion.

Isolated left-side heart failure is associated with dyspnea, fatigue, weakness, cough, paroxysmal nocturnal dyspnea, and orthopnea in the absence of peripheral edema, jugular venous distention (JVD), or hepatojugular reflux.

Chest pain, dyspnea, and tachypnea are the most frequent signs and symptoms of pulmonary embolism. Other manifestations may include tachycardia, pleurisy, low-grade fever, apprehension, and productive cough with blood-tinged sputum. Massive PE may manifest as sudden collapse, crushing substernal chest pain, shock, diaphoresis, hypotension, distended neck veins, and loss of consciousness.
Correct answer:
Perform needle decompression

Explanation
This patient has the signs and symptoms of a tension pneumothorax; in this patient's case, it could have resulted from the diagnostic thoracentesis, or it could have resulted from his underlying pulmonary disease. The most appropriate measure would be to perform needle decompression as you prepare for insertion of an underwater sealed chest tube.

Symptoms of tension pneumothorax include unilateral chest pain, dyspnea, tachypnea, and hypotension. On examination, there is diminished chest wall excursion on the affected side, increased tympany on percussion, and diminished or absent tactile fremitus. Mediastinal shift to the contralateral side may be detectable as displacement of the trachea, cardiac dullness, and apex beat away from the affected side. Breath sounds are markedly depressed or absent. The chest X-ray usually shows air without peripheral lung markings, limited by a sharp visceral pleural margin with lung markings medially, indicating the position of the collapsed lung.

When the clinical situation suggests a tension pneumothorax, a needle decompression of the affected side (side with decreased breath sounds and hyperresonance on percussion) should be performed. Air is removed by inserting into the chest wall a large bore needle attached through a 3-way stopcock to a large syringe. The needle may be inserted anteriorly in the 2nd intercostal space in the midclavicular line.

Giving the patient oxygen, either by mask or endotracheally, would not relieve him of his symptoms, which are due the pleural accumulation of the air causing a shift of the mediastinum. Due to the rapid deterioration of the patient's clinical condition, it would not be advisable to send him for a chest X-ray before relieving the tension pneumothorax. In tension pneumothorax, a bronchopleural fistula acts as a valve and allows air to enter but not leave the pleural space, causing pressure in the space to rise above the atmospheric pressure. The continued accumulation of air in the chest results in lung collapse and a shift of the mediastinal structures to the opposite side, which impedes venous return to the heart. In turn, this results in hypotension, abnormal gas exchange, and ultimately cardiovascular collapse. When the presentation is less urgent and there is time for a chest X-ray, sites where the lung is held to the chest wall by adhesions should be avoided. Air is alternately withdrawn from the pleural space and expelled from the syringe into the room until an underwater sealed tube thoracostomy has been inserted.
Correct answer: Small cell carcinoma

Small cell carcinoma is most frequently a central lesion not associated with cavitation. A hilar mass is associated with small cell carcinoma. Small cell carcinoma is associated with paraneoplastic syndromes. Cushing's syndrome secondary to ectopic ACTHproduction can sometimes be seen with small cell carcinoma, as can syndrome of inappropriate antidiuretic hormone production (SIADH).

An adenocarcinoma is usually seen peripherally on X-ray. An adenocarcinoma is characterized pathologically by mucus production. On the rare occasion when lung cancer occurs in somebody who has never smoked, it is most often classified as an adenocarcinoma. Adenocarcinomas may develop in areas of the lung where there is scarring or fibrosis. For example, a cause of the scarring can be a granulomatous infection, such as tuberculosis. For this reason, adenocarcinoma is sometimes referred to as "scar carcinoma".

Wilms tumor is a pediatric malignancy of the kidney. It can metastasize to the lungs.

Large cell carcinoma is usually seen peripherally on X-ray. Large cell carcinoma shows poorly differentiated cells. It does not show squamous features or glandular features. Large cell carcinoma does not react to mucicarmine staining. Large cell carcinoma is also called undifferentiated carcinoma or anaplastic carcinoma. The cell of origin is unknown.

Squamous cell carcinoma is centrally located. Cavitation can also sometimes be seen with X-ray. Keratin formation, keratin pearl formation, and intercellular bridges can be seen with squamous cell carcinoma.
Correct answer: History of previous PCP infection

History of previous Pneumocystis jiroveci pneumonia (PCP) infection is the correct answer. Patients who are HIV positive should take antibiotic prophylaxis for PCP if they have a history of PCP infection, if their CD4 cell count is below 200, or if they have evidence of immunodeficiency, such as oral candidiasis. Prophylaxis for PCP is typically daily trimethoprim/sulfamethoxazole double strength, but it can also be done with daily dapsone or monthly aerosolized pentamidine. If patients are on antiretroviral therapy and their CD4 cell count remains above 200 for at least 3 months, then they can stop prophylaxis. If their CD4 cell count drops below 200 again, they need to restart prophylaxis.

CD4 cell count <400 is not the correct answer. Patients who are HIV positive need to be on PCP prophylaxis for various reasons, one of which is a CD4 count <200. A CD4 cell count between 200 and 400 would not warrant prophylaxis, until the point that the count drops below 200.

CD4 cell count >200 is not the correct answer. A CD4 cell count over 200 would actually be a reason NOT to have the patient on PCP prophylaxis, as one of the criteria to start prophyhlaxis is a CD4 cell count below 200. In addition, when a patient has been on prophylaxis and antiretrovirals and their CD4 cell count is over 200 for 3 months, they can stop the prophylaxis.

History of any pneumonia infection is not the correct answer. Only patients who have had previous PCP infections need to be treated with prophylaxis for PCP. Having had other types of pneumonia does not warrant PCP prophylaxis.

HIV viral load >100,000 copies/mL is not the correct answer. HIV viral loads over 100,000 warrant antiretroviral treatment of HIV, but PCP prophylaxis is not given based on viral load. Rather, it is given based on CD4 cell count, previous PCP infection, or evidence of immunodeficiency.
Correct answer: Syndrome of inappropriate antidiuretic hormone secretion (SIADH)

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a paraneoplastic syndrome associated with bronchogenic carcinoma. There is excessive water reabsorption by the kidneys due to the elevated antidiuretic hormone (ADH). Patients may complain of feeling weak and lethargic. Other symptoms include confusion and coma. Laboratory tests reveal hyponatremia, high urine sodium, low serum osmolality, and high urine osmolality in a euvolemic patient.

In hypertrophic pulmonary osteoarthropathy (HPO), there is formation of new subperiosteal cancellous bone at the distal ends of long bones. Patients present with periarticular pain, polyarthralgia, and painful swellings at the wrists, knees, elbows, and ankles. On examination, there is clubbing of the fingers and toes. There may also be localized articular erythema, tenderness, swelling, and even effusions. X-rays may reveal subperiosteal bone formation with periosteal elevation seen as thickening and detachment of the periosteum.

Lambert-Eaton myasthenic syndrome is an immune-mediated disorder of neuromuscular transmission resulting in impaired release of acetylcholine from nerve terminals. Patients complain of weakness of the scapular and pelvic girdles musclesand the resultant difficulties in performing activities such as climbing stairs. Other symptoms such as dry mouth, paresthesias, and sexual impotence due to autonomic dysfunction may also be present. On examination, there is proximal muscle weakness. Deep tendon reflexes are decreased or absent.

Horner's syndrome is due to a bronchogenic carcinoma tumor mass extending to the sympathetic chain. Horner's syndrome symptoms include miosis, ptosis, enophthalmos, and hemianhidrosis. In addition, the patients may also have atrophy of hand muscles.

Cushing's syndrome may develop as a result of the small cell carcinomas secreting ectopic adrenocorticotropic hormone (ACTH). Patients may complain of bruising easily. On examination, they may have red-purplish striae, especially over their abdomen, in addition to the typical moon facies and truncal obesity. They may also have muscle wasting, especially of the proximal limb girdle muscles. Laboratory investigations reveal hypokalemia and high plasma ACTH, as well as increased serum and urine cortisol.
Correct answer: Bronchial carcinoid tumor

Bronchial carcinoid tumors, though rare in children, are the most common primary malignant lung tumor in children. They tend to arise in the perihilar region. Carcinoid tumors are rare neuroendocrine tumors occurring most often in the GI tract, especially the appendix. They can be associated with systemic symptoms due to the release of a variety of hormones. One of these hormones, serotonin, is thought to cause carcinoid syndrome. This consists of episodic flushing, wheezing and diarrhea, and it is noted in less than 1% of patients with bronchial carcinoid tumors. It is more common for midgut primary tumors. Patients may also present with recurrent pneumonia, cough, hemoptysis, wheezing, asthma, or chest pain. Metastasis, if present, usually occurs in mediastinal lymph nodes, the liver, bone, or skin. Resection is the preferred treatment for localized tumors; survival rates, when localized, are greater than 90%.

Pulmonary embolism is extremely rare in pediatric patients, but it may be considered in a young female patient using oral contraceptives or in one who has had a recent abortion. It is also possible in a young male with recent leg trauma. Presenting symptoms include dyspnea, fever, pleuritic pain, cough, and hemoptysis.

Bacterial pneumonia patients typically present with high temperatures and associated pleural effusions, along with a high percentage of band forms on lab study. Most commonly, it is secondary to acute viral bronchitis occurring during minor upper respiratory infection, or it is complicating an underlying chronic illness.

Pulmonary hemosiderosis can occur as primary lung disease, or it can be secondary to cardiovascular or systemic disease. It is characterized by repeated episodes of intra-alveolar bleeding leading to abnormal accumulation of iron as hemosiderin in alveolar macrophages with subsequent fibrosis and severe anemia. Nonspecific recurrent or chronic pulmonary symptoms such as cough, dyspnea, tachypnea, and wheezing are observed most often. Hemoptysis may or may not occur in children.

Vascular malformations include congenital structural anomalies between arteries and veins within blood vessels of the lungs, resulting in blood shunting and poor oxygenation. Many patients have no symptoms; others have dyspnea, shortness of breath with exertion, or bloody sputum. Long-term changes include clubbing, low oxygen saturation, and elevated hematocrit. Chest X-ray will usually reveal abnormal blood vessels. In addition, a murmur may be heard on auscultation.
Correct answer: Sandostatin (Octreotide)

This patient's diagnosis is consistent with carcinoid tumor. Carcinoid tumors are of neuroendocrine origin and derived from primitive stem cells in the gut wall, but they can be seen in other organs, including the lungs,mediastinum, thymus, liver, pancreas, bronchus, ovaries, prostate, and kidneys. Carcinoid tumors have high potential for metastasis. Neuroendocrine tumors contain somatostatin receptors. This feature allows for the localization of primary tumors and tumor metastases by scintigraphy with the radiolabeled somatostatin analog octreotide. It can be used to detect certain types of cancer arising from the neuroendocrine system.

As a somatostatin analog, sandostatin is highly effective in reducing symptoms. Survival advantage with the use of this drug has not yet been proven. Sandostatin reduces the amount of the growth factor produced, thus theoretically impairing growth.

Danocrine suppresses pituitary-ovarian axis by inhibition of pituitary gonadotropin output. It is indicated in the treatment of endometriosis, fibrocystic breast disease, and hereditary angioedema.

Cytarabine is an antimetabolite chemotherapeutic agent that inhibits DNA polymerase during the S phase; it is indicated in the treatment of acute myelogenous leukemia and acute lymphocytic leukemia.

Imatinib is a tyrosine kinase inhibitor used in the treatment of acute lymphoblastic leukemia, myelodysplastic, or myeloproliferative diseases, hypereosinophilic syndrome, eosinophilic leukemia, and chronic myeloid leukemia.

Gemcitabine is indicated as first-line treatment for locally advanced (nonresectable Stage II or Stage III) or metastatic (Stage IV) adenocarcinoma of the pancreas.
Correct answer: Treatment with theophylline

Treatment with theophylline is the correct response. Theophylline is a bronchodilator medicine frequently used in the treatment of asthma and other airway disease including chronic bronchiolitis. Some of the side effects associated with its use are nausea, fast heartbeat, and tremor. The ECG result depicted in the figure illustrates a fast heartbeat condition called ventricular tachycardia, in which the ventricles are contracting faster than normal. This can be a consequence of long-term administration of theophylline, especially in elderly people.

Treatment with inhaled corticosteroids is an incorrect response. Inhaled corticosteroids are used to reduce airway inflammation, but their side effects are different; they include oral infection and bruising.

Treatment with oral steroids is an incorrect response. Oral steroids are prescribed to prevent worsening of chronic obstructive pulmonary disease (COPD). Their side effects are weight gain, diabetes, osteoporosis, cataracts, and an increased risk of infection; these are different from the symptoms of the examined patient.

Treatment with a phosphodiesterase-4-inhibitor is an incorrect response. Phosphodiesterase-4 inhibitors, such as roflumilast, are used to decrease the inflammation of the airways. The most common side effects associated with their use are weight loss and diarrhea.

Treatment with antibiotics is an incorrect response. Respiratory infections are generally viral infections that cannot be cured with antibiotics. Sometimes, they are used for their anti-inflammatory properties and to prevent bacterial infections that might worsen the condition; however, they do not cause cardiac problems.
Correct answer: Ectopic adrenocorticotropic hormone (ACTH) secretion

Cushing's syndrome may develop as a result of the small cell carcinomas secreting ectopic adrenocorticotropic hormone (ACTH). Patients may complain of bruising easily. On examination, they may have red-purplish striae, especially over their abdomen, in addition to the typical moon facies and truncal obesity. They may have muscle wasting, especially of the proximal limb girdle muscles. Laboratory investigations reveal hypokalemia and high plasma ACTH, as well as increased serum and urine cortisol.

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a paraneoplastic syndrome associated with bronchogenic carcinoma. There is excessive water reabsorption by the kidneys due to the elevated antidiuretic hormone (ADH). Patients may complain of feeling weak and lethargic. Other symptoms include confusion and coma. Laboratory tests reveal hyponatremia, high urine sodium, low serum osmolality, and high urine osmolality in a euvolemic patient.

In cases of hypertrophic pulmonary osteoarthropathy (HPO), there is formation of new subperiosteal cancellous bone at the distal ends of long bones. Patients present with periarticular pain, polyarthralgia, and painful swellings at the wrist, knees, elbows, and ankles. On examination, there is clubbing of the fingers and toes. There may also be localized articular erythema, tenderness, swelling, and even effusions. X-rays may reveal subperiosteal bone formation with periosteal elevation that is seen as thickening and detachment of the periosteum.

Lambert-Eaton myasthenic syndrome is an immune-mediated disorder of neuromuscular transmission. It results in impaired release of acetylcholine from nerve terminals. Patients complain of weakness of the scapular and pelvic girdles musclesand the resultant difficulties in performing activities such as climbing stairs. Other symptoms such as dry mouth, paresthesias, and sexual impotence due to autonomic dysfunction may also be present. On examination, there is proximal muscle weakness. Deep tendon reflexes are decreased or absent.

Horner's syndrome is due to a bronchogenic carcinoma tumor mass extending to the sympathetic chain. Horner's syndrome symptoms include miosis, ptosis, enophthalmos, and hemianhidrosis. In addition, the patients may also have atrophy of hand muscles.
Correct answer:
Distended neck veins with prominent a or v waves

This patient's diagnosis is chronic cor pulmonale. Cor pulmonale is estimated to account for 6-7% of all types of adult heart disease in the United States, with chronic obstructive pulmonary disease (COPD) due to chronic bronchitis or emphysema the most common cause (the causative factor in more than 50% of cases). Cor pulmonale usually presents chronically; however, 2 main conditions can cause acute cor pulmonale: pulmonary embolism (more common) and acute respiratory distress syndrome (ARDS).

In chronic cor pulmonale, right ventricular (RV) hypertrophy (RVH) generally predominates. Common symptoms are fatigue, tachypnea, exertional dyspnea, and cough. Other symptoms include anginal chest pain (due to right ventricular ischemia), hemoptysis, and, rarely, hoarseness due to compression of the left recurrent laryngeal nerve by a dilated pulmonary artery. Anorexia, right upper quadrant abdominal discomfort, and jaundice may occur due to passive hepatic congestion.

Physical exam findings reflect the underlying lung disease or pulmonary hypertension, right ventricular hypertrophy (RVH), and RV failure. Expected signs include an increase in chest diameter, labored respiratory efforts with retractions of the chest wall, distended neck veins with prominent a or v waves, and cyanosis. Wheezes and crackles may be audible upon auscultation due to underlying lung disease. S2 heart sound splitting with an accentuated pulmonic component may be found early, while a systolic ejection murmur with sharp ejection click over the region of the pulmonary artery may be heard in advanced disease, along with a diastolic pulmonary regurgitation murmur. Other cardiac findings include third and fourth sounds and the systolic murmur of tricuspid regurgitation. RVH is characterized by a left parasternal or subxiphoid heave. Hepatojugular reflux and pulsatile liver are signs of RV failure with systemic venous congestion. On percussion, hyperresonance of the lungs may be a sign of underlying COPD; ascites can be seen in severe disease. Examination of the lower extremities reveals evidence of pitting edema.

Dullness to percussion and increased tactile fremitus of the lungs suggests a lung consolidation, as in pneumonia, pulmonary edema, or pulmonary hemorrhage.

Pericardial friction rubs suggest inflammation of the pericardial sac, while a laterally displaced and enlarged point of maximal impulse occurs with left ventricular hypertrophy, congestive heart failure, cardiomyopathy, and ischemic heart disease.

The finding of a systolic ejection murmur located at the aortic valve area most likely suggests a diagnosis of aortic stenosis.
The correct response is pneumococcal vaccine (PCV13).

Your patient suffers recurrent respiratory infections and diarrhea; he also has skin changes and a family history highly suggestive of an X-linked immune deficiency, most likely a severe combined immunodeficiency (SCID). SCID is a group of congenital diseases caused by different genetic mutations resulting in the severe deficiency of T and B- lymphocytes. Onset before age 6 months suggests a T-cell defect, as maternal antibodies are present for the 1st 6 to 9 months. Live vaccines are contraindicated in SCID because introducing an infectious agent, even a weakened one, can cause actual disease. However, pneumococcal vaccine PCV13 is considered risk-specific and is recommended in a 4-dose series at ages 2, 4, and 6 months (minimum age of 6 weeks), as well as at age 12 through 15 months.

Rotavirus (RV) is a live vaccine and cannot be given to SCID patients.

The measles, mumps, and rubella (MMR) vaccine is a live vaccine and cannot be given to patients with SCID.

BCG, or Bacille Calmette-Guerin, is not generally recommended for use in the United States because of the low risk of infection with Mycobacterium tuberculosis, the variable effectiveness of the vaccine against adult pulmonary TB, and the potential interference with tuberculin skin test reactivity. More importantly, in regard to this case, it is a live vaccine and should not be given to any immunosuppressed person.

Variccella (Var) is also a live vaccine and cannot be given to SCID patients.
Correct answer: Horner's syndrome

Horner's syndrome is due to a bronchogenic carcinoma tumor mass extending to the sympathetic chain. Horner's syndrome comprises of miosis, ptosis, enophthalmos, and hemianhidrosis. In addition, the patients may also have atrophy of hand muscles.

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a paraneoplastic syndrome associated with bronchogenic carcinoma. There is excessive water reabsorption by the kidneys due to the elevated antidiuretic hormone (ADH). Patients may complain of feeling weak and lethargic. Other symptoms include confusion and coma. Laboratory tests reveal hyponatremia, high urine sodium, low serum osmolality, and high urine osmolality in a euvolemic patient.

Cushing's syndrome may develop as a result of the small cell carcinomas secreting ectopic adrenocorticotropic hormone (ACTH). Patients may complain of bruising easily. On examination, they may have red-purplish striae, especially over their abdomen, in addition to the typical moon facies and truncal obesity. They may also have muscle wasting, especially of the proximal limb girdle muscles. Laboratory investigations reveal hypokalemia, high plasma ACTH, as well as increased serum and urine cortisol.

In cases of hypertrophic pulmonary osteoarthropathy (HPO), there is formation of new subperiosteal cancellous bone at the distal ends of long bones. Patients present with periarticular pain, polyarthralgia, and even painful swellings at the wrists, knees, elbows, and ankles. On examination, there is clubbing of the fingers and toes. There may also be localized articular erythema, tenderness, swelling, and even effusions. X-rays may reveal subperiosteal bone formation, with periosteal elevation seen as thickening and detachment of the periosteum.

Lambert-Eaton myasthenic syndrome is an immune-mediated disorder of neuromuscular transmission, resulting in impaired release of acetylcholine from nerve terminals. Patients complain of weakness of the scapular and pelvic girdles muscles,and the resultant difficulties in performing activities such as climbing stairs. Other symptoms such as dry mouth, paresthesias, and sexual impotence due to autonomic dysfunction may also be present. On examination, there is proximal muscle weakness. Deep tendon reflexes are decreased or absent.
Correct answer: Bronchoalveolar lavage typically reveals neutrophilia and eosinophilia.

This patient's most likely diagnosis is idiopathic pulmonary fibrosis. Reportedly, up to 30% of patients with idiopathic pulmonary fibrosis (IPF) have positive tests for antinuclear antibodies or rheumatoid factor; however, these titers are generally not high. The presence of high titers of antinuclear antibodies or rheumatoid factor may suggest the presence of a connective-tissue disease. The C-reactive protein value and erythrocyte sedimentation rate can be elevated in patients with idiopathic pulmonary fibrosis; however, this finding is nondiagnostic.

The typical findings on pulmonary function tests in patients with idiopathic pulmonary fibrosis are a restrictive ventilatory defect and a reduced diffusion capacity for carbon monoxide.

Bronchoscopy with BAL and/or transbronchial biopsy is not required for the diagnosis of idiopathic pulmonary fibrosis. However, it can be used to ensure that alternative diagnoses are excluded. Given the high-quality evidence regarding high resolution CT-scanning (HRCT) specificity for the recognition of histopathologic idiopathic pulmonary fibrosis pattern, transbronchial or surgical lung biopsy is not essential in making the diagnosis.

Increased numbers of neutrophils in BAL fluid are found in 70-90% of all patients with idiopathic pulmonary fibrosis, and increased numbers of eosinophils in BAL fluid are found in 40-60%.

Virtually all patients with idiopathic pulmonary fibrosis (IPF) have an abnormal chest radiograph at the time of diagnosis, with typical findings of peripheral reticular opacities (netlike linear and curvilinear densities) predominantly at the lung bases, honeycombing (coarse reticular pattern), and lower lobe volume loss.

The chest radiograph lacks diagnostic specificity for idiopathic pulmonary fibrosis. The typical findings are peripheral reticular opacities predominantly at the lung bases, honeycombing, and lower lobe volume loss. High-resolution computed tomography (HRCT) findings are significantly more sensitive and specific for the diagnosis of idiopathic pulmonary fibrosis and are an essential component of the diagnostic pathway of idiopathic pulmonary fibrosis. On HRCT images, idiopathic pulmonary fibrosis is characterized by patchy, predominantly peripheral, predominantly subpleural, and bibasilar reticular opacities.
Correct answer: Breast cancer

A pleural effusion results from an abnormal accumulation of fluid in the pleural space. Transudative pleural effusions suggest the absence of localized pleural disease and are caused by increased hydrostatic pressure, as seen in heart failure, or decreased oncotic pressure, as seen in cirrhosis. Because there is no underlying pleural disease process, transudative pleural effusions are often bilateral. Exudative pleural effusions are indicative of an underlying pleural disease process, such as pneumonia or a malignancy. A diagnostic thoracentesis can help distinguish transudative from exudative pleural effusions. The fluid should be sent for a cell count, chemistry, and, if indicated, cytology or culture. A pleural effusion is diagnosed as exudative based on the chemistry when the pleural fluid meets 1 or more of the following criteria: "1) ratio of pleural fluid protein to serum protein greater than 0.5; 2) ratio of pleural fluid LDH to serum LDH greater than 0.6; and 3) pleural fluid LDH greater than 2/3 the upper limit of normal serum LDH."

The patient presented in this case was treated for left-sided breast cancer with a lumpectomy and radiation therapy 10 years ago. She underwent a thoracoscopy and pleurodesis. During the procedure, 1500 cc of pleural fluid was removed. Cytology was positive for breast cancer.

A patient with chronic kidney disease, heart failure, or cirrhosis is more likely to have bilateral transudative pleural effusions. Hypertension by itself is not a risk factor for a pleural effusion.
Correct answer: Hospice care with the diagnosis of end stage HIV/AIDS disease

This patient has end-stage HIV/AIDS, and hospice care is appropriate for him. Patients with HIV/AIDS should meet the following criteria to be eligible for hospice services:

CD4+ count < 25 cells/mcL or persistent viral load >100,000 copies/mlfrom 2 or more assays at least 1 month apart, and at least 1 of the following conditions:

Central nervous system (CNS) lymphoma,
Visceral Kaposi's sarcoma unresponsive to therapy,
Progressive multifocal leukoencephalopathy (PML),
Cryptosporidiosis,
Mycobacterium avium complex (MAC) bacteremia, untreated, refractory, or treatment refused
Untreated or refractory wasting (loss of >33% lean body mass),
Renal failure in the absence of dialyses, and
Refractory toxoplasmosis
There should also be a palliative performance scale of < 50% (the patient requires considerable assistance and frequent medical care as activity is mostly limited to bed or chair).

Other supporting documentations include:

Chronic persistent diarrhea for 1 year,
Persistent serum albumin < 2.5 gm/dL,
Concomitant substance abuse,
Age greater than 50,
Congestive heart failure which is symptomatic at rest,
Patient has elected to forego antiretroviral and prophylactic medication related specifically to HIV.
There is no indication that the patient has end-stage heart disease, end-stage pulmonary disease, or end-stage renal disease; however, pneumonia and congestive heart failure are helpful factors for making decisions regarding patients with end-stage HIV disease.
Correct answer: Tension pneumothorax

Air trappings, which can occur in patients with lower airway diseases such as BPD, may result in tension pneumothorax. Since the tracheal tube tends to enter the straighter right main bronchus, a tension pneumothorax most frequently occurs on the right side. This presents as a marked, sudden deterioration in oxygenation, as a decreased portion of the lungs is now being adequately ventilated, marked bradycardia, as the increasing pressure on the affected side of the chest directly impedes venous return to the heart, and central cyanosis. Breath sounds and chest rise will be decreased on the affected side due to increased pressure impeding air entry.

Hyperresonance to percussion on the affected side and tracheal deviation away from the affected side may also be present, but it is better appreciated on a larger patient. Treatment consists of immediate needle decompression. Waiting for x-ray confirmation may prove fatal. An over-the-needle catheter of appropriate size for the patient is inserted in the intercostal space over the 3rd rib to avoid nerve structures in the midclavicular line. A gush of air and sudden improvement in oxygenation, cardiac output, and clinical status will be evident.

Inadequate tidal volume being delivered would cause a slow and gradual deterioration that would be most evident on blood gas measurements.

A large leak around the tracheal tube would be most evident by a hissing sound around the tube during the inspiratory phase. A large leak would also be unlikely in a small former premie with small airways.

Displacement of the tracheal tube, though a common occurrence, would most commonly cause decreased breath sounds on the left because tracheal tube tends to enter the straighter right main bronchus. Indeed, this probably does precede a tension pneumothorax in most cases.

Though a disconnected oxygen supply might cause a sudden change in clinical status, it would probably not be as marked, and it would not involve the findings of decreased breath sounds on the right and asymmetric chest rise.
Correct answer: Legionnaire's disease

Legionnaire's disease is caused by Gram-negative, non-acid-fast bacilli that present as an atypical pneumonia with high fever, pleuritic chest pain, non-productive cough, and dyspnea. Associated systemic symptoms include diarrhea, confusion, and renal dysfunction. A mild leukocytosis is generally present. Sputum production is scant and fails to reveal pathogens. CXR often shows patchy alveolar infiltrates with consolidation in the lower lobe; pleural effusion is seen in 50% of the patients. Serology by urine antigen testing is highly sensitive and specific for the diagnosis of Legionnaire's disease. Treatment includes administration of oxygen, antipyretics, and antibiotic therapy with erythromycin with or without rifampin, co-trimoxazole, tetracycline, doxycycline, or ciprofloxacin used alone or in combination (depending on the severity of the disease).

Tuberculosis may present either as a primary infection with fever, productive cough, and pleurisy, or as post-primary infection with constitutional symptoms of anorexia, weight loss, and night sweats along with productive cough and hemoptysis. Typical features of the chest X-ray are unilateral middle or lower lobe infiltrate and cavitation. Sputum staining shows acid-fast bacilli.

Klebsiella pneumonia typically produces illness in debilitated patients, especially alcoholics, presenting as hemoptysis, dense lobar consolidations, and a high incidence of abscess formation. Sputum Gram stains show large Gram-negative bacilli and abundant PMN leucocytes.

Cryptococcal pneumonia is often seen in the setting of immunodeficiency. Typical CXR is that of diffuse bilateral interstitial infiltrates. The diagnosis can be confirmed with sputum test for cryptococcal antigen (CRAG) and culture.

Haemophilus influenza is the one of the most common causes of bacterial pneumonia, more common in children than adults. Haemophilus pneumonia may be multilobar; there may be areas of pathy bronchopneumonia or frank consolidation. Effusions may be present and occasionally progress to empyema. Gram stain of sputum may reveal coccobacilli, though they may stain poorly.
Correct answer: Oxygen

This patient's most likely diagnosis is idiopathic pulmonary fibrosis (IPF). It is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). Patients with hypoxemia (PaO2 < 55 mmHg or oxygen saturation as measured using pulse oximetry [SpO2] < 88%) at rest or with exercise should be prescribed oxygen therapy to maintain a saturation of at least 90% at rest, with sleep, and with exertion.

Corticosteroids have not been evaluated in a randomized, placebo-controlled trial to determine their benefit in treating patients with idiopathic pulmonary fibrosis.

Retrospective uncontrolled studies have reported no survival benefits. Evidence-based guidelines recommend that patients with idiopathic pulmonary fibrosis should not be treated with corticosteroid monotherapy.

Colchicine has been shown to inhibit fibroblast proliferation and collagen synthesis in vitro; however, evidence-based guidelines recommend that patients with idiopathic pulmonary fibrosis should not be treated with colchicine.

Evidence-based guidelines recommend that the majority of patients with IPF should not be treated with N-acetylcysteine monotherapy.

Bosentan is an endothelin receptor A and B antagonist that is approved for the treatment of pulmonary hypertension. While bosentan has been shown to have antifibrotic effects in an animal model of pulmonary fibrosis, current evidence-based guidelines recommend that patients with idiopathic pulmonary fibrosis should not be treated with bosentan.
Correct answer: Oral omeprazole

Explanation
This patient likely has idiopathic pulmonary fibrosis (IPF). IPF is a chronic, progressive restrictive pulmonary disease of the lung parenchyma. IPF presents with exertional dyspnea and non-productive cough, as well as the imaging characteristics described for this patient. Treatment is complex. Overall, medications play a minor role in this relatively rare disease. However, a prescription for a proton-pump inhibitor (PPI), such as oral omeprazole, is likely to provide the most benefit for this patient. There are various theories (such as microaspiration) regarding the association of GERD and IPF. Treatment with acid-lowering therapies is linked with disease stabilization and improved survival in patients with IPF.

Cisplatin-based chemotherapy has a role in treatment of non-small cell lung cancer. It has not been proven to alter the course of IPF.

Inhaled albuterol may appear helpful in a patient with dyspnea, but it does not treat IPF. The beta-agonistic effects cannot override the damaged, fibrotic tissue.

Inhaled budesonide, an inhaled steroid, is commonly used for asthma and chronic obstructive pulmonary disease. However, this patient has a restrictive pulmonary disease. The pathophysiologic basis for IPF is much more a disorder of fibroblastic disease, in which there is an abnormal accumulation of extracellular matrix, and not inflammatory in nature. Anti-inflammatory medications (such as inhaled or oral steroids) do not alter the course of IPF.

Oral prednisone, another steroid, is often used in IPF in acute exacerbations, but has little evidence for any benefit and does not seem to alter the disease course. This patient is not having an acute exacerbation, so the PPI would be the best choice of the listed medications.
Correct answer: Ceftriaxone 1gm IV Q24H and azithromycin 500mg IV Q24H

The patient is possibly suffering from community-acquired pneumonia (CAP). He was admitted to the hospital because of vomiting; as a result, he would be unable to keep oral antibiotics down, so IV antibiotics are required. CAP is defined as a pneumonia that starts outside the hospital or nursing home or occurs within 48 hours of being admitted to a hospital, without any history of residence in a long-term facility for 2 weeks or more prior to the onset of symptoms. The most common causative organism is streptococcus pneumoniae. Other organisms include Haemophilus influenzae (especially in smokers), atypical organisms (e.g., Mycoplasma pneumoniae, chlamydia, and legionella), and viruses, most commonly influenza, but occasionally parainfluenza, respiratory syncytial virus, adenovirus, etc. Staphylococcus aureus is found in patients with influenza, cystic fibrosis, bronchiectasis, nursing home residents, and drug abusers. Klebsiella pneumoniae may be isolated in alcoholics and diabetics. Anaerobic bacteria can cause pneumonia in patients with poor dental hygiene or aspiration. Pseudomonas is another bacteria found in cystic fibrosis, bronchiectasis, and institutionalized patients. A complete blood count with differential, basic metabolic panel and pre-antibiotic sputum and blood cultures should be done in all hospitalized patients. Sputum culture is recommended in non-hospitalized patients. When sputum is not provided by a patient and culture is necessary, then transtracheal aspiration, fiberoptic bronchoscopy, and transthoracic needle aspiration may be done to obtain samples. Chest X-ray is helpful in many ways. Usual findings include lobar consolidation, patchy infiltrates, pleural effusion, cavitations, and other pulmonary abnormalities. Radiography helps in diagnosis and is also useful in assessing severity and response to treatment. Lung infiltrates may take up to 6 weeks to clear on X-ray. Pleural tap may be done in patients to rule out empyema; it may also be done for diagnostic reasons.

The causative organism directs treatment. A combination of ceftriaxoneandazithromycincoversGram-positive and atypical organisms.

Levofloxacin IV is also a good monotherapy in CAP, but it is not recommended in order to avoid drug resistance to quinolones. It should be used only in cases where the other 2 medications are contraindicated, such as in the case of a drug allergy; however, it is an invaluable antibiotic in cases of severe healthcare-acquired pneumonia (HCAP). Treatment should be continued for 7 - 14 days.

Levofloxacin PO is not recommended in this case due to the patient vomiting. If this patient had not been vomiting and was overall stable, outpatient treatment with oral levofloxacin for 7 - 14 days would have been an option; however, it would not have been highly recommended due to the danger of resistant organisms. In such a case, oral azithromycin for 7 - 10 days would also be a good option. Clarithromycin and doxycycline for 7 - 10 days may also be used.

Ceftazidime and vancomycin combination is used in HCAP; its coverage includes Gram-negative organisms and methicillin-resistant staphylococcus aureus.
Correct answer: Rectal biopsy

Explanation Hirschprung's disease is the possible diagnosis in this child. It is a genetic disorder named after Harold Hirschsprung, the Dutch physician who first described the disease in 1886. The diagnosis of Hirschsprung's disease is based on the absence of parasympathetic ganglion cells in the submucosal and myenteric plexuses in the affected segment of the large intestine.
Diagnosis of Hirschsprung's disease was formerly established by means of a full thickness biopsy, a procedure requiring general anaesthesia. The development of rectal suction biopsy techniques has greatly reduced the patient risk but has given diagnostic difficulties to the pathologist because they contained few or no ganglion cells. Also, the immature ganglion cells of the neonates could be confused with other cells such as macrophages, fibroblasts, and Schwann cells. The acetylcholine esterase (AchE) enzyme stains have diagnostic value but require frozen section, and results are difficult to interpret. Recent studies have shown that immunoperoxidase technique with antibodies against various nervous system proteins could prove as a potential aid in the diagnosis of Hirschsprung's disease. They can be used to demonstrate the hyperplastic nerve fibres and the ganglion cells for diagnostic value in cases suspected of Hirschsprung's disease.

To diagnosis Hirschsprung's disease, a barium enema x-ray test is used to identify the narrow collapsed segment of bowel in front of the affected regions. In an infant younger than 3 months of age, or in any child with a very short or very long collapsed portion of bowel, the x-ray may appear normal despite the presence of Hirschsprung's disease. A biopsy, or tissue sample, from the part of the bowel an inch above the anus is then necessary to confirm the absence of ganglia. In cooperative children, a painless test called anorectal manometry may complement the rectal biopsy result.

If Hirschsprung's disease is not recognized in the neonatal period, the affected infant may present with symptoms such as abdominal distension, pencil-thin stools, failure to thrive, and bilious vomiting. If an infant has any of these symptoms, and the physical examination shows an empty rectum, Hirschsprung's disease should be suspected. A delay in diagnosing this disease places the infant at risk for enterocolitis, with fever, explosive bloody diarrhea, and abdominal distension, in the second or third month of life.

Treating Hirschsprung's disease requires surgery to remove the affected bowel and then joining the healthy bowel segments.

For most children with Hirschsprung's disease, there are no long-term complications after successful surgery. A significant minority of children, though, are troubled with persistent constipation, encopresis (stool incontinence), or persistent enterocolitis.
Correct answer: Meckel's diverticulum

The Meckel diverticulum appears to be a remnant of developmental structures that were not fully reabsorbed. It is a common congenital abnormality that consists of a small pouch called a diverticulum located off the wall of the small bowel. Symptoms generally occur during the 1st few years of life. Symptoms include passing of blood either with or without stool and abdominal discomfort ranging from mild to severe. Tests should include stool smear for occult blood (stool guaiac), hematocrit, hemoglobin, and technetium scan to demonstrate diverticulum. Surgery to remove the diverticulum is recommended if bleeding develops. Iron replacement may be needed to correct anemia. If bleeding is significant, blood transfusion may be necessary.

Diverticulitis is inflammation of an abnormal pouch (diverticulum) in the intestinal wall, usually found in the large intestine (colon). Small protruding sacs of the inner lining of the intestine (diverticulosis) may occur in any part of the intestine. They occur with increasing frequency after the age of 40. Diverticulitis is an inflammatory condition where gross or microscopic perforation (hole) of the diverticula has occurred. A low-fiber diet may be a contributing factor to the development of diverticula. Symptoms include left lower abdominal pain, constipation or diarrhea, chills, fever, swallowing difficulty, stools (clay colored or bloody), nausea and vomiting, heartburn, cough, and breath odor. Tests should include colonoscopy, sigmoidoscopy, barium enema, rectal examination (showing bleeding), abdominal palpation (showing left lower quadrant mass), and stool hemoccult test (revealing blood). Treatment should include increasing the bulk in the diet with high-fiber foods and bulk additives.

Peutz-Jeghers syndrome is transmitted as an autosomal dominant trait. Pigmented spots, brownish or bluish gray, develop from infancy through childhood around the lips, gums, and mucus membranes in the mouth. Symptoms include crampy abdominal pain, vomiting, occasional gross blood in the stool, and intussusception (a telescoping of one portion of the intestine into another). Intestinal polyps also develop that can be detected with special studies. Diagnostic tests include X-ray of abdomen (shows polyposis), occult blood in stool, CBC, serum iron, serum, total iron binding capacity, and biopsy of polyps. Treatment includes surgery to remove polyps that cause chronic problems. Iron replacement therapy (iron supplements) helps counteract blood loss. Periodic studies are recommended to watch for malignant changes in polyps.

Crohn's disease (also called ileitis or enteritis) causes inflammation in the small intestine. The most common symptoms of Crohn's disease are abdominal pain (often in the lower right area) and diarrhea. Rectal bleeding, weight loss, decreased appetite, and fever may also occur. Bleeding may be serious and persistent, leading to anemia. Tests should include CBC (increased white count), upper GI, and colonoscopy. The goals of treatment are to control inflammation, relieve symptoms, and correct nutritional deficiencies. Surgery is indicated to relieve chronic symptoms that do not respond to treatment.

Ulcerative colitis is chronic, episodic, inflammatory disease of the large intestine and rectum characterized by bloody diarrhea. Ulcers form in the inner lining, or mucosa, of the colon or rectum, often resulting in diarrhea, blood, and pus. The inflammation is usually most severe in the sigmoid and rectum and usually diminishes higher in the colon. It may affect any age group, although there are peaks at ages 15 to 30 and then again at ages 50 to 70. Symptoms include diarrhea of between 10 and 25 times a day (in which blood and pus may be present), abdominal pain and cramping that usually subsides after a bowel movement, abdominal sounds (borborygmus, a gurgling or splashing sound heard over the intestine), fever, weight loss, stools (foul smelling), and tenesmus (pain while passing stool). Tests include colonoscopy with biopsy, and barium enema. The goals of treatment are to control the acute attacks and prevent recurrent attacks. Corticosteroids are prescribed to reduce inflammation. Sulfasalazine may decrease the frequency of attacks. Surgery may be indicated in refractory disease.
Correct answer: Niacin

This patient probably has niacin deficiency. Niacin (nicotinamide, nicotinic acid) deficiency is uncommon in the United States. It is often found in people who live on a diet that consists mainly of corn (maize). This is due to the fact that the niacin in corn cannot be absorbed unless it is chemically treated with alkali first. If a person consumes a diet rich in tryptophan, but low in niacin, they are able to compensate since tryptophan can be converted into niacin. Deficiency may also result from alcoholism, cirrhosis, or diarrhea. Men and women are affected equally. Symptoms of niacin deficiency include nausea, vomiting, diarrhea, rash, glossitis, stomatitis, depression, and psychosis. Niacin deficiency, also known as pellagra, manifests as the '3 Ds': diarrhea, dermatitis, and dementia. Treatment consists of niacin supplementation.

The causes of zinc deficiency include malnutrition, chronic debilitating diseases, chronic renal disease, alcoholism, drugs such as penicillamine and diuretic, and genetic disorders, such as sickle cell disease. Clinical manifestations in severe cases include alopecia, diarrhea, weight loss, infections, dermatitis, hypogonadism in men, and intercurrent infections. Supplementation with zinc is the treatment of choice.

Folate deficiency causes megaloblastic macrocytic anemia, as folate plays a key role in nucleic acid synthesis. The early manifestation of folate deficiency, especially in its suboptimal state, predisposes to occlusive vascular disease and thrombosis. These manifestations are linked to increased homocysteine levels found in folate deficiency. Neurological disturbances, such as mood disturbance and spinal cord syndromes, are also seen. It is also associated with predisposition to neoplasia and interferes with immunologic status. Folate replacement is the option to prevent and to treat the deficiency.

Vitamin B1 (or thiamine deficiency) causes beri beri, occurring mostly in the malnourished and alcoholics. The deficiency manifests with acute heart failure, neurologic deficits, and epilepsy. Empiric use of thiamine and prophylactic use in high-risk population is strongly recommended even before blood reports are obtained, as the treatment is inexpensive and prevents major catastrophes.

Vitamin B12 (or cobalamin deficiency) manifests as megaloblastic macrocytic anemia, pancytopenia, and a spectrum of neuropsychiatric disorders such as peripheral neuropathy, parasthesias, and demyelination of corticospinal tract. Nutritional deficiency, alcoholism, and malabsorption syndromes are some causes of B12 deficiency. It is also associated with homocysteinemia and atherosclerosis. Diagnosis is by serum estimation of B12, and oral supplementation is safe and effective. Intramuscular injections may also be used.
Correct answer: Endoscopically insert a biliary stent

Pancreatic cancer is more common in men (1.2 to 1.5 male-to-female ratio). The disease is rare before the age of 50 years, and the median age of diagnosis is 69 in whites and 65 in blacks. Risk factors include smoking (heavy smokers have a two to threefold higher incidence), chronic pancreatitis, long-standing diabetes, family history, and obesity, The most common signs and symptoms are jaundice (in obstructive lesions), weight loss, and abdominal pain. More rarely, diabetes mellitus, splenomegaly, a palpable gallbladder (Courvoisier's sign), and migratory thrombophlebitis (Trousseau's sign) are seen. The delayed diagnosis explains the disease's 98% lethality rate.

Several imaging modalities (e.g., transabdominal ultrasound, computed tomography, magnetic resonance imaging, endoscopic ultrasound, and endoscopic retrograde cholangiopancreatography [ERCP]) can visualize these tumors, but the vast majority of them are diagnosed late in their course because symptoms only occur in advanced disease stages.

This patient has unresectable, stage IV pancreatic cancer (see pancreatic cancer staging) and biliary obstruction. Treatment is palliative, and the initial goal is to re-establish patency of the biliary tract. Several approaches are possible: endoscopic stenting using endoscopic retrograde cholangiopancreatography, percutaneous drain placement, and operative redirection of the bile ducts. Endoscopy is usually the preferred method. Several techniques are available, including plastic and expandable metal stents, lasers, and photodynamic therapy.

Duodenopancreatectomy consists of removal of the pancreatic head, duodenal arch, gallbladder, and gastric antrum. Reconstruction is performed by anastomosing the remaining jejunal loop end-to-end to the pancreatic remnant, a biliary-enteric anastomosis, and a Billroth-II gastric reconstruction. It's the standard procedure for pancreatic cancer resection with curative intent. However, this patient has unresectable disease.

Gemcitabine affords symptomatic improvement but improves survival only slightly. While it might be useful in this patient, establishing biliary drainage takes precedence over chemotherapy.

Radiotherapy is sometimes useful for palliation of pain. However, pancreatic cancer is relatively radioresistant, and survival is not improved.
Correct answer: Meckel diverticulum

This patient is most likely suffering from a common congenital abnormality of the development of the ileum called a Meckel's diverticulum. In the embryo, the vitelline duct is a communication between the yolk sac and the lumen of the gastrointestinal tract at the midgut. Normally, it degenerates completely, but the persistence of a portion of the vitelline duct leads to the development of a cul-de-sac on the ileum (Meckel's diverticulum). This congenital birth defect follows a rule of 2s. It occurs in 2% of the population, but only 2% show symptoms. It is usually located about 2 feet from the ileocecal valve and is about 2 inches long. It can contain 2 types of ectopic tissue: gastric or pancreatic. It usually presents by 2 years old. The presence of ectopic gastric mucosa in the diverticulum can lead to secretion of stomach acid downstream from the duodenum, which has bicarbonate-secreting submucosal Brunner glands to neutralize gastric acid. Ectopic gastric tissue in the ileal diverticulum can lead to ulceration and bleeding of the adjacent ileal mucosa.

Vascular malformations, intussusception, and coagulation disorders are more likely to manifest themselves in the first year of life. Cow's milk colitis is a problem of the first year of life and spontaneously resolves by the end of that year for most children. NEC is a problem of the stressed, usually premature, newborn. Rectal polyps are likely to present in older children, as is HSP. Babies and older children can have gastric bleeding from gastritis or gastric ulcers. Duodenal ulcers are much more common in older children.
Correct answer:
Prevent constipation

Explanation This patient is already in the initial phases of hepatic encephalopathy due to alcoholic liver disease as evidenced by the disorientation and tremors, also known as asterixis. The primary cause of hepatic encephalopathy is unclear. Metabolic abnormalities due to liver dysfunction, resulting in a spectrum of neuropsychiatric signs and symptoms, are seen. High levels of ammonia are found in the blood. Constipation causes increased ammonia production and absorption due to prolonged intestinal contact and aggravates the condition. Lactulose should be administered frequently to eliminate the ammonia in the stool. It is an indigestible sugar that acts as an osmotic laxative by increasing the water content of the stool and promoting bowel movements. It is digested by the colonic bacteria, and the acidic remains convert ammonia into ammonium ions in the colon, which are then excreted in the stool. Oral antibiotics can lower blood ammonia levels by decreasing ammonia production and absorption. The commonly used ones are neomycin, metronidazole, vancomycin, and, lately, rifaximin. The last three are better tolerated than neomycin. However antibiotics have their side effects and can cause bacterial overgrowth syndromes. Their main use continues to be in patients who cannot tolerate disaccharides like lactulose. Acarbose and fermentable fiber can also cause decrease in intestinal ammonia production and absorption. Newer studies with sodium benzoate are ongoing. Benzoate and glycine react to form hippurate, and for every mole of benzoate utilized this way, one mole of nitrogen is excreted in the urine, thereby enhancing ammonia metabolism. This, however, still needs to be studied further to be used widely. Studies with ornithine-aspartate are also being done as a stimulator of ammonia metabolism. All the products mentioned are yet to replace lactulose as the first line of treatment but are potentially useful once more studies are done. Other precipitating factors for hepatic encephalopathy include azotemia, hypokalemia, gastrointestinal bleeding, high protein diet, alkalosis, infection, sedatives, and other hepatotoxic agents.
High protein diet is a contraindication in this condition, as protein catabolism causes increase in ammonia levels. Daily protein should be restricted to 40 g/day.

Sedation of the patient should also be avoided, since sedatives cause cerebral depression and worsening of encephalopathy. These drugs are also not metabolized adequately by the diseased liver.

Though infection is an important precipitating factor for hepatic encephalopathy, empiric treatment is not recommended. However, early and adequate treatment of an infection should be done, especially for spontaneous bacterial peritonitis. In fact multiple randomized control trials have been done regarding antibiotic prophylaxis for SBP, and they have shown not only a decrease in bacterial infections but also a significant reduction in mortality. Prophylaxis is recommended in cirrhotic patients with risk factors for SBP like GI bleeding, prior history of SBP, and low ascitic fluid protein.

Thiamine and folic acid should be added for nutritional support to all alcoholic patients, since they are malnourished and vitamin depleted. However, this will not change the outcome in hepatic encephalopathy as quickly or as much as avoiding constipation.
Correct answer:
Whipple disease

Explanation
Whipple disease is a rare condition that can involve any organ of the body but principally affects the intestine, central nervous system, and joints. It is caused by gram-positive actinomycetes called Tropheryma whippelii. Histologically, the small intestinal mucosa is laden with distended macrophages in the lamina propria. These macrophages contain periodic acid-Schiff positive granules and rod-shaped bacilli by electron microscopy. These patients usually present with a form of malabsorption, including diarrhea and weight loss, and generally respond to antibiotic therapy.

Celiac sprue is a rare chronic disease in which there is a characteristic mucosal lesion of the small intestine in the form of blunting of the villi and an overall increase in plasma cells, lymphocytes, macrophages, and eosinophils in the lamina propria. Also known as the gluten-sensitive enteropathy, non-tropical sprue, and celiac disease, it is due to sensitivity to gluten, which is an alcohol soluble, water insoluble protein component called gliadin of wheat and other closely related grains. Detection of circulating anti-gliadin or anti-endomysial antibodies strongly favors the diagnosis. Both the symptoms and the mucosal histology improve on withdrawal of gluten from the diet.

Tropical sprue is a celiac-like disease occurring in people living or visiting the tropics. Bacterial overgrowth by an enterotoxigenic organism is said to be the cause for this disease. Intestinal changes are similar to celiac sprue, but injury is seen at all levels of the small intestine in contrast to celiac sprue, where it is concentrated in the proximal small intestine. It is mainly treated by broad-spectrum antibiotics.

The disaccharidases, of which the most important is lactase, are located in the apical cell membrane of the villous absorptive epithelial cells, the deficiency of which leads to the incomplete breakdown of the disaccharide lactose into its monosaccharides, glucose, and galactose, leading to diarrhea from the unabsorbed lactose. Bacterial fermentation of the unabsorbed sugars leads to increased hydrogen production, which is readily measured in the exhaled air by gas chromatography. There are both hereditary and acquired forms. Histologically in both, there is no abnormality of the mucosal cells of the bowel. Malabsorption is promptly corrected when exposure to milk and milk products is terminated.

Amebic dysentery is caused by Entamoeba histolytica, a protozoan, and generally causes diarrhea with blood and mucus. A stool examination will show the cysts and trophozoites of Entamoeba histolytica, which is diagnostic.
Correct answer: Whipple disease

Whipple disease is a rare condition that can involve any organ of the body but principally affects the intestine, central nervous system, and joints. It is caused by gram-positive actinomycetes called Tropheryma whippelii. Histologically, the small intestinal mucosa is laden with distended macrophages in the lamina propria. These macrophages contain periodic acid-Schiff positive granules and rod-shaped bacilli by electron microscopy. These patients usually present with a form of malabsorption, including diarrhea and weight loss, and generally respond to antibiotic therapy.

Celiac sprue is a rare chronic disease in which there is a characteristic mucosal lesion of the small intestine in the form of blunting of the villi and an overall increase in plasma cells, lymphocytes, macrophages, and eosinophils in the lamina propria. Also known as the gluten-sensitive enteropathy, non-tropical sprue, and celiac disease, it is due to sensitivity to gluten, which is an alcohol soluble, water insoluble protein component called gliadin of wheat and other closely related grains. Detection of circulating anti-gliadin or anti-endomysial antibodies strongly favors the diagnosis. Both the symptoms and the mucosal histology improve on withdrawal of gluten from the diet.

Tropical sprue is a celiac-like disease occurring in people living or visiting the tropics. Bacterial overgrowth by an enterotoxigenic organism is said to be the cause for this disease. Intestinal changes are similar to celiac sprue, but injury is seen at all levels of the small intestine in contrast to celiac sprue, where it is concentrated in the proximal small intestine. It is mainly treated by broad-spectrum antibiotics.

The disaccharidases, of which the most important is lactase, are located in the apical cell membrane of the villous absorptive epithelial cells, the deficiency of which leads to the incomplete breakdown of the disaccharide lactose into its monosaccharides, glucose, and galactose, leading to diarrhea from the unabsorbed lactose. Bacterial fermentation of the unabsorbed sugars leads to increased hydrogen production, which is readily measured in the exhaled air by gas chromatography. There are both hereditary and acquired forms. Histologically in both, there is no abnormality of the mucosal cells of the bowel. Malabsorption is promptly corrected when exposure to milk and milk products is terminated.

Amebic dysentery is caused by Entamoeba histolytica, a protozoan, and generally causes diarrhea with blood and mucus. A stool examination will show the cysts and trophozoites of Entamoeba histolytica, which is diagnostic.
Correct answer: Immunosuppressant (systemic corticosteroids)

Your patient most probably has Pyoderma gangrenosum, which is a ulcerative skin lesion of an uncertain etiology; in more than 50% of cases, it is associated with systemic diseases, most commonly inflammatory bowel disease. It usually develops rapidly and can progress from a pimple to an ulcer in 1 or 2 days. In a process termed pathergy, new ulcerations may occur after trauma or injury to the skin. Pain is the predominant symptom, but symmetrical arthritis, myalgias, and malaise are also common. When the lesions heal, they usually leave the scars that are often cribriform. Immunosuppression is the mainstay of treatment; it is believed that dysregulation of the immune system (specifically, altered neutrophil chemotaxis) is involved. Most clinicians use both topical and systemic therapy. The most commonly used treatments include topical potent corticosteroids or tacrolimus to treat early lesions; systemic corticosteroids, TNF-α inhibitors, or other anti-inflammatories or immunosuppressants are used to treat more severe manifestations.

Surgery or debridement is contraindicated because of the presence of pathergy. Even if there was no pathergy, surgery is contraindicated because skin trauma can trigger the pathergy.

Acyclovir is not indicated; the clinical picture is not consistent with acute herpes infection and the patient is not immunocompromised.

Fluconazole is not indicated; the clinical picture is not consistent with tinea (or any other fungal infection).

Paracetamol alone probably will not relieve the debilitating pain. Sufficient pain medication in this case will probably include paracetamol and some other medication, sometimes opioids.
A 38-year-old man presents with sudden onset of acute upper abdominal pain since the previous night associated with nausea, several episodes of vomiting, and weakness. The pain is mostly in the epigastric region with constant, severe, and steady radiation to the back. He also has a low grade fever since this morning without any chills. He denies diarrhea or dysuria. His past history is significant for hypertension, for which he takes amlodipine 10 mg daily and enalapril 5 mg daily. He has smoked half a pack of cigarettes daily for the past 12 years. He initially denies drinking alcohol except on the weekends occasionally, but on further and repeated questioning he says he drinks 3-4 beers daily and had been drinking continuously for the last 2 days with his friends while watching sports on TV. Family history is unremarkable.
On examination he has a temperature of 100.6° F, pulse 106/minute, BP 150/92 mm Hg, and respiratory rate is 20/minute. There is no pallor, icterus, cyanosis, or lymphadenopathy. Mucus membranes are dry, and skin is somewhat clammy. Lungs are clear, and heart sounds normal except for sinus tachycardia. Abdominal exam reveals diffuse tenderness in the epigastric and right as well as left upper quadrants. There is some distension and mild guarding in the upper abdomen. Bowel sounds are hypoactive, but there is no ascites or hepatosplenomegaly. Rectal exam is normal. Labs reveal Hb 15g%, WBC 14,500/uL, platelets 400,000/uL, AST 42 U/L, ALT 36 U/L, AP 26 U/L, amylase 3600 U/L, lipase 546 U/L, BUN 25 mg/dL, creatinine, 1.5 mg/dL, bilirubin 1.2 mg/dL, and random blood sugar 110 mg/dL.

Which of the following is one of the predictors of acute pancreatic necrosis if present at diagnosis along with 2 other factors?

1. Age over 50 years
2. WBC count more than 16,000/uL
3. Blood glucose over 180mg/dL
4. Serum LDH over 300 U/L
5. AST more than 200 U/L
Correct answer: WBC count more than 16,000/uL

This patient is suffering from acute alcoholic pancreatitis. The 2 most common causes of acute pancreatitis are gallstones and alcohol. Other causes include hypertriglyceridemia, hypercalcemia, abdominal trauma, ERCP, and drugs like valproic acid, azathioprine, mercaptopurine, didanosine, thiazides, tetracyclines etc. Assessment of severity is done by either Ranson's criteria on admission and at 48 hours or by the Acute Physiology and Chronic Health (APACHE) II scoring system. Ranson's criteria include:
(1) Age more that 55 years
(2) WBC count more than 16,000/Ul
(3) Blood glucose more than 200mg/dL
(4) Serum LDH over 350 U/L
(5) AST over 250 U/L.

3 or more criteria on admission predict a complicated course with possibility of pancreatic necrosis.

At 48 hours development of any of the following indicates a worsening prognosis:

(1) Hematocrit drop of more than 10%
(2) BUN rise greater than 5 mg/dL
(3) Arterial PO2 less than 60 mm hg
(4) Serum calcium less than 8 mg/dL
(5) Base deficit over 4 meq/L
(6) Estimated fluid sequestration of more than 6 L.

An elevated C reactive protein at 48 hours suggests the development of pancreatic necrosis. A high amylase and lipase are suggestive of acute pancreatitis. Leukocytosis is usually present.

Other than the labs ordered above, imaging may also be done. Plain abdominal X-ray may show radio-opaque gallstones, the sentinel loop sign (localized ileus of a small segment of small intestine, usually in the left upper quadrant), and the colon cut-off sign (lack of air in the colon in the area of the inflamed pancreas immediately preceded by a gas filled segment of transverse colon). There may be a reactive pleural effusion with atelectasis in the lower lobes of the lungs. Ultrasound is non specific and may show gallstones. CT scan of the abdomen will show the inflamed pancreas and detect complications like necrosis or pseudocyst formation. Since Ranson's criteria and APACHE scoring systems are cumbersome and time consuming, a CT Severity Index (CTSI) has recently become popular. It uses a grading system based on unenhanced CT of the pancreas and a necrosis score based on contrast enhanced CT of the pancreas. CTSI is the unenhanced score plus the necrosis score, the maximum of which can be 10 and more than 6 indicates severe disease. MRI and MRCP are being increasingly used where available for diagnosis and management of pancreatitis.

Treatment involves bowel rest, aggressive hydration, pain control, and bed rest in a hospital. Electrolyte imbalances should be corrected, especially calcium since saponification may lower the level. Broad spectrum antibiotics are not routinely recommended, but in patients with impending necrotizing pancreatitis, antibiotics have shown to decrease mortality. Nutritional support is of utmost importance. For mild to moderate cases, IV hydration and gentle advancement of oral feeding is recommended, where as in severe cases total parenteral nutrition may be needed. Alcohol abstinence will be needed to prevent further episodes. Complications include prerenal azotemia, acute tubular necrosis, shock, pancreatic necrosis, pseudocyst formation, ARDS, and pancreatic abscess. Intra-abdominal hemorrhage may cause ecchymoses around the umbilicus (Cullen's sign) or in the flanks (Grey-Turner sign), though these are rare and not specific for pancreatitis. Prognosis is good for mild cases who abstain from drinking. Recurrences are common in alcoholics. Prognosis is poor for severe necrotizing pancreatitis, especially with multi-organ involvement.
Correct answer: Acetaminophen

Patients classified as having mild or intermittent symptoms of esophagitis and/or gastroesophageal reflux disease (GERD) typically are seen as not adversely affecting the patient's quality of life. Initially action is taken in terms of modification of behaviors, such as eating smaller meals and eliminating acidic foods or known foods that precipitate the reflux (fatty foods, alcohol, chocolate, or peppermint). The next step in terms of treatment would be to discontinue any medications that may be increasing the symptoms of GERD. Medications that potentially irritate the esophagus and have a high incidence of causing pill-induced esophagitis include: tetracycline, bisphosphonates, iron supplements, Non-Steroidal Anti-inflammatories (NSAIDS), and potassium supplements. Medications that can increase acid reflux and worsen the condition of GERD include: anticholinergics, calcium channel blockers, narcotics, progesterone, quinidine, benzodiazepines, or even theophylline. A proton pump inhibitor would be initiated in a patient who has moderate to severe esophagitis or gastroesophageal reflux symptoms; this patient does not fit this picture.

Since this patient is currently choosing ibuprofen chronically for a recent onset of headaches, the appropriate instruction would be to discontinue the ibuprofen regimen and begin an acetaminophen regimen in its place. Antibiotics would be an incorrect answer. H1 receptor antagonists, or H1-antihistamines, are an inappropriate choice because these are used to treat the symptoms of allergies (seasonal and perennial). The mechanism of action of anticholinergics does not make it an indication for this type of medical complaint.
Correct answer: A 14% decrease of his hematocrit at 48 hours after admission

A patient who is admitted to the hospital for treatment of acute pancreatitis is assessed using the Ranson criteria to determine the severity of their disease, which in turn helps determine prognosis. About 70 - 80% of cases of acute pancreatitis are considered mild and result in virtually no morbidity or mortality. The remainder are severe attacks and have a 10 - 30% mortality rate. Fast identification of the severe cases is helpful to reduce the morbidity and mortality for each patient. There are 11 Ranson criteria, 5 of which are determined upon admission, and 6 at 48 hours after admission. Patients who have 2 or fewer of the criteria have minimal mortality. Patients with 3 - 5 of the criteria have about a 10 - 20% chance of mortality. Patients with 5 or more of the criteria have at least a 50% mortality rate. A patient with at least a 10% decrease in their hematocrit at 48 hours after admission meets one of the Ranson criteria, so the correct answer is a 14% decrease in hematocrit 48 hours after admission.

WBC count of 10,000 cells/mm3 is not one of the Ranson criteria. A WBC count of >16,000 cells/mm3 would meet one of the Ranson criteria.

Serum glucose of 130 mg/dL upon admission is also not one of the Ranson criteria. The patient's serum glucose would have to be at least 200 mg/dL to count as one of the Ranson criteria and increase the severity of their disease.

Aspartate transaminase of 240 U/dL upon admission is not one of the Ranson criteria either. The patient's aspartate transaminase would have to be at least 250 U/dL to count as a Ranson criteria and increase the severity of their disease.

A serum calcium of 10 mg/dL 48 hours after admission is not one of the Ranson criteria. If the patient's serum calcium is under 8 mg/dL 58 hours after admission, this would count as one of the Ranson criteria.
Correct answer: Barium enema

The baby probably has Hirschsprung's disease. It is a congenital motility disorder of the colon caused by lack of innervation in a short segment of the colon, usually the rectosigmoid. This results in failure of relaxation of that part and functional obstruction. Failure of passage of meconium in the first 48 hours is suggestive. If not treated quickly, sometimes eneterocolitis can occur with fever, sepsis, and life threatening toxic megacolon, as is the case in this patient. Barium enema shows a narrowed segment with a dilated proximal colon. Rectal biopsy is the gold standard and will reveal absence of ganglion cells in the affected segment. Treatment is surgical. The goal is to resect the affected segment, bring down normal ganglionic bowel up to the anus, and preserve sphincter function. It may be done as 2-step procedure with an initial diverting colostomy followed by definitive repair or a single stage repair. It should be done soon after diagnosis, often within the first few days or months of diagnosis. Emergency surgery is indicated in Hirschsprung-associated eneterocolitis (HAEC), which is the most lethal complication. Volume resuscitation, intravenous antibiotics, and rectal irrigation are the supportive measures.

Ultrasound of the abdomen will be non-diagnostic and is not indicated.

Anal manometry is a good screening test for this condition but is less accurate in infants less than 1 month old. It demonstrates lack of relaxation of the internal anal sphincter on rectal distension.

Endoscopy is not indicated as visualization is adequate with barium enema and rectal biopsy is done by a mucosal suction technique.

Blood cultures may be done in a septic baby for antibiotics, but it is non diagnostic for the cause of the obstruction.
A 38-year-old man presents with a 24-hour history of fever and severe abdominal pain. He has had 10 loose stools that were bloody and contained mucus; they were associated with abdominal distension. He denies any contact with sick individuals, or eating any unusual meals. He denies any recent use of antibiotics. His past medical history is significant for ulcerative colitis since the age of 30. His medication includes mesalamine and acetaminophen. He smokes about 5 - 10 cigarettes each day, and he drinks small amounts of alcohol on occasion.

Upon examination, he looks ill and dehydrated. His BP is 130/80 mm Hg, pulse is 120 BPM, temperature is 38.3°C, and RR is 20/min. His abdomen is distended and tender, but without guarding or rigidity; his bowel sounds are decreased. Chest auscultation is clear, with good bilateral air entry. Cardiac sounds are normal, but his pulse is tachycardic.

His lab results are:

Serum glucose 66 mg/dL
Sodium 130 mmol/L
Potassium 3.1 mmol/L
Chloride 99 mmol/L
Bicarbonate 19 mmol/L
BUN 25 mg/dL
Creatinine 0.7 mg/dL
Calcium 11.0 mg/dL
Leukocytes count 13,800/μL
Segmented neutrophils 95%
Hemoglobin 8.1 g/dL
Hematocrit 24.2%
Platelet count 245,000/μL
Supine plain radiographs of the abdomen show moderate dilation of the colon with loss of haustration in the descending colon, along with thumbprinting in the region of the splenic flexure of the colon.

Because of the patient's condition and test results, what action is contraindicated?

1 Barium enema
2. CT abdomen
3. Steroids
4. Metronidazole
5. Cyclosporine
Correct answer: Cholangitis

Cholangitis is the most common cause of liver abscesses. Biliary pathologies are the most common predisposing cause of liver abscesses and account for approximately 60% of the cases.

The etiological classification of a bacterial abscess of the liver (pyogenic liver abscess) is made on the basis of the route by which the infection reaches the liver. There are 5 mechanisms:

Portal bacteremia (via the portal vein) from an intra-abdominal site (e.g., diverticulitis, suppurative appendicitis, infected carcinoma of the colon especially after resection, ulcerative colitis, inflamed hemorrhoids, typhoid, and paratyphoid fever)
Along the bile ducts, causing ascending cholangitis in a biliary tract partially or completely obstructed by stone, tumor, or stricture
Systemic bacteremia originating in a distant location with organisms reaching the liver via the hepatic artery; causes include septicemia and pyemia, endocarditis, pyelonephritis, and infection of the hydatid cyst
Direct extension from an adjacent infection outside the biliary tract (e.g., from a sub-diaphragmatic abscess, from an empyema thoracis, and from trauma, either penetrating with direct implantation of bacteria into the liver, or blunt causing a hematoma that becomes secondarily infected
From the umbilicus along the umbilical vein of the newborn and along the paraumbilical veins
A cause is usually obvious, but sometimes the abscess is unexplained. Although most abscesses are single, multiple (usually microscopic) abscesses are common with systemic bacteremia or complete biliary tract obstruction. Prompt antibiotic treatment, with agents such as meropenem, imipenem, and cefuroxime, followed by percutaneous drainage, form the essential components of treatment.

Biliary tract diseases account for about 60% of pyogenic liver abscesses. Systemic bacteremia accounts for 15% of the cases. About 1/4 can be attributed to pylephlebitis, an inflammation of the portal vein or any of its branches.

Direct extension from an adjacent infection, such as a subphrenic abscess, account for <5% of cases.
Correct answer: Rapid antibody test

An office-based rapid antibody test is still considered the most cost effective mode of diagnosis of infection with H. pylori, especially in a patient not treated in the past for the same. This test is simple, inexpensive, and sensitive, as well as specific. However, it is not useful as a follow-up test to confirm eradication of infection. Other tests include stool antigen testing, which is also inexpensive, noninvasive, highly sensitive, and specific, and can be used as a follow-up test as well. This infection is common in the general population. It is the cause of 50 - 80% of peptic ulcers, and incidence of ulcer disease in those with the infection is 20%. Testing is indicated in active or documented past history of peptic ulcer disease and gastric mucosa associated lymphoid tissue (MALT) lymphoma. Many physicians test patients with functional dyspepsia, as this patient, though it is still controversial. However, if testing is done and it is positive, treatment with triple therapy must be done.

Referral to a specialist for endoscopy is indicated in patients with known peptic ulcer disease, in suspected MALT lymphoma, and follow-up of ulcers. Endoscopic based diagnostic testing of H.pylori should only be done if endoscopy is indicated for other reasons.

Empiric treatment for H.pylori should never be done without testing.

Urea breath test is recommended at least 4 weeks after treatment and is the test of choice to confirm eradication of infection.

Fecal antigen test is done in a lab and delays the diagnosis, so it is recommended to confirm eradication of the infection 4 weeks after treatment.
Correct answer: Refer for emergency upper endoscopy
kk

This patient is most likely bleeding from a gastric ulcer. His recent NSAID use, as well as his alcohol and tobacco habits, make him at risk for peptic ulcer disease. His symptoms of melena and hematemesis, along with his anemia, make the diagnosis quite straightforward.

It appears that this patient is still actively bleeding based on the results of the nasogastric tube irrigation; therefore, the priority should be getting the ulcer to stop bleeding. Upper endoscopy should be performed so that the bleeding site can be identified and treated with electrocautery, coagulation, or injection of epinephrine or a sclerosing agent. If the bleeding cannot be stopped with endoscopic interventions, angiographic embolization should also be tried. If these interventions do not succeed, the patient has rapid deterioration, or if he requires more than 6 units of blood in a 24-hour period, then emergency surgery may be indicated.

The other choices are not the best options for immediate management. This individual cannot be followed simply with transfusions and serial CBC's because he appears to still be actively bleeding.

Helicobacter pylori infection may very well be playing a part in the etiology of this man's ulcer, but evaluation for H. pylori can be done with a biopsy at the time of his endoscopy; it will not help in his immediate management.

A barium esophagram will not identify actively bleeding ulcers and cannot treat active bleeding.

While NSAID, alcohol, and tobacco use may have precipitated this man's GI bleed, counseling about his use of these substances will not sufficiently treat his immediate bleed.
A 44-year-old woman is referred to a hospital because of occasional headache accompanied by irritability, confusion, sweating, and hunger. She states that symptoms appeared approximately 3 months ago; they frequently tend to appear early in the morning before breakfast or between meals. On one occasion, during the crisis period, a decreased blood glucose level (55 mg/dL) was detected despite the fact that she consumes a diet rich in glucose. She admits to excessive abuse of alcohol, and she occasionally used aspirin for headaches up to 6 days before admission. Her medical records suggest she was previously treated as a psychopath who was prone to malingering. The patient herself confirms that she was haloperidol-treated for more than 1 year, but she discontinued haloperidol use 2 weeks ago.

Her general physical findings are unremarkable. Routine laboratory tests taken on admission reveal no abnormalities. During echosonographic examination of abdomen, no abnormalities were noted. Fasting test was attempted, but it had to be discontinued due to a fall in blood glucose level from baseline value of 75 to 33 mg/dL 8 hours later. It required intravenous glucose administration and termination of the test. Laboratory analyses taken at the time of test termination also revealed elevated serum insulin (9 uU/ml), elevated serum proinsulin (6.3 pmol/l), and elevated C peptide (0.3 mmol/ml) levels. Insulin receptor antibodies are not present in the patient's serum, and sulfonylurea is absent in the patient's sera and urine.

What disorder is the most likely cause of patient's hypoglycemia?

Intake of aspirin
2. Chronic alcoholism
3. Insulin-producing tumor
4. Deliberate insulin intake
5. Haloperidol therapy
Correct answer: Insulin-producing tumor

An insulin producing tumor (insulinoma) is characterized by an increased endogenous production of insulin by insulin-secreting tumor. Since a proportion of insulin is released into the blood in the form of proinsulin, proinsulin production is also increased in patients with insulinoma. Also, proinsulin production is increased in patients with liver cirrhosis or diabetes mellitus type II. Therefore, an increased proinsulin level in a hypoglycemic patient who has no liver cirrhosis indicates hypoglycemia is caused by increased production of endogenous insulin.

Deliberate insulin intake causes hypoglycemia. However, if hypoglycemia is caused by intake of exogenous insulin, serum proinsulin level tends to be normal or low. Also, serum level of C peptide that is produced by insulin-secreting cells together with insulin is increased in patients with insulinoma. C peptide level is, however, decreased if hypoglycemia is caused by exogenous insulin administration. Since the patient has both elevated proinsulin and C peptide levels, it is unlikely that hypoglycemia is caused by deliberate patient's administration of insulin.

Chronic alcohol intake may cause hypoglycemia by decreasing glucose release from the liver. However, it is not likely that patient's hypoglycemia can be attributed to alcohol intake because her diet was rich in glucose. Also, elevated serum levels of insulin, proinsulin, and C peptide, as well as high serum insulin/glucose ratio (9/33=0.33), point to the existence of insulinoma.

Intake of aspirin may cause hypoglycemia. However, after discontinuation of aspirin intake, hypoglycemia is readily corrected. Hypoglycemia that persists 6 days after discontinuation of aspirin therapy cannot be attributed to aspirin intake.

In addition to aspirin, haloperidol therapy may cause hypoglycemia that disappears after haloperidol withdrawal. The hypoglycemia in the presented patient appeared after almost 1 year of haloperidol treatment and persisted 2 weeks after haloperidol withdrawal, so it is unlikely that it is haloperidol-related.
Correct answer: Blood tests to measure level of cobalamin

Blood tests should be performed to measure the level of cobalamin. Based on the symptoms, the patient likely suffers from subacute combined degeneration of spinal cord due to degeneration of the posterior and lateral columns of the spinal cord. This is a result of a vitamin B12 (cobalamin) deficiency; a blood test to determine the level of vitamin B12 should be performed to confirm the diagnosis.

Performing blood tests to measure the level of thiamine is an incorrect response. A deficit in vitamin B1 (thiamine) causes symptoms that are characteristic of patients with Wernicke-Korsakoff syndrome. These include confusion and movement disability; however, the patients with Wernicke-Korsakoff syndrome do not generally have tingling or numbness sensations.

Performing blood tests to measure the level of glucose is an incorrect response. Symptoms such as pain, tingling, and numbness in the hands and feet, as well as depression, can be signs of developing diabetic neuropathy. This patient lacks other symptoms associated with diabetes, making this diagnosis unlikely; therefore, testing for glucose levels is not a necessary next step.

A thyroid-stimulating hormone (TSH) test is an incorrect response. Muscle weakness, numbness, pain, as well as depression, are associated with the late stages of chronic hypothyroidism; however, these symptoms are not sufficient to suspect hypothyroidism. Hypothyroidism is characterized by weight gain, loss of concentration, and hearing loss in the elderly, which are not conditions that affect this patient; therefore, a TSH test would not be useful in this case.

Neuroimaging studies are performed to provide information about brain tumors, skull fractures, and other diseases of the brain and blood vessels in the brain. Based on the symptoms seen in this patient, neuroimaging would not help in establishing a diagnosis.
A 65-year-old woman presents with a 3-week history of stomach pain. Her family history is remarkable for her father's death due to prostate cancer and the deaths of her mother and sister due to breast cancer. Her 2 brothers experienced myocardial infarctions; both of them have had coronary bypass operations. The patient suffers from hypertension, hyperlipidemia, and coronary heart disease. She underwent angioplasty and stent placement 1 year prior to presentation. She denies having any GI problems in the past.

Her daily medications include aspirin (81 mg daily), simvastatin (3 x 5 mg daily), metoprolol (3 x 50 mg daily), vitamin E (400 I.U. daily), and a multivitamin supplement. In addition, for the past 12 weeks, she has been taking diclofenac (3 x 50 mg daily) for osteoarthritis of the right knee. She admits to sometimes taking up to 5 x 50 mg of diclofenac for management of intense pain. She states that she always used to be constipated; lately, her stool is occassionally black.

Her temperature is 36.8° C, blood pressure is 135/90 mm Hg, pulse rate is 105/min, and respiratory rate is 18/min. Physical examination reveals an obese, pale woman in no apparent distress. ECG shows slight ST depression and atrial fibrillation. There is pain upon pressure in the epigastric area. The rest of the examination is unremarkable.

Lab analysis is as follows: hemoglobin 10.8 g/dL (normal 14 to 18 g/dL); hematocrit 35.2% (normal 42% - 52%); mean corpuscular volume 92 μm3 (normal 80 - 94 μm3), white blood cell count 26,900/mm; and platelet count 233,000/mm3.

What is the most appropriate next step?
1. Gastroscopy
2 Colonoscopy
3 Helicobacter pylori treatment
4. Barium X-ray
5. Rectosigmoidoscopy
Correct answer: Niacin

Niacin deficiency has been linked to pellagra. This condition, now quite rare, consists of nausea with vomiting, diarrhea, dermatitis, and cognitive decline (such as irritability and memory loss). Vitamin B6 (pyridoxine) deficiency and excess has been associated with neurological disease. Both lead to a distal symmetrical polyneuropathy. A deficiency of B6 is commonly found in patients taking INH, an anti-tuberculosis drug. INH inhibits pyridoxine phosphorylation, thereby decreasing the concentration of the active compound-pyridoxal phosphate. Excessive use of B6 leads to an excess of pyridoxine, which competes with pyridoxal phosphate for binding sites on the enzyme. Vitamin B12 deficiency causes both a peripheral neuropathy and a degeneration in the spinal cord known as subacute combined degeneration. This affects the dorsal column sensory functions (position sense and vibration) and the lateral corticospinal tract. The resulting symptoms include paresthesias of the feet and hands with subsequent weakness and stiffness of the legs and a spastic gait.

Vitamin B1 (Thiamine) deficiency has been linked to the neurological syndrome of Wernicke's encephalopathy. This condition, although often associated with alcoholism, is a true nutritional deficiency, causing neuropathological lesions in areas such as the thalamus, mammillary bodies, and the periaqueductal areas. A polyneuropathy has also been linked to Vitamin B1 deficiency, especially in alcoholism. This deficiency plays at least a role in the development of this disease, along with the possible neurotoxic role of alcohol.

Vitamin E deficiency occurs usually with malabsorption of the fat-soluble vitamins A, D, E, and K. This can occur with conditions such as cholestatic liver disease or celiac disease. Neurological symptoms include a peripheral neuropathy and spinocerebellar degeneration. The spinocerebellar degeneration can occur as a defect in abetalipoproteins, as occurs in Bassen-Kornzweig syndrome. This is also associated with acanthocytes and retinitis pigmentosa.
Correct answer: Fluticasone metered dose inhaler

The correct response is fluticasone metered dose inhaler.

The patient described in the above scenario has the diagnosis of eosinophilic esophagitis (EE). Because this is a relatively new diagnosis, the prevalence of EE is unknown, although the incidence is increasing in both children as well as adults. These patients will have symptoms typically 4 - 5 years before they have a confirmed diagnosis. EE is more commonly seen in male patients who are Caucasian, and up to 70% of patients with EE have personal history of atopy to environmental or foods.

The most common symptoms found in adults with EE are dysphasia, refractory heartburn, chest pain, and food impaction. Usually, no physical exam abnormalities will be detected. An endoscopy with esophageal biopsy is the only definitive method to diagnose EE. The presence of eosinophils of 15 or more per high power field is required for a diagnosis. Other physical findings that may be visualized during an endoscopy that are highly suggestive of EE include stacked circular rings ('feline esophagus'), proximal strictures, linear furrowing, as well as white exudates or even papules that signify eosinophilic purulent material.

Currently there are no FDA approved medications for the treatment of EE. The most efficient treatment for symptoms is topical corticosteroids, either via fluticasone metered dose inhaler or viscous budesonide. These medications are swallowed and therefore coat the esophagus; the symptoms as well as the overall eosinophilia improve fairly quickly. Patient response is usually in the first few days of treatment; however, symptoms will reoccur once the medication regimen is discontinued. Topical fluconazole is not the correct option; EE is not a fungal infection. Oral ampicillin is not correct because EE's underlying pathology is not a bacterial infection. Oral omeprazole is not specifically recommended for treatment of EE; however, it may treat the co-existent GERD. Oral cetirizine is also not an appropriate choice; if there is evidence of severe atopy, therapy should be initiated, but no evidence presents in the case scenario showing that the treatment of airborne allergic disorders improves EE.
Correct answer: Vitamin C deficiency

In cases of vitamin C (or ascorbic acid) deficiency, patients can present with bleeding tendencies (as a result of weakened capillaries) and impaired wound healing due to impaired formation of connective tissue. On examination, the gums may be swollen and friable; the teeth may be loose. There may also be multiple splinter hemorrhages on the nails and ecchymoses, especially over the lower limbs. Causes include inadequate dietary intake and certain conditions, such as pregnancy and lactation, which increase vitamin C requirements. Dietary sources of vitamin C include citrus fruits, such as oranges, lemons, and tangerines, as well as tomatoes and potatoes.

Vitamin E deficiency may cause a hemolytic anemia in premature infants. Laboratory investigations reveal low plasma tocopherol levels, a low hemoglobin level, reticulocytosis, hyperbilirubinemia, and creatinuria. Causes of vitamin E deficiency in premature infants include limited placental transfer of vitamin E and the resultant low levels at birth combined with its relative deficiency in the infant diet. Dietary sources for older children and adults include wheat germ, vegetable oils, egg yolk, and leafy vegetables.

In cases of vitamin A deficiency, patients can present with inability to see well in dim light or night blindness. There may also be conjunctival and corneal xerosis, as well as pericorneal and corneal opacities, and Bitot's spots. Bitot's spots are a collection of keratin appearing as triangular foamy spots on the conjunctiva. Patients may also have xeroderma, hyperkeratotic skin lesions, and increased susceptibility to infections. Causes include inadequate dietary intake and malabsorption. Dietary sources of vitamin A include fish, liver, egg yolk, butter, cream, dark green leafy vegetables, as well as yellow fruits and vegetables.

Niacin deficiency causes pellagra, which is characterized by:

A symmetrical dermatitis, usually on parts of the body exposed to sunlight
Scarlet glossitis and stomatitis
Diarrhea
Mental aberrations, such as memory impairment, depression, and dementia. These may appear alone or in combination. Causes include inadequate dietary intake, especially in patients with corn-based diets or alcoholism. Dietary sources include legumes, yeast, meat, and enriched cereal products.
In cases of pyridoxine or (vitamin B6 deficiency), patients can present with peripheral neuropathy, seborrheic dermatosis, glossitis, and cheilosis. Laboratory investigations reveal anemia with lymphopenia. Causes include malabsorption as well as medications, such as isoniazid and penicillamine. Dietary sources of vitamin B6 include liver, legumes, whole grain cereals, and meats.
Correct answer: Use of proton pump inhibitors

This patient is presenting with Barrett's esophagus, a type of chronic esophagitis in which the normal squamous epithelium is replaced with columnar epithelium. Barrett's esophagus is a complication of chronic gastroesophageal reflux disease (GERD) and can develop into esophageal adenocarcinoma. Use of proton pump inhibitors (PPIs) reduces the risk of cancer. In order to monitor for the development of cancer, a routine endoscopy should be periodically performed in patients with known Barrett's esophagus.

While the antacids are not necessarily adequate to control the GERD and damage to this patient's esophagus, avoidance of antacids will have no role in prevent subsequent Barrett's esophagus complications, such as adenocarcinoma.

While the acid reflux appears to be a major mechanism for damage to the esophageal tissue, anti-reflux surgeries do not appear to prevent complications from Barrett's esophagus and subsequent development of adenocarcinoma. This patient does not need referral for fundoplication (surgical 'wrapping' of the stomach around the esophagus).

Regular exercise would be helpful for this patient in general terms for cardiovascular health and weight reduction. However, exercise does not play a significant role in reducing risks for Barrett's esophagus complications.

Obesity is a risk factor for development of Barrett's esophagus. (Other risk factors include male gender, smoking, and especially chronic GERD.) Once Barrett's esophagus has developed, however, weight loss does not dramatically alter the risk of complications, such as adenocarcinoma. Weight loss should be recommended for any obese patient, but the PPIs are the best approach to reduce complications from his Barrett's esophagus.
Correct answer:
Vitamin E deficiency

Explanation
Vitamin E deficiency may cause a hemolytic anemia in premature infants. Laboratory investigations reveal low plasma tocopherol levels, a low hemoglobin level, reticulocytosis, hyperbilirubinemia, and creatinuria. Causes of vitamin E deficiency in premature infants include limited placental transfer of vitamin E and the resultant low levels at birth; this is combined with its relative deficiency in the infant diet. Dietary sources for older children and adults include wheat germ, vegetable oils, egg yolk, and leafy vegetables.

In cases of vitamin A deficiency, patients can present with night blindness or an inability to see well in dim light. There may also be conjunctival and corneal xerosis, as well as pericorneal and corneal opacities and Bitot's spots. Bitot's spots are a collection of keratin appearing as triangular, foamy spots on the conjunctiva. The patient may also have xeroderma, hyperkeratotic skin lesions, and increased susceptibility to infections. Causes include inadequate dietary intake and malabsorption. Dietary sources of vitamin A include fish, liver, egg yolk, butter, cream, dark green leafy vegetables, and yellow fruits and vegetables.

In cases of pyridoxine (vitamin B6 deficiency), patients can present with peripheral neuropathy, seborrheic dermatosis, glossitis, and cheilosis. Laboratory analysis reveals anemia with lymphopenia. Causes include malabsorption, as well as medications (e.g., isoniazid and penicillamine). Dietary sources of vitamin B6 include liver, legumes, whole grain cereals, and meats.

In cases of vitamin C (ascorbic acid) deficiency, patients can present with bleeding tendencies (as a result of weakened capillaries) and impaired wound healing due to the impaired formation of connective tissue. On examination, the gums may be swollen and friable; the teeth may be loose. There may also be multiple splinter hemorrhages on the nails and ecchymoses, especially over the lower limbs. Causes include inadequate dietary intake and certain conditions (e.g., pregnancy and lactation) which increase vitamin C requirements. Dietary sources of vitamin C include citrus fruits, (e.g., oranges, lemons, and tangerines), as well as tomatoes and potatoes.

Niacin deficiency causes pellagra, which is characterized by:

A symmetrical dermatitis, usually on parts of the body exposed to sunlight
Scarlet glossitis and stomatitis
Diarrhea
Mental aberrations (e.g., memory impairment, depression, and dementia) which may appear alone or in combination
Causes of niacin deficiency include inadequate dietary intake (especially in patients with corn-based diets or alcoholism). Dietary sources include legumes, yeast, meat, and enriched cereal products.
Correct answer:
Intravenous hydration and analgesia followed by laparoscopic cholecystectomy within 72 hours

Explanation
The correct answer is intravenous hydration and analgesia followed by laparoscopic cholecystectomy within 48 hours. Once a diagnosis of cholecystitis has been made, it is important to stabilize the patient with hydration and pain medication prior to surgical correction. Laparoscopic cholecystectomy is a preferred surgical treatment compared with open cholecystectomy due to decreased post-operative recovery period and decreased complication rate. Studies have investigated the optimal timing for surgical treatment and suggest that surgery within 72 hours results in a shorter hospital stay and lower readmission rate.

Intravenous hydration and analgesia followed by discharge is not the correct answer. Once a diagnosis of cholecystitis has been made, it is important to stabilize the patient with hydration and pain medication. However, definitive management of the cholecystitis requires removal of the gallbladder (as the stones themselves cannot be removed) in order to decrease recurrent symptomatic episodes.

Emergency laparoscopic cholecystectomy within 8 hours is not the correct answer. While early surgical intervention has been shown to be beneficial to the patient, this is defined as within the first 72 hours. Emergent surgical treatment should be reserved for patients suffering from a complication from acute cholecystitis, such as empyema, emphysematous cholecystitis, or a perforation.

Intravenous hydration and analgesia followed by laparoscopic cholecystectomy within the following 2 weeks is not the correct answer. While hydration and pain medication are appropriate, studies have investigated the optimal timing for surgical treatment and suggest that surgery within 72 hours results in a shorter hospital stay and lower readmission rate.

Oral analgesia followed by elective laparoscopic cholecystectomy is not the correct answer. Studies have investigated the optimal timing for surgical treatment and suggest that surgery within 72 hours results in a shorter hospital stay and lower readmission rate. Delayed surgical intervention is best reserved for patients with overall medical conditions that need to be stabilized prior to surgery or patients in whom the diagnosis of acute cholecystitis is in doubt.
Correct answer: Gilbert's syndrome

Gilbert's syndrome is a multifactorial inherited disorder that affects the way bilirubin is processed by the liver and causes jaundice. The symptom of jaundice appears under conditions of exertion, stress, fasting, and infections. The condition is usually benign. Symptoms include mild jaundice, fatigue, nausea, and abdominal pain in the upper right quadrant. A serial serum indirect bilirubin shows changes consistent with Gilbert's disease. Usually no treatment is necessary, but phenobarbital can reduce the bilirubin level and relieve the jaundice. Jaundice may fluctuate and persist throughout the patient's life. It usually causes no health problems.

Crigler-Najjar syndrome is inherited as an autosomal recessive trait. Infants who inherit the trait from both parents (this is called homozygous inheritance) develop severe jaundice (hyperbilirubinemia) a few days after birth. If these infants are not treated, they may develop kernicterus (a condition in the newborn marked by severe neural symptoms, associated with high levels of bilirubin in the blood). The jaundice will persist into adult life and require daily treatment. The constantly elevated levels of bilirubin eventually will produce an adult form of kernicterus despite daily treatment. Symptoms include yellow skin and eyes (jaundice) that begins on the 2nd or 3rd day of life and progressively worsen, jaundice that persists beyond 2 weeks without an obvious cause, and confusion (resulting from brain damage). Liver transplantation may be considered for some people with this disorder.

Breast milk jaundice is a persistent jaundice in the newborn caused by a hormone found in breast milk. Bilirubin is eliminated by chemically hooking a sugar molecule to the bilirubin molecule, which then makes the bilirubin water-soluble. In its water-soluble form, bilirubin can be excreted in the urine. Breast milk contains a hormone, pregnanediol, which interferes with the body's ability to hook the sugar onto the bilirubin. Symptoms include jaundice in a breast-fed newborn that lasts longer than a week. Cessation of nursing for 24 to 48 hours will result in a rapid drop of bilirubin.

Newborn jaundice is a condition of yellowish skin color during the newborn period. This results from the immaturity of liver function combined with the destruction of redblood cells present in the newborn infant. The jaundice usually appears between the 2nd and 5th days of life and clears by 2 weeks. Symptoms include yellow color of the skin (jaundice), poor feeding (may be present), and lethargy (may be present). An elevated serum bilirubin level will be present. Usually treatment is unnecessary. Sometimes artificial lights (called bili lights) are used on infants whose levels are very high, or on premature infants. The jaundice resolves without treatment within 1 to 2 weeks.

Dubin-Johnson syndrome is an inherited disorder characterized by mild jaundice throughout life. The transport of bilirubin from the liver into the biliary system is abnormal, and the bilirubin accumulates in the liver. Affected people have lifelong low-grade jaundice that may be aggravated by alcohol, pregnancy, infection, and other environmental factors. The symptoms include a mild jaundice, which may not appear until puberty or adulthood.
Correct answer: Staphylococcus aureus

In cases of Staphylococcus aureus food poisoning, patients present with severe nausea and vomiting. They may also develop diarrhea, abdominal pain, and occasionally headache and fever. Stools are watery but not blood stained. The onset of symptoms is 1 to 6 hours after ingesting the contaminated food. Common culprits include processed meat, dairy products, and potato salads, especially when prepared by food handlers with skin infections that are left at room temperature. Several persons may be similarly affected. There are no fecal leukocytes or trophozoites on examination of the stool. Management includes intravenous replacement of fluid and electrolytes.

In cases of Clostridium perfringens, food poisoning, patients present with watery diarrhea 8 to 24 hours after ingesting contaminated food like inadequately cooked meat or legumes. No fecal leukocytes or trophozoites are present on stool examination.

In cases of Escherichia coli serotype O157:H7 food poisoning, patientspresent with watery diarrhea, which can become bloodstained. Sources of infection include undercooked beef. A stool examination reveals polymorphonuclear leukocytes.

Giardia lamblia can be acquired by drinking contaminated water. Patients present with watery diarrhea. The incubation period is around 1 week. There are trophozoites in the stool, but no leukocytes.

Patients with Salmonella typhi food poisoning usually present with bloody diarrhea. Sources of infection include beef, poultry, eggs, and dairy products. Examination of the stool reveals mononuclear leukocytes.
Correct answer: Administration of vitamin C

The patient suffers from scurvy due to a deficiency of dietary vitamin C. Absence of vitamin C leads to impaired hydroxylation of proline residues in the nascent procollagen chains leading to weakness of blood vessel walls. Clinically, the deficiency syndrome is characterized by perifollicular hemorrhages, fragmentation of hairs, purpura, ecchymoses, splinter hemorrhages, and hemorrhages into muscle. In patients with normal dentition, gum changes (e.g., swelling, bleeding, and loosening of teeth) are also noted. Without supplementation with vitamin C, death may eventually occur.

Administration of factor VIII would be indicated for factor VIII deficiency. This deficiency would lead to a prolonged PTT (partial thromboplastin time), which was not noted in this patient.

Administration of iron would be of benefit in iron-deficiency anemia, but there is no indication of a hypochromic, microcytic anemia in this patient. The anemia of scurvy is typically normochromic and normocytic due to bleeding.

Administration of vitamin B12 would be indicated for a megaloblastic anemia. Although a macrocytic anemia may be observed in scurvy (due to concomitant dietary folate deficiency or perturbations in the folate pool), this patient did not show macrocytosis.

Administration of vitamin K would be appropriate for vitamin K deficiency. This deficiency would produce prolongations of the prothrombin time (PT), followed by prolongation of the PTT as the vitamin K-dependent factors (II, VII, IX, X, protein C, and protein S) are depleted.
Correct answer: Visible bowel serosa and mesenteric fat

A flexible sigmoidoscope is a fiberoptic instrument used to screen patients for colon cancer. Many authorities recommend routine screening starting at age 50-60. If there are no symptoms or family history, most experts recommend performing a combination of three hemoccult cards and flexible sigmoidoscopy every five years. If there are any danger symptoms (rectal bleeding, positive hemoccults, nausea, weight loss) then colonoscopy should be considered.
Sigmoidoscopy is a fairly safe procedure, but there are some potential complications. The most feared complication is bowel wall perforation, where the instrument suddenly views bowel serosa (not intraluminal mucosa!) and peritoneal mesentery. This complication requires emergency surgery.

A "red out" occurs when the scope is difficult to advance and the view becomes red. This indicates that the scope has been pressed up against the wall of the colon and red vasculature is all you can see.

A "white out" occurs when the scope has been pressed up against the wall of the colon so hard that the vasculature blanches, turning the screen white. This is extremely dangerous, and signifies impending bowel perforation.

A completely black view simply indicates that the fiberoptic lens (or the light source) at the tip of the instrument has been covered by debris, typically retained stool. Most instruments have the ability to clean the lens with pressurized water and air.

Sliding by a bowel wall spasm is often performed by experienced clinicians, but requires a delicate touch to minimize the risk of perforation. The tip of the instrument is angled and advanced so that it bluntly pushes its way past an obstruction.
Correct answer:
High lactate levels

Explanation
Chronic metabolic acidosis in von Gierke's disease is due to elevated lactic acid levels.

In von Gierke's disease, there is a deficiency of glucose 6-phosphatase, which produces a block in the conversion of glucose 6-phosphate to glucose. Hence, glycogen breakdown, which normally produces glucose, diverts the glucose 6-phosphate into glycolysis. The clinical features include hepatomegaly, cherubic face, growth retardation, delayed onset of puberty, weakness, convulsions, bleeding tendencies due to altered platelet function, xanthomas, osteopenia, and fractures. The metabolic effects include hypoglycemia, hyperlactacidemia, hyperuricemia, and hyperlipidemia. Definitive diagnosis is by assessment of glucose 6-phosphatase activity in fresh frozen liver tissue specimen, which is obtained by liver biopsy. The aim of the treatment is prevention of hypoglycemia and metabolic acidosis by frequent small carbohydrate-rich meals and restriction of galactose and fructose intake, which increases the lactic acid levels. Persistent elevated uric acid levels may be treated with allopurinol.

In von Gierke's disease, there is an excess of lactic acid and a chronic lactic acidemia. In an attempt to buffer the chronic metabolic acidosis, the calcium phosphate salts in the bones disassociate to free inorganic phosphate anions into the blood stream. The bones become demineralized, and they easily fracture from ordinary daily stress, as in the case of the above patient.

In this disease, blood uric acid levels are increased. This is due to increased generation and decreased excretion of uric acid by the kidneys. It may be that the excess glucose 6-phosphate is also diverted into the pentose pathway and ultimately into the formation of phosphoribosyl-pyrophosphate, which is the starting substrate for the formation of pyrimidines.

Because in this disease there is a bottleneck in glycogen breakdown at the glucose 6-phosphate step, glycogen stores build up in the liver and in the kidney; neither event in and of itself is a cause of the chronic acidosis.
Correct answer: Zinc

This patient most probably has a zinc deficiency. The causes of zinc deficiency include malnutrition, chronic debilitating diseases, chronic renal disease, alcoholism, drugs such as penicillamine and diuretics, and genetic disorders, such as sickle cell disease. Clinical manifestations in severe cases include alopecia, diarrhea, weight loss, infections, dermatitis, hypogonadism in men, and intercurrent infections. Supplementation with zinc is the treatment of choice.

Niacin (nicotinamide, nicotinic acid) deficiency is uncommon in the United States. It is often found in people who live on a diet that consists mainly of corn. Deficiency may also result from alcoholism, cirrhosis, or diarrhea. Men and women are affected equally. Symptoms of niacin deficiency include nausea, vomiting, diarrhea, rash, glossitis, stomatitis, depression, and psychosis. Niacin deficiency, also known as pellagra, manifests as the '3 Ds': diarrhea, dermatitis, and dementia. If this diagnosis is suspected, supplemental niacin should be given immediately. Some patients deficient in niacin may also be deficient in other vitamins, such as B vitamins, so they should be given that as well.

Folate deficiency causes megaloblastic macrocytic anemia, as folate plays a key role in nucleic acid synthesis. The early manifestation of folate deficiency, especially in its suboptimal state, predisposes to occlusive vascular disease and thrombosis. These manifestations are linked to increased homocysteine levels found in folate deficiency. Neurological and immunological disturbances may also be seen. Folate replacement is the option to prevent and to treat the deficiency.

Vitamin B1 (or thiamine deficiency) causes beri beri, occurring mostly in the malnourished and alcoholics. The deficiency manifests with acute heart failure, neurologic deficits, and epilepsy. Empiric use of thiamine and prophylactic use in high-risk population is strongly recommended even before blood reports are obtained, as the treatment is inexpensive and prevents major catastrophes.

Vitamin B12 or cobalamin deficiency manifests as megaloblastic macrocytic anemia, pancytopenia, and a spectrum of neurological disorders such as peripheral neuropathy, parasthesias, and demyelination of corticospinal tract. Nutritional deficiency, alcoholism, and malabsorption syndromes are some causes of B12 deficiency. It also is associated with homocystenemia and atherosclerosis. Diagnosis is by serum estimation of B12, and oral supplementation is safe and effective, but intramuscular injections may also be used.
Correct answer: Prescribe a proton pump inhibitor

The most common causes of a chronic cough in immunocompetent adults (explaining 95% of cases) are post-nasal drip syndrome, gastroesophageal reflux disease (GERD), asthma, chronic bronchitis from cigarette smoking, bronchiectasis, eosinophilic bronchitis, or the use of an angiotensin-converting enzyme inhibitor (ACEI). The rest of the patients (5%) have chronic cough caused by bronchogenic carcinoma, carcinomatosis, sarcoidosis, left ventricular failure, or aspiration. A psychogenic cough, or a cough persisting from habit, is rare and is a diagnosis of exclusion.

Post-nasal drip syndrome could be due to vasomotor rhinitis, allergic or non-allergic rhinitis, or chronic bacterial sinusitis. It would be unusual for a patient to present with a chronic cough due to a post-nasal drip syndrome with no symptoms referable to the HEENT (e.g., the absence of post-nasal drip, sore throat, nasal symptoms, or a feeling of fullness/gagging in the throat); therefore, the prescription of a decongestant/antihistamine is unwarranted. Sinus X-rays are indeterminate in the diagnosis of chronic sinusitis.

Asthma may present with chronic cough in adults, without wheezing or shortness of breath; however, even if used suboptimally, the steroid inhaler should have some effect. It should be noted that a steroid inhaler is also the treatment for eosinophilic bronchitis. Moreover, if a further trial of steroids were to be indicated, it would be prudent to try the steroid inhaler again (this time with a spacer) instead of subjecting the patient to possible complications of oral steroids.

Of the patients who present with a chronic cough who are subsequently found to have GERD as a cause, about 75% do not have symptoms of heartburn or regurgitation. Given this patient's presentation, it seems reasonable to approach her as if she had GERD and prescribe a proton-pump inhibitor as a trial for 2 - 3 weeks. It is very likely that this will control her cough, and over time the proton-pump inhibitor (PPI) can be downgraded to less aggressive treatment with H2-blockers. This would avoid both the high cost of PPIs, as well as the chronic use of PPIs in patients. The use of codeine in an elderly patient is not a good idea unless there is a high degree of assurance that its use will substantially help the patient. Excessive sedation, falls, and constipation are common side effects of narcotics in the elderly.
Correct answer: Chronic pancreatitis

Pancreatitisis an inflammation or infection of the pancreas.

Chronic pancreatitis, which is the correct response, is caused by alcohol abuse, hemochromatosis (a condition of excess iron in the blood), and other unknown factors. Inflammation and fibrosis cause the destruction of functioning glandular tissue in the pancreas. This results in an inability to properly digest fat caused due to a lack of pancreatic enzymes. The production of insulin is also affected. Symptoms include abdominal pain (mainly in the upper abdomen), nausea, vomiting, weight loss, and fatty stools. Additional symptoms may include swelling (overall), stools (clay colored), and abdominal indigestion. Tests should include serum lipase (may be elevated), serum amylase (may be elevated), serum trypsinogen (may be low), and fecal fat test (shows fatty stools). Abdominal ultrasound and CT may show an enlarged pancreas. Treatment of chronic pancreatitis includes reducing pancreas stimulation, alleviating fat indigestion, reducing pain, and treating diabetes. A reduced-fat diet, vitamin supplementation, no alcohol or caffeine, and regulation of blood sugar levels are indicated in the treatment.

The chief causes of acute pancreatitis in adults are gallstones, other biliary disease, and alcohol use. Viral infection (mumps, Coxsackie B, mycoplasma pneumonia, and Campylobacter), injury, pancreatic or common bile duct surgical procedures, and certain medications (especially estrogens, corticosteroids, thiazide diuretics, acetaminophen, tetracycline) are other causes. After the triggering event, the process continues with autodigestion that causes swelling, hemorrhage, and damage to the blood vessels. An attack may last for 48 hours. Symptoms include abdominal pain (mainly located in the upper abdomen) nausea, vomiting, weakness, sweating, anxiety, fever, clammy skin, and mild jaundice. General examination may show a low blood pressure and a heart rate above 90. Most cases resolve within a week with supportive measures, such as analgesics and fluid replacement. However, some cases can be life threatening.

Pancreatic abscess occurs in 5 to 10% of people with acute pancreatitis. An abscess may be caused by inadequate drainage of a pancreatic pseudocyst, which is a complication associated with pancreatitis. Symptoms include fever, chills, abdominal pain, and abdominal mass. Physical exam will show signs of pancreatitis, and tests should include an abdominal CT and ultrasound. Treatment will include laparotomy with drainage and possible resection of dead tissue.

Pancreatic cancer is the 4th most common cancer causing death in the U.S. The disease is more common in men, especially those between 60 and 70 years. The cause is unknown; however the incidence is greater in smokers. A high fat diet and chemical exposures may increase the risk. Symptoms include weight loss, abdominal pain, loss of appetite, jaundice, nausea, weakness, fatigue, vomiting, diarrhea, indigestion, back pain, stools (clay colored), pallor, and depression. Tests should include a pancreatic biopsy, an abdominal CT scan, and abdominal ultrasound. Only 20% of the tumors are operable at the time of diagnosis. Palliation is generally the treatment, along with chemotherapy and radiation.

Insulinomas are generally benign tumors of the insulin-secreting cells of the pancreas, which secrete excess amounts of insulin. Risk factors include a prior history of multiple endocrine neoplasia Type I (MEN I). Symptoms include sweating, tremor, rapid heart rate, anxiety, hunger, dizziness, headache, clouding of vision, confusion, behavioral changes, convulsions, and loss of consciousness. Surgery is the treatment of choice to remove the tumor. If the tumor is not found during surgery, diazoxide may be given. A diuretic is always given with this medication to avoid retaining too much salt.
Correct answer:
Vitamin A deficiency

Explanation
In cases of vitamin A deficiency, patients can present with inability to see well in dim light or night blindness. There may also be conjunctival and corneal xerosis, as well as pericorneal and corneal opacities, and Bitot's spots. Bitot's spots are a collection of keratin appearing as triangular foamy spots on the conjunctiva. The patient may also have xeroderma, hyperkeratotic skin lesions, and increased susceptibility to infections. Causes include inadequate dietary intake and malabsorption. Dietary sources of vitamin A include fish, liver, egg yolk, butter, cream, dark green leafy vegetables, yellow fruits, and vegetables.

Vitamin E deficiency may cause a hemolytic anemia in premature infants. Laboratory investigations reveal low plasma tocopherol levels, a low hemoglobin level, reticulocytosis, hyperbilirubinemia, and creatinuria. Causes of vitamin E deficiency in premature infants include limited placental transfer of vitamin E and the resultant low levels at birth combined with its relative deficiency in the infant diet. Dietary sources for older children and adults include wheat germ, vegetable oils, egg yolk, and leafy vegetables.

Niacin deficiency causes pellagra, which is characterized by:

A symmetrical dermatitis usually on parts of the body exposed to sunlight
Scarlet glossitis and stomatitis
Diarrhea
Mental aberrations, such as memory impairment, depression, and dementia. These may appear alone or in combination. Causes include inadequate dietary intake, especially in patients with corn-based diets and alcoholism. Dietary sources include legumes, yeast, meat, and enriched cereal products.
In cases of vitamin C (or ascorbic acid) deficiency, patients can present with bleeding tendencies (as a result of weakened capillaries) and impaired wound healing due to impaired formation of connective tissue. On examination, the gums may be swollen and friable; the teeth may be loose. There may also be multiple splinter hemorrhages on the nails and ecchymoses, especially over the lower limbs. Causes include inadequate dietary intake and certain conditions, such as pregnancy and lactation, which increase vitamin C requirements. Dietary sources of vitamin C include citrus fruits, such as oranges, lemons, and tangerines, as well as tomatoes and potatoes.

In cases of pyridoxine (or vitamin B6 deficiency), patients can present with peripheral neuropathy, seborrheic dermatosis, glossitis, and cheilosis. Laboratory investigations reveal anemia with lymphopenia. Causes include malabsorption as well as medications, such as isoniazid and penicillamine. Dietary sources of vitamin B6 include liver, legumes, whole grain cereals, and meats.
Correct answer:
Observation only

Explanation
Pancreatic pseudocysts complicate about 10% of attacks of acute pancreatitis. A pseudocyst is formed when pancreatic fluid leaks and is confined by organs adjacent to the pancreas. Eventually, a fibrous wall forms around the collection. Most cysts regress spontaneously over a period of several weeks, but in some cases complications such as bleeding, abscess formation, and intractable pain may occur. Several interventional approaches — surgical, radiologic and endoscopic — have been tried.

Endoscopic ultrasound has gained popularity in the management of pseudocysts, as it can identify complex cyst wall structures, in particular pseudoaneurysms, do fine-needle aspiration to rule out a neoplasm, and find a favorable spot for drainage. Ruling out a cystic pancreas neoplasm before endoscopic drainage is essential, as attempts at transgastric emptying of a neoplasm can have disastrous effects and compromise further surgical management of the neoplasia.

Indications for intervention are intractable pain, expanding lesions, and infection. A frequently quoted study found that complications such as bleeding, abscess formation, or perforation rose sharply after 6 weeks of observation, and that intervention is warranted if a pseudocyst does not resolve in this period. However, more recent studies have challenged this assumption and proposed that observing asymptomatic pseudocysts for longer periods is safe.

Pseudoaneurysms are found in 10% of pseudocysts. Signs suggesting a pseudoaneurysm include overt bleeding, sudden pseudocyst enlargement, and an abrupt fall in hematocrit. Their presence is a concern when drainage is considered and does not lead to a higher overall bleeding rate or constitute indication for intervention.

Angiography has higher sensitivity and can perform therapeutics, but an angio-CT scan has adequate sensitivity for ruling out a pseudoaneurysm before proceeding with therapeutics. When intervention is warranted, angiographic coil embolization and operative resection can be performed.
Correct answer: Carcinoid syndrome

Explanation In Carcinoid Syndrome, 60-90% of tumors arise from the small bowel. It is caused by a metastasizing carcinoid tumor and is characterized by cutaneous flushing, diarrhea, bronchoconstriction, and endocardial plaques composed of a unique type of fibrous tissue. These effects are due to the circulating humoral substances as serotonin and bradykinin. Cardiac affection happens only when there is metastasis to the liver. The right side of the heart is mainly affected when the humoral substances are not inactivated by the liver due to hepatic metastasis. The tricuspid and pulmonary valves are affected with fibrous plaques (composed of smooth muscle cells in stroma rich in mucopolysaccharide) and as a result, they are fibrosed and fixed in a semi-opened position with both incompetence and stenosis. Fibrous plaques can invade the endocardium of the cardiac chambers, the intima of the inferior vena cava, pulmonary artery, and coronary sinus. Left side valvular affection occurs less commonly and is usually associated with right to left shunts, or when the tumor originates from the lung.
The patients mainly complain of flushing, diarrhea with manifestations of systemic congestion as lower limbs edema, congested liver, congested neck veins, and ascites. Physical examination usually reveals a systolic murmur along the left sternal border produced by tricuspid regurgitation; there may be a mid-systolic murmur or early diastolic murmur due to pulmonary valve affection. There are 2 key diagnostic modalities for the diagnosis of carcinoid heart disease, which includes 24-hour urinary excretion of 5-hydroxy-indole-acetic-acid (5HIAA) and transthoracic echocardiography. ECG and chest X-ray are of limited value. The chest X-ray is normal in half the patients, but may reveal enlargement of the heart and pleural effusions or nodules. The pulmonary artery trunk is typically of normal size without evidence of post-stenotic dilatation as occurs in congenital pulmonic stenosis. ECG shows ST, T wave changes, right atrial abnormalities, and possibly sinus tachycardia. Echocardiography may reveal evidence of tricuspid and/or pulmonary valve thickening, along with right atrial and right ventricular dilatation; small pericardial effusions are present in a minority.

The principles of management of patient with carcinoid heart disease involve treatment of heart failure, pharmacotherapy for the reduction of tumor products, and surgical/interventional management of valvular pathology. Treatment includes symptomatic digitalis and diuretics for treatment of heart failure, and somatostatin analogs such as (Octreotide). Octreotide has been found to improve symptoms as well as survival. GIT main side effects are loose stool, abdominal discomfort, and cholelithiasis. Balloon valvuloplasty of the tricuspid and pulmonic valves may improve survival and symptoms. Surgical treatment, like tricuspid valvotomy and valvectomy, may result in symptomatic improvement; mortality is high especially in the elderly. The long-term mortality remains high regardless of treatment modality, with half the patients dead within 1 to 2 years.

Churg-Strauss syndrome is a granulomatous medium and small vessel vasculitis. The American College of Rheumatology (ACR) has proposed 6 criteria for the diagnosis of Churg-Strauss syndrome, which include the following:

1. Asthma
2. Eosinophilia of more than 10% in the peripheral blood
3. Pulmonary involvement such as infiltrates
4. Paranasal sinusitis
5. Mononeuritis multiplex or polyneuropathy
6. Histological proof of vasculitis with extravascular eosinophils.

Cardiac manifestations include heart failure, myocarditis, pericarditis, constrictive pericarditis, and myocardial infarction. Chest X-ray may show mild infiltrates or nodules. Treatment is symptomatic and systemic anti-inflammatory may improve symptoms.

Dilated cardiomyopathy is characterized by dilated hypokinetic left ventricle with impaired both systolic and diastolic functions and mitral incompetence. Patients complain of left sided heart failure manifestations such as dyspnea and orthopnea. The right ventricle may be affected as well with manifestations of congestive heart failure. Treatment is usually symptomatic with diuretics, vasodilators, and inotropic, cardiac transplantation may be the treatment of choice especially in end stage patients.

Hypereosinophilic Syndrome typically presents in fourth decade males living in a temperate climate. The cause of hypereosinophilia (persistent eosinophilia with 1500 eosinophils/μL for at least 6 months) is unknown and is not associated with leukemia or reaction to parasitic infestations, allergic, hypersensitivity, or neoplastic disorders. There is organ involvement other than the heart, such as the lungs, skin, and bone marrow. Early in the disease (before fibrosis), medical therapy is the treatment of choice. It includes corticosteroids and cytotoxic drugs such as hydroxyurea. Routine cardiac therapy with digitalis, diuretics, afterload reduction, and anticoagulation as indicated are adjuncts in the management of these patients. Surgical therapy is indicated with advanced stage of the disease (fibrosis).

Endocardial fibroelastosis is a condition commonly seen in infants and young children, which is characterized by endocardial thickening and myocardial dysfunction. This condition has a poor prognosis and terminates in death within weeks, especially in progressive heart failure. Viral etiology has been implicated in most cases. Infants present with feeding difficulty, failure to thrive, breathlessness, excessive sweating, and wheezing. Treatment is usually symptomatic with diuretics, vasodilators, and inotropic drugs. Cardiac transplantation may be the treatment of choice, especially in end stage patients.
Correct answer:
Supportive measures

Explanation
Colicky infants cry excessively (more than 3 hours per day, more than 3 days per week and for more than 3 weeks) without any identifiable need. It occurs in all socioeconomic, racial, and ethnic groups with no sex preference. Colic begins typically at 2 weeks of age, peaks at 6 weeks, and usually resolves completely by 16 weeks. Crying spells are episodic and unrelated to feeding with a diurnal pattern; crying is increased in the evening and night. Such infants are inconsolable for long intervals and cry with extreme intensity. They draw up their legs, have tense abdomens, arch their backs, and become 'gassy,' suggesting a GI etiology. However, aerophagia or swallowing of air will occur with excessive crying and the presentation of aerophagia and gas does not coincide with the timing of colic, thus the excessive crying accompanying colic usually leads to aerophagia and subsequent gas. It is believed to be multifactorial with behavioral, social, and neurodevelopmental components. Diagnosis is made by history along with a normal physical exam in a healthy and normally growing infant. Appropriate management should be directed toward supportive interventions, such as recommendations to simulate the fetal environment with tight swaddling, rapid swinging or juggling, non-nutritive sucking, and steady white noise. Holding for prolonged periods has been shown to decrease the intensity but not the duration of crying.

Multiple formula changes or dietary changes for a breastfeeding mother have not been shown to benefit colic in otherwise healthy infants, though a subgroup of infants have colic that may be caused by allergies. If the history is suggestive for this, and emesis, diarrhea, blood in the stool, severe eczema, or urticaria are present, then a short trial of hydrolyzed formula may be appropriate.

Dicyclomine, an anticholinergic, has been shown to decrease symptoms of colic; however, it has also caused sedation, apnea, seizures, and coma, causing manufacturers to contraindicate its use in infants younger than 6 months of age.

Simethicone has also been suggested for gas and colic, but it has not been shown to be more effective than placebo in randomized controlled trials.

Increased crying can be associated with gastroesophageal reflux (GER), however only 2% to 4% of infants with colic have been shown to have GER. Conservative measures of thickening feedings and trial of anti-reflux medication may be considered if symptoms are consistent with possible GER. Prone sleep positioning, once recommended, has the potential for increased risk of infant death syndrome.

Lactose intolerance is uncommon in the first year of life and includes nonspecific symptoms that may be colic-like with bloating and gaseous distention, but also vomiting, spit-up, and nonspecific diarrhea, which are not exhibited here. There are very few indications for lactose-free formula; these include galactosemia and secondary to small intestinal injury not evident in this problem.
Correct answer: Radionuclide scan

The clinical presentation of the child with intermittent painless bleeding per rectum is suggestive of Meckel diverticulum (MD), which accounts for 50% of lower GI bleeds in children below 2 years of age.

Radionuclide scan is the most sensitive study that can confirm the presence of Meckel diverticulum. It is performed after intravenous infusion of technitium-99m pertechnetate. The mucous secreting cell of the ectopic gastric mucosa in the Meckel diverticulum takes up pertechnetate so that it can be visualized on the scan. The uptake can be enhanced by ranitidine, cimetidine, or glucagon. Sensitivity of the enhanced scan is about 85%, and specificity is approximately 95%. During active GI bleeding, the radioactive red cells leak into the intestines where bleeding is occurring and will appear as 'hot areas' on the scan. 'Hot areas' are not seen on the scan in the absence of active bleeding.

Meckel divertculum is a remnant of the omphalomesenteric duct, which connects the yolk sac with the gut in the developing embryo and provides nutrition to the embryo until the placenta develops. Later this duct is obliterated and separates from the intestine. The lining of the yolk sac is similar to the lining of the stomach. Failure of involution of the omphalomesenteric duct results in various residual structures, of which Meckel diverticulum is the most common. It is a 3-6 cm long pouch of ileum, about 50-75 cm from the ileocecal valve. This distance depends on the age of the patient. Meckel divertulum is lined by the acid-secreting mucosa that can cause intermittent, painless rectal bleeding due to ulceration of the diverticular and the adjacent ileal mucosa.

Meckel divertculum can act as a lead point for intussusception or may lead to a volvulus of itself and of the small intestines, thus presenting with bowel obstruction.

Routine barium studies usually do not fill the diverticulum; hence, they are not helpful in the diagnosis. Double contrast barium enema can be performed for evaluation of unexplained lower GI bleeds. However, it should not be undertaken during acute hemorrhagic phase, as it makes subsequent diagnostic evaluation like colonoscopy very difficult. Small bowel barium examination has a low yield because the divertculum fills transiently and the surrounding loops of small bowel overlap and obscure the diverticulum.

CT scan is rarely used as a primary imaging modality where Meckel diverticulum is suspected. If Meckel diverticulum is associated with intussusception, it may be revealed as an intraluminal mass, but it cannot be differentiated from intussusception due to other causes.

Ultrasound has a limited role in evaluating GI hemorrhage. Sometimes intussusception due to Meckel diverticulum has been diagnosed by ultrasound, but the sensitivity and specificity is generally low.

Colonoscopy cannot positively diagnose bleeding from a Meckel diverticulum because the colonoscope usually cannot reach the part of small intestines where the Meckel diverticulum is located. It can be helpful in its diagnosis if blood-filled colon is seen without another source of bleeding, particularly if it is accompanied by an abnormal Meckel radionuclide scan.
Correct answer: Open cholecystectomy

Patients withsymptomatic porcelain gallbladdersare much more commonly female than male, with the typical age range being from 38 - 70 years old. In general, patients will describe a history of biliary type pain. Diagnostically, an ultrasound or CT scan will most efficiently give the visualization of the calcification necessary to confirm a porcelain gallbladder.Those found to have complete mural calcification (complete type) generally will need to have it treated with an open cholecystectomy. This is because the mucosal calcification that creates the porcelain characteristic makes the gallbladder very thick and fibrotic, which in turn makes it potentially difficult to grab by forceps and dissect out in a laparoscopic approach. There have been recent studies suggesting a laparoscopic cholecystectomy would be acceptable in patients who have evidence of a long cystic duct and biliary anatomy that is well-defined perioperatively, as well as in those who have evidence of a less advanced form of porcelain gallbladder, although there is no confirmed change of recommended treatment plan at this time.

Anytime a porcelain gallbladder is found, there has to be a heightened alert for the suspicion of gallbladder carcinoma. The more advanced and severe the calcification is of the gallbladder, the heightened potential of it being malignant. Reassurance only and periodic ultrasounds would not be appropriate in this scenario. This approach may be followed in some part in a patient with less severe signs and symptoms. Percutaneous biliary stent placement is not the appropriate choice for this clinical scenario, as it is not treating the main pathology being presented.
A 65-year-old man presents with a bulge in his lower abdomen that has been present for 5-10 years. He can usually push this bulge back, but, about 4 hours earlier, it came out and could not be pushed back. There is an aching pain, but no nausea or vomiting. He admits to straining at the stool and getting up twice a night to urinate. He smokes 1 pack of cigarettes per day.

On physical examination, his vital signs are within normal limits; his pulse oxy is 91%. There is no jugular vein distention. Chest shows an increased A-P diameter, but breath sounds are good. Heart sounds are distant, and the rate and rhythm indicate normal sinus. There is an obvious bulge in the right lower quadrant extending into the right scrotal sac. The abdomen itself is soft, flat, and non-tender. Bowel sounds are slightly hyperactive; there are no rushes or tingling sounds. The bulge cannot be reduced and is tender. Bowel sounds are present within the mass. A somewhat enlarged, non-tender prostate is detected on rectal examination. Brown stool is obtained and is negative for blood. There is no abnormality on EKG. Chest x-ray shows some hyperinflation, but no infiltrates. An x-ray, flat and upright, of the abdomen shows some dilated small bowel extending into the right scrotal sac, but signs of obstruction are absent. Routine lab and urine studies are within normal limits. You start an intravenous line of 0.9 N/S to run at keep-open rate.

What would be the next logical course of action?

1 Order an immediate surgical consultation
2 Admit the patient to the observation unit for 4 hours and reassess
3 Sedate the patient well and attempt to manually reduce the mass
4 Pass an intestinal tube to reduce small bowel distention
5 Pass a Foley catheter to reduce bladder distention
Correct answer: Sedate the patient well and attempt to manually reduce the mass

The most likely diagnosis in this patient is incarcerated indirect hernia. Direct hernias do not usually incarcerate and come straight through the abdominal wall. Indirect hernias, by definition, come down the inguinal canal into the scrotal sac. Intestinal contents accompany the sac in either one.

This patient's lung signs and signs of prostate enlargement are both impediments to emergency surgery, and they are additional reasons to delay repair to a more elective time if possible. Every reasonable attempt should be made to reduce this hernia using manual compression after adequate sedation. A warm compress on the area may help reduce muscle spasm and relax the abdominal musculature. There are times when either a nasogastric tube and/or a long intestinal tube are warranted. This patient has no signs of intestinal obstruction (vomiting, constipation, etc.). The minimal dilatation of the small bowel is an accompanying sign of an incarcerated hernia. An obstructed segment of bowel may become incarcerated and/or strangulated; when reduced or replaced in the peritoneum, signs and symptoms of mechanical obstruction occur. Of course, should it not be possible to reduce the hernia, obstruction and strangulation both may develop. Strangulation occurs when blood flow (venous, arterial, or both) is closed off, and the bowel segment dies. Then an emergency exists, and repair must occur upon recognition. A Foley catheter has no value unless there is urinary outlet obstruction.
Correct answer:
Ischemic colitis

Explanation
This is a classic presentation of ischemic colitis. Ischemic colitis occurs when there is obstruction of the colonic blood supply, causing ischemia with inflammation and ulceration of the colonic mucosa. The area of the splenic flexure is most at risk for ischemia because it is the watershed area of the arterial supply to the colon.

Ischemic colitis is most commonly seen in elderly individuals. These patients typically present with an acute onset of pain associated with bloody diarrhea, and they have an area of tenderness corresponding to the ischemic segment of colon. Patients may experience recurrent bouts of ischemic colitis. Sigmoidoscopy makes the diagnosis in 85% of patients; 15% will have ischemia in areas proximal to the reach of a sigmoidoscopy. The majority of these patients will improve with supportivecare (hydration and prophylactic antibiotics, in case of bacterial transmigration). However, some patients have much more severe disease, which may even require emergency colectomy.

The most important condition to differentiate from ischemic colitis is that of small bowel ischemia. Small bowel ischemia can lead to gangrene of the intestine. Most commonly, these patients have developed occlusion of some of the distribution of the superior mesenteric artery, either from acute thrombosis in an area of atherosclerosis, or from embolic disease. These patients classically have pain out of proportion to their exam, at least early in the process, and they do not usually have an area of localized tenderness on palpation. These patients can have leukocytosis on the level of 20,000 to 30,000/μL, and they may exhibit a lactic acidosis and elevated amylase levels. Their stool is usually positive for occult blood, but frank blood is only rarely seen. Early diagnosis is very important, although often difficult, and urgent surgery is the only treatment.

Renal or ureteral lithiasis can cause severe abdominal pain, and these patients can have diarrhea as well, but it should not cause hematochezia or localized anterior abdominal tenderness.

Diverticulitis is more common on the left, which is the case with this patient's pain, but it typically does not cause hematochezia. Additionally, the sudden onset of very severe pain would not be typical of diverticulitis.

Infectious colitis can cause abdominal pain and hematochezia, but the improvement in her pain over such a short time period would be atypical for an enterotoxic infection. Nevertheless, infection should be considered in this patient because of her recent travel history.
Correct answer:
Wireless capsule endoscopy

Explanation
Wireless capsule endoscopy is performed by ingestion of a battery-powered capsule containing a camera and a transmitter. The capsule transmits images to sensors outside the patient during its transit in the gastrointestinal tract. Indications for capsule endoscopy are still evolving, but several series have shown that capsule endoscopy is more sensitive than small bowel radiography or push enteroscopy for locating the source of small-bowel bleeding. The most common etiologies of bleeding in this region are angioectasia and Crohn's disease. Several other small bowel lesions, such as tumors (leiomyoma, lymphomas, carcinoids, or carcinomas), varices, and polyps, have also been described.

An important concern is the possibility of capsule impaction and small bowel obstruction. Some centers are reluctant to perform capsule endoscopy in patients with partial or intermittent obstruction of the small bowel. However, capsule impaction may reveal the site of a lesion and allow its surgical removal, obviating the need for intraoperative enteroscopy. Other relative contraindications are incapacity of cooperating with the examination (e.g. dementia), swallowing disorders, esophageal stricture, gastroparesis, and poor surgical risk. In patients with obstruction or gastroparesis, the examiner may place the capsule endoscopically.

This patient has no signs of ulcer activity. Therefore, biopsy of the duodenal scar is not indicated.

A 99mTc scan is the test of choice for Meckel's diverticulum, a remnant of the embryonic omphalomesenteric duct found in 1.5% of the general population. Its most common location is the antimesenteric border of the mid-to-distal small bowel. Some diverticula contain ectopic, acid-producing gastric mucosa. Therefore, they can ulcerate and bleed. Rectal bleeding caused by Meckel's diverticulum is often maroon or brick-red in color, and its incidence decreases with age. Therefore, this diagnosis is unlikely in this case.

Angiography can be used if other less invasive procedures (i.e. small bowel radiography, push enteroscopy, or small bowel radiography) are non-diagnostic.

Computed tomography is not indicated in the absence of other symptoms indicating a specific etiology (e.g. a palpable mass or suspicion of pancreatic disease).

Of note, colonoscopy after rapid oral purging has emerged as the procedure of choice for the evaluation of acute lower GI bleeding, and it also provides a means for therapy. Scintigraphy and/or angiography also play important roles in diagnosis and embolization when colonoscopy reveals negative findings or when it is not feasible.
Correct answer: Lactose intolerance

Lactose intolerance results from a deficiency of lactose, a disaccharidase in the mucosal cells of the small intestine which splits the disaccharide lactose into glucose and galactose. Patients note borborygmi, flatulence, nausea, abdominal cramps, pain, and diarrhea after ingesting lactose-containing food (e.g., milk). Laboratory investigations reveal that the diarrheal stools are acidic. When the hydrogen breath test is administered, there is elevated hydrogen content in the expired air because the colonic flora digests the unabsorbed lactose. Treatment includes following a lactose-free diet.

Celiac disease is a hereditary disorder caused by gluten intolerance; gluten is found in wheat, rye, barley, and oats. Patients may be asymptomatic or may present with diarrhea, abdominal discomfort, distention, and steatorrhea; their stools are pale, malodorous, and difficult to flush because they float on the toilet water. Patients may have anemia from iron and folate deficiencies, osteomalacia and bone pains from calcium deficiency, and edema from hypoproteinemia. Diagnosis is confirmed by a small intestine mucosal biopsy which shows a flat mucosa due to villous atrophy, and by subsequent improvement on a gluten-free diet. Specific treatment includes a gluten-free diet.

Whipple's disease mainly affects men; it is caused by the bacterium Tropheryma whippelii. It is a multisystemic disease which affects the small intestines, joints, brain, heart, and eyes. Patients can present with diarrhea, steatorrhea, abdominal pain, weight loss, and joint pains. On examination, they may be pale and have lymphadenopathy. Histological examination of a small bowel mucosal biopsy reveals PAS-positive, foamy macrophages. Treatment is with trimethoprim-sulfamethoxazole or chloramphenicol.

Short bowel syndrome is usually the result of surgical resection of the intestines or a jejunoileal bypass. The malabsorption is a result of inadequate absorptive surface. Malabsorption of vitamin B12 results in paresthesias; malabsorption of calcium results in bone pain and carpopedal spasms.

Tropical sprue is an acquired disease that affects both visitors and natives of tropical areas (e.g., the Caribbean and South India). Its etiology is unknown. Patients usually present with diarrhea and weight loss; they report that their stools are soft and bulky (steatorrhea). They may also develop deficiencies of folate and cobalamin. Stool microscopy should be done to look for cysts and trophozoites. Histological examination of a small bowel mucosal biopsy aids in making the diagnosis. Treatment is with tetracycline or oxytetracycline.
Correct answer: Rectal fistula

This patient most likely has a rectal fistula. A fistula (or fistula-in-ano) is a communicating tract (usually from an anal crypt) to the skin surface, and it often develops from a rectal abscess. Rectal fistulas are more common in patients with Crohn's disease. Fistulas are associated with rectal pain, itching and tenderness.

External hemorrhoids are a common condition and can be associated with anal pain, constipation and bright red rectal bleeding. Hemorrhoids are dilated blood vessels and appear as protrusions, either singly or several clustered together, such as a bunch of grapes. (Internal hemorrhoids would not be visualized on this patient without anoscopy or colonoscopy.)

Genital herpes simplex lesions are caused by sexual transmission of herpes simplex virus types 1 or 2 (HSV-1 and HSV-2). The classic presentation is a tingling, burning, and/or painful sensation from 1 or more vesicular or ulcerated lesions on the genitals. This patient's single perianal pustule and history of Crohn's disease make fistula a much more likely diagnosis.

Lymphogranuloma venereum (LGV) is a sexually transmitted disease; it presents with a primary painless lesion on the genitals/anus. LGV is rare is developed countries, with greater incidence in the tropics. This patient noted a painful lesion, making LGV unlikely.

Similarly, a syphilitic chancre is the primary lesion of the sexually transmitted Treponema pallidum. It presents as a solitary, painless anogenital lesion.
Correct answer: Prescribe ursodiol

Both morbid obesity and rapid weight loss are risk factors for development of cholecystitis. Gallstones may be present within the gallbladder and remain asymptomatic, or the gallbladder walls may become inflamed, resulting in cholecystitis. Ursodiol is approved for prevention of gallstones in obesity patients with rapid weight loss. It would be the best choice in health maintenance and delaying or preventing need for surgical cholecystectomy.

Orlistat is a prescription medication approved for treatment of obesity. Its mechanism is to block fat absorption. It does not have a direct role in health maintenance or prevention of cholecystitis or cholelithiasis. In fact, it has pronounced gastrointestinal side effects.

Over-the-counter omeprazole is a commonly used proton-pump-inhibitor (PPI); it is helpful in acid lowering in the stomach and treatment of gastroesophageal reflux disease. It has not been shown to help gallbladder disease, and there is some evidence that PPIs may worsen it.

Reducing the patient's exercise may aggravate her condition and negatively impact her weight loss. Exercise has an inverse relationship with cholecystitis and rates of cholecystectomies, so this patient should be encouraged to continue frequent exercise.

This patient should not be instructed to restrict caloric consumption further. She is already losing weight quite rapidly at around 4 pounds per week. Her daily caloric intake is low; recommending further restriction will likely discourage her, and there will not be any benefit in regard to her gallbladder disease.
Correct answer: No treatment

No treatment is the correct answer. The patient's left lower quadrant abdominal pain is not caused by the gallstones (cholelithiasis), which is an incidental finding on the ultrasound. If the gallstones were causing symptoms, this would be known as cholecystitis and the patient would have different abdominal complaints. Only 5-10% of patients with cholelithiasis actually have cholecystitis, and the symptoms could include right upper quadrant abdominal pain, epigastric abdominal pain, nausea, vomiting, indigestion, or fatty food intolerance. Since this patient is not experiencing symptoms related to the stones, the stones are under 2 - 3 cm, and the gallbladder is not reported as "calcified" in general, she does not need to undergo treatment for the incidental finding of cholelithiasis.

Prophylactic elective cholecystectomy is not the correct answer. Treatment is not needed for asymptomatic stones. If her stones were greater than 2 - 3 cm, her gallbladder were calcified (risk for gallbladder cancer), or she were suffering from recurrent pancreatitis secondary to microlithiasis, then a prophylactic cholecystectomy would be indicated.

Oral analgesics is not the correct answer. The patient has noted pain, but the source has not yet been determined. Whether the patient receives oral analgesics for the left lower quadrant pain while undergoing further workup would be up to the provider. However, analgesics would not be warranted for the cholelithiasis, as the stones are asymptomatic.

Urgent cholecystectomy is not the correct answer. Urgent cholecystectomy is typically performed for patients who have severe symptoms, particularly pain that is intolerable or severe vomiting. Cholecystectomy is usually performed electively (for reasons discussed in "prophylactic elective cholecystectomy"), and very rarely needs to be done on an urgent basis.

Oral dissolution therapy is not the correct answer. Oral dissolution therapy is very rarely used today and was much more popular 10 - 20 years ago. Oral dissolution therapy consists of taking bile acid in pill form to try to dissolve stones. It can work on a small percentage of patients with small, cholesterol gallstones, but has significant negative aspects that have taken the therapy out of popular use. It can be very expensive, take several years, and has a high recurrence rate. As this patient's stones are asymptomatic, the best choice is to watch the stones conservatively.
Correct answer: Antacids

Mild or intermittent symptoms of gastroesophageal reflux disease (GERD) are not typically seen as adversely affecting patients' quality of life. Initially, action is taken in terms of modification of behaviors, such as eating smaller meals as well as eliminating acidic foods and foods known to precipitate the reflux (fatty foods, alcohol, chocolate, or peppermint). Weight loss has also been shown to help decrease issues, and so has advising patients to avoid lying down at least 3 hours after eating meals. Elevation of the head of the bed is also suggested to initiate relief.

From a pharmacological standpoint, antacids are considered the mainstay for rapid relief of occasional heartburn. Antacids work by neutralizing stomach acid only; alone, they will not heal any inflammation that may be caused by stomach acid. Patients should be told that the duration of action of these medications is typically less than 2 hours. Antacids are also contraindicated in any patients with renal failure. Many types of antacids are available over the counter.

The next step up in terms of treatment would be considered to be H2-receptor antagonists. These medications work by reducing stomach acid production. Again, these agents are available over the counter as 50% the prescription dose. Examples include cimetidine 200 mg, ranitidine 75 mg, and famotidine 10 mg. When these agents are taken for active reflux symptoms, they have a delay of onset of at least 30 minutes; once they do take effect, relief is supplied for up to 8 hours per dose.

Proton pump inhibitors are initial pharmacological treatment reserved for patients with moderate to severe symptoms of reflux as well as known complications from GERD. Proton pump inhibitors work by actually blocking the production of acid. Examples of these include omeprazole 20 mg or lansoprazole 30 mg. There are proton pump inhibitors available over the counter, but some are only available with a prescription. Proton pump inhibitors help patients achieve adequate control of their heartburn symptoms, and they can potentially offer resolution of these symptoms; they even aid in healing of erosive esophagitis if it is present.

Antibiotics in relation to reflux or heartburn would not be indicated directly for the symptoms of this pathology; antibiotics could potentially be used as a component of the treatment of a H. pylori infection.

Prokinetic agents are not indicated as treatment of mild, intermittent reflux symptoms.
A 58-year-old man presents with a 1-day history of severe abdominal pain, nausea, and vomiting. He initially thought he had some indigestion, with pain located in the epigastric region, and tried some calcium carbonate (Tums) with no relief. The pain and vomiting progressed through the night and kept him from sleeping and going to work. He feels the pain boring through to his back. He denies hematemesis, fever, diarrhea, out-of-the-country travel, and contact with sick people. Prior to onset of pain, he reports good health. He has no known medical conditions and takes no medications. He has had no surgeries. He smokes cigarettes (1ppd x 40 years), admits "moderate" alcohol use, and denies drug use. He is married and works as a welder.

Vitals are: BP: 102/56 mmHg; HR: 116bpm; RR: 15; Temp: 98.9F; O2Sat: 95% on room air.

On physical exam, the patient appears uncomfortable on the exam table and grimaces when changing position for exam. He is cooperative, alert, and oriented. Abnormal physical exam findings include: abdomen distended, decreased bowel sounds, and tender epigastric region, with guarding. He is tachycardic. No jaundice is noted. The remainder of the exam is normal.



Complete blood count (CBC)
WBC: 15.1
RBC: 4.63
Hemoglobin: 15.4
Hct: 49.9
MCV 87.2
MCH: 29.4
MCHC: 32.1
RDW: 11.7
Platelets: 335
MPV: 7.3
WBC differential: Normal

Complete Metabolic Panel (CMP)
Sodium: 144
K: 3.7
Cl: 102
HCO3: 25
Glu: 82
BUN: 21
Creatinine: 1.0
Ca: 8.9
Albumin: 3.5
Total Protein: 6.4
AST (SGOT): 72
ALT (SGPT): 85
Alk Phosphatase: 155
Total Bilirubin: 0.9
eGFR: >60
Amylase: 272
Lipase: 290
Hepatitis panel: Negative
Abdominal CT: Diffuse pancreatic enlargement and inflammation, with single fluid collection
Urinalysis: Normal

What pharmacologic treatment is the most important intervention for this patient's likely condition?

1. Ertapenem
2. Hyoscyamine
3 Lactated Ringer's
4 Pancrelipase
5 Promethazine
Correct answer:
Lactated Ringer's

Explanation
This patient is presenting with an episode of acute pancreatitis. Pancreatitis is characterized by epigastric pain, nausea, and vomiting. Many other acute abdomen conditions may present similarly. Significant elevations of lipase and amylase, as well as CT evidence of pancreatic inflammation, fluid collections, and/or necrosis, will establish the diagnosis. Pancreatitis can be acute or chronic, and those with chronic disease may have periodic acute flares. In acute pancreatitis, early fluid resuscitation is one of the "few medical interventions that appears to affect outcome," so (of the choices listed) administering lactated Ringer's (or saline) would be the best answer for this patient. Also, ensuring the patient has "nothing by mouth" (NPO) until symptoms decrease is standard treatment.

Ertapenem is a carbapenem antibiotic, indicated for complicated intra-abdominal, skin, and urinary tract infections. Although this patient's white blood cell count (WBC) is elevated, the primary process is not infectious. The WBC increases in inflammatory states. Rarely are antibiotics needed in acute pancreatitis.

Hyoscyamine is an anticholinergic medication, with a wide range of uses, including many gastrointestinal conditions. It may be used for reducing spasm in irritable bowel syndrome and for biliary colic. Before the test results pointed to pancreatitis, as in this patient's diagnosis, biliary colic and obstruction would be on the differential. However, hyoscyamine would not be a primary treatment for pancreatitis.

Pancrelipase is a digestive enzyme, taken with meals, and indicated for pancreatic insufficiency, a common complication of many years of chronic pancreatitis. This patient may be at risk for future pancreatitic disease, but the pancrelipase would not benefit him at this time.

Promethazine is a common antiemetic with antihistamine and anticholinergic properties. Although it may be reasonable to consider antiemetic treatment for a patient with acute pancreatitis, it is an adjunct treatment and does not have a vital impact on the course of the disorder.
Correct answer:
Celiac disease

Explanation
Celiac disease is a hereditary disorder caused by gluten intolerance. Gluten is found in wheat, rye, barley, and oats. Patients may be asymptomatic or may present with diarrhea, abdominal discomfort, distention, and steatorrhea (stools are pale, malodorous, and difficult to flush because they float on the toilet water). Patients may have anemia from iron and folate deficiencies, osteomalacia and bone pains from calcium deficiency, and edema from hypoproteinemia. Diagnosis is confirmed by a small intestine mucosal biopsy which shows a flat mucosa due to villous atrophy, and by subsequent improvement on a gluten-free diet. Specific treatment includes a gluten-free diet.

Whipple's disease mainly affects men; it is caused by the bacterium Tropheryma whippelii. It is a multisystemic disease that affects the small intestines, joints, brain, heart, and eyes. Patients can present with diarrhea, steatorrhea, abdominal pain, weight loss, and joint pains. On examination, patients may be pale with lymphadenopathy. Histological examination of a small bowel mucosal biopsy reveals PAS-positive, foamy macrophages. Treatment is with trimethoprim-sulfamethoxazole or chloramphenicol.

Short bowel syndrome is usually the result of surgical resection of the intestines or a jejunoileal bypass. The malabsorption is a result of inadequate absorptive surface. Malabsorption of vitamin B12 results in paraesthesias; malabsorption of calcium results in bone pain and carpopedal spasms.

Tropical sprue is an acquired disease that affects both visitors and natives of tropical areas (e.g., the Caribbean and South India). Its etiology is unknown. Patients usually present with diarrhea and weight loss; they report that their stools are soft and bulky (steatorrhea). They may also develop deficiencies of folate and cobalamin. Stool microscopy should be done to look for cysts and trophozoites. Histological examination of a small bowel mucosal biopsy aids in making the diagnosis. Treatment is with tetracycline or oxytetracycline.

Lactose intolerance results from a deficiency of lactose which is a disaccharidase in the mucosal cells of the small intestine; it splits the disaccharide lactose into glucose and galactose. Patients complain of borborygmi, flatulence, nausea, abdominal cramps, pain, and diarrhea after ingesting lactose-containing food (e.g., milk). Treatment includes following a lactose-free diet.
Correct answer: Adenocarcinoma of esophagus

Barrett's esophagus is an abnormality of the distal esophagus characterized by the replacement of normal squamous epithelium by metaplastic columnar epithelium with goblet cells, as a result of continuous inflammation most commonly from acid reflux. This is a premalignant condition with potential to develop into adenocarcinoma over time. Surveillance with screening endoscopy and mucosal biopsy is indicated to detect early onset of high grade dysplasia, which is then treated with esophagectomy. Failure to be compliant with the screening regimen may lead to adenocarcinoma of the esophagus, which has a poor prognosis as it presents late.
Achalasia of the cardia is a motility disorder resulting from degeneration of ganglion cells in the esophagus and lower esophageal sphincter. Barrett's esophagus does not predispose to this condition.

Esophageal stricture is a distinct complication of long-standing GERD and may co-exist with Barrett's esophagus, since both have the same etiological factor, namely, reflux esophagitis. However, one does not necessarily predispose to the other, and in this patient absence of dysphagia makes the possibility of a stricture unlikely.

Squamous cell carcinoma of esophagus is as frequent as adenocarcinoma, but risk factors are smoking and drinking alcohol, not Barrett's esophagus.

Dysphagia lusoria occurs when an anomalous blood vessel crosses behind the esophagus causing extrinsic obstruction. The most common vessel is the right subclavian artery.
Correct answer: Thrombotic microangiopathy

Based on the symptoms in the case, the patient is diagnosed with hemolytic uremic syndrome (HUS). E coli serotype O157:H7, which causes more than 80% of infections leading to HUS, produces shiga-like toxin. Toxin enters the circulation and causes the endothelial injury with the formation of arteriolar and capillary thrombi and red cell fragmentation. Thrombi damage glomerular filtration system in the kidneys and cause kidney failure; therefore, the progressive renal failure in HUS is the result of microangiopathic non-immune hemolytic anemia, and thrombocytopenia and the main mechanism is endothelial injury.

Consumptive coagulopathy,which is also knowndisseminated intravascular coagulation (DIC), is a result of a pathological activation of coagulation cascade that causes intravascular clot formation. Signs and symptoms depend on the organ involved, but the most common is bleeding due to the consumption of platelets and clot factors in the blood. Consumptive coagulopathy is rarely seen in HUS.

Autoimmune mechanism can also cause coagulopathy with bleeding symptoms, but the formation of autoantibodies is not the characteristic of the hemolytic uremic syndrome.

Focal segmental glomerulosclerosis is caused by a variety of factors (infections, inflammations, toxins, hemodynamic and genetics) that cause nephrotic syndrome. Proposed mechanisms are either primary alteration of epithelial cells (idiopathic, viral-associated, drug-induced, and genetic) or secondary to reduction in nephron mass or hemodynamic adaptation (obesity, a single kidney, renal dysplasia/agenesis, reflux nephropathy, sickle cell disease, hereditary nephropathies, and other primary glomerular diseases). It is not characteristic for the HUS.

In acute interstitial nephritis, renal tubular cells dysfunction is caused primarily by a hypersensitivity reaction to drugs or by infection. In hemolytic uremic syndrome, the initial damage is in the vascular endothelium.
Correct answer: Alpha-fetoprotein (AFP)

Alpha-Fetoprotein is a normal fetal serum protein synthesized by the liver, yolk sac, and gastrointestinal tract that is similar to albumin. It is a major component of fetal plasma, but its primary clinical utility is in diagnosing hepatocellular carcinoma (HCC). AFP is also elevated in testicular germ cell tumors containing embryonal or endodermal sinus elements. A positive marker value is highly sensitive in indicating HCC relapse or response of existing HCC to treatment. The AFP is less frequently elevated in other malignancies such as pancreatic cancers, gastric cancers, colonic cancers, and bronchogenic cancers. AFP is rarely elevated in healthy persons, but mild AFP elevation can occur in certain liver diseases, especially acute viral or drug induced hepatitis and conditions associated with hepatic regeneration.

Carcinoembryonic antigen (CEA) is a protein found in many types of cells but associated with tumors and the developing fetus. Although CEA was first indentified in colon cancer, an abnormal CEA blood level is specific neither for colon cancer nor for malignancy in general. Elevated CEA levels are found in a variety of cancers other than colonic, including pancreatic, gastric, lung, and breast. Elevated CEA levels can also be detected in benign conditions, though, including cirrhosis, inflammatory bowel disease, chronic lung disease, and pancreatitis.

CA-125 is an antigen present in over 80% of nonmucinous ovarian carcinomas. In the setting of ovarian carcinoma, CA-125 often follows the patient's clinical course. With surgical resection or chemotherapy, the level correlates with patient response. It should be noted that the CA-125 level may be elevated in other cancers, including endometrial, pancreatic, lung, breast, and colon cancer, and in menstruation, pregnancy, endometriosis, and other gynecologic and non-gynecologic conditions.

CA19-9 is a monoclonal antibody generated against a colon carcinoma cell line. CA19-9 is found it to be elevated in 20-40% of cases of gastric cancer, 20-40% of colon cancer, and 70-90% of pancreatic cancer. It may be able to differentiate benign from malignant pancreatic disease, but this utility has not yet been established.

Galactosyl Transferase II, an isozyme of galactosyl transferase, has been shown to be elevated in a variety of malignancies, predominantly gastrointestinal. In colon cancer, its level correlated with the extent of disease and disease progression. In pancreatic cancer, Galactosyl Transferase II proved to be more sensitive and specific in distinguishing benign from malignant disease than CEA.
Correct answer: Barium esophagography

This patient's most concerning diagnosis is a malignant esophageal lesion. However, nonmalignant etiologies may also be responsible for this presentation.

A barium esophagography should be obtained as the first study to evaluate dysphagia. In patients with progressive esophageal dysphagia, a radiographic barium study is used to differentiate between mechanical lesions (such as peptic stricture and esophageal cancer) and motility disorders (such as achalasia). The sensitivity of barium radiography for detecting esophageal strictures is greater than that of endoscopy. Barium studies also provide an assessment of esophageal function and morphology that may be undetected on endoscopy. Hypopharyngeal pathology and disorders of the cricopharyngeal muscle are better appreciated on radiographic examination, particularly with videofluoroscopic recording. Either a positive or a negative study is usually followed by an endoscopic evaluation; it is done in order to clarify findings in the case of a positive examination or to add a level of certainty in the case of a negative one.

A chest X-ray may reveal adenopathy, a widened mediastinum, metastatic lesions to the lungs or bone, or signs of tracheoesophageal fistula, such as pneumonia. It will not, however, reliably identify esophageal lesions.

Endoscopy with biopsy establishes the diagnosis of esophageal carcinoma with high reliability. It is the study of choice for evaluating persistent heartburn, odynophagia, and structural abnormalities detected on barium esophagography.

Ambulatory esophageal pH monitoring is the preferred study for documenting acid reflux. It is indicated in the following clinical scenarios: for patients being considered for anti-reflux surgery with a normal endoscopy; in patients with a normal endoscopy but with continued reflux symptoms following therapy with a proton pump inhibitor; and in the evaluation of atypical reflux symptoms that continues despite anti-reflux therapy.

Patients with alarm symptoms, such as unexplained weight loss, odynophagia, jaundice, recurrent vomiting, blood loss, a palpable mass or lymph node, or a family history of GI malignancy, should have diagnostic testing performed promptly.
Laparoscopic fundoplication

Laparoscopic fundoplication leads to long-term relief for about 90% patients with gastroesophageal reflux disease (GERD). The most common technique is the Nissen procedure (i.e., suturing the gastric fundus to the lower esophagus throughout the full esophageal circumference). Side effects are uncommon and mostly minor: abdominal bloating, inability to belch, paraesophageal herniation, dysphagia, and diarrhea. Barrett's esophagus sometimes disappears after fundoplication, but this procedure does not eliminate the risk of dysplasia and carcinoma in all patients. Therefore, endoscopic screening after the procedure is mandatory.

The other major therapeutic option for patients with GERD is long-term proton pump inhibitors (PPIs). Patients treated with these drugs have esophageal healing and symptom relief rates comparable to those of operated patients. However, about 30-40% of patients only improve after dose escalation. Therefore, PPI users take longer on average to achieve symptom control. Esophagectomy would be an option if there were an esophageal carcinoma or high-grade dysplasia, particularly in younger patients without significant comorbidities. The decision to operate must take into account the patient's general health and comorbidities, which are common given that Barrett's esophagus often occurs in elderly patients. However, advanced age by itself does not contraindicate esophagectomy.

Several endoscopic techniques (e.g. mucosectomy, laser ablation, and photodynamic therapy) have had some success in the treatment of Barrett's esophagus. However, complication such as bleeding after extensive mucosectomies, laser perforations, and skin burns from photoporphyrin-induced photosensibility are common. Incomplete mucosectomies, after which small areas of high-grade dysplasia or even cancers remained in the esophageal wall, have also been documented.

Radiofrequency thermal coagulation of the esophageal wall (Stretta procedure) consists of using a special catheter to induce controlled heating of the esophageal wall at the level of the gastroesophageal junction. This induces fibrosis and contraction of the gastroesophageal junction, which increases sphincter pressure and improves reflux. However, recurrence is high after this procedure (about 50%), and it cannot be performed when there is a hiatal hernia, as in this case.
Correct answer: Intravenous hydration and antibiotics

The presence of fever, jaundice, and right-upper quadrant pain defines Charcot's triad, which is the classical presentation of acute cholangitis. Reynolds' pentad consists of Charcot's triad plus sepsis/shock and mental status changes. 95% of patients presenting with these syndromes have common duct stones.

In most cases, there is a favorable response to conservative treatment, which consists of interrupting oral feeding, analgesia, intravenous hydration, and antibiotics. The following antibiotic schemes can be used:

A third-generation cephalosporin + an aminoglycoside
Piperacillin or ampicillin + metronidazole + an aminoglycoside
Monotherapy with imipenem, meropenem, mezlocillin, ampicillin-sulbactam, ticarcillin-clavulanate, or piperacillin-tazobactam
After clinical improvement, cholecystectomy is undertaken in the first few days after the initial hospitalization in order to avoid recurrence. Surgery is warranted because the recurrence rate is as high as 10% per year in patients whose gallbladders are not removed. The use of cholestyramine is another risk factor for recurrence in this patient, and gallbladder calcifications (porcelain gallbladder) are thought to pose a risk of malignization and are considered an indication for cholecystectomy.

When the disease progresses despite the initial conservative treatment (i.e., there is worsening fever, leukocytosis, abdominal pain, and guarding), emergency biliary drainage is warranted because of the possibility of gallbladder perforation or gangrene. Some possible approaches are cholecystectomy (conventional or laparoscopic), cholecystostomy, or percutaneous drainage. The latter 2 are usually reserved for sicker patients, who are less likely to tolerate surgery well.
Correct answer: Resection of the bowel

Intussusception is a common cause of childhood intestinal obstruction in which there is invagination of a segment of the bowel (usually small bowel) into the lumen of a more distant bowel segment. Peristalsis causes the invaginated segment, called the intussusceptum, to be carried distally and along with it the mesentery and vessels also become involved with the intraluminal loop. This segment is squeezed within the engulfing segment, which is called the intussuscipiens.

Most often the cause is idiopathic. However, in neonates and in children older than 3 years, a mechanical lead point is usually the cause. Lead points are nonidiopathic causes of intussusception and include Meckel's diverticulum, lymphoma or leukemia of the bowel, Peutz-Jeghers syndrome, polyps, lipomas, post-abdominal trauma, and Henoch-Schönlein purpura.

Bowel resection and primary anastomosis are required when pathological lead points are present. Also, surgery may be required when intussusception occurs outside the ileocolic region. Delay in presentation also necessitates surgery, as reduction by air or contrast would be ineffective or associated with complications.

Air enema reductions are less likely to succeed in patients less than 3 months old and in those more than 3 years old.

Supportive therapy by rehydration is not the definitive management. Adequate rehydration and stabilization are required for all cases of intussusception.

Reduction by contrast enema and observation for spontaneous reduction would not be beneficial in a child with a lead point presenting late.
Correct answer: Physical examination

Most likely this patient is suffering from an anal fissure. Anal fissures most often affect infants, as well as middle-aged individuals. The majority of fissures are considered primary and caused by local trauma such as passage of hard stool, prolonged diarrhea, vaginal delivery, or anal sex. Presentation of anal fissures is a tearing pain accompanying bowel movements as well as bright red rectal bleeding that is limited to a small amount noted on the toilet paper or surface of the stool. The patient described all of these components. Patients will also complain of perianal pruritus or irritation, which he also admits to experiencing.

The best diagnostic approach to confirming an anal fissure is usually based on the history of pain with defecation, as well as simply the physical examination finding of a superficial tear noted in the anoderm; this type of tear, no matter if located posteriorly or laterally, is considered a pathognomonic feature of an acute anal fissure. The examiner should carefully spread the buttocks apart and examine the area gently.

Digital rectal examination, or anoscopy, is not recommended for diagnosis of this condition due to the fact that patients will not be able to tolerate them due to the severe pain they will be experiencing from the anal fissure.

Sigmoidoscopy or colonoscopy are both inappropriate diagnostic tools in this patient case scenario. These studies should only be considered if there is presence of otherwise unexplainable rectal or gastrointestinal bleeding. Either a sigmoidoscopy or a colonoscopy can assist in evaluating the corresponding source of bleeding.
Correct answer:
Staphylococcus aureus

Staphylococcal food poisoning results from the ingestion of heat-stable enterotoxin B. An acute clinical syndrome of salivation, vomiting, and abdominal cramps is followed by watery diarrhea. The onset of vomiting and nausea within the 1st hours following ingestion is characteristic of staphylococcal intoxication (refer to the table). S. aureus are tolerant of high-salt concentrations, making salted meats (e.g., ham) a common source of intoxication.

Clostridium perfringens produces several enterotoxins that cause diarrhea. 1 of the most common agents of food-borne illness, it is transmitted primarily by meat and poultry. Symptoms include watery diarrhea, vomiting, and abdominal cramps (usually without fever). Unlike the rapid onset of S. aureus, symptoms of clostridial food poisoning occur 12 - 18 hours after ingestion.

Clostridium difficile is also capable of producing an enterotoxin and a cytotoxin, and it is not associated with food-borne illness. This organism causes colitis frequently associated with antibiotic use. Patients, usually the elderly or hospitalized, present with mild to moderate diarrhea and abdominal cramps; they may develop more serious illnesses such as pseudomembranous colitis or an acute abdomen.

Shigella causes enteritis within 12 - 18 hours after ingestion of as few as 200 organisms. This clinical syndrome consists of acute onset fever and abdominal cramps which are followed by an enterotoxin-mediated acute onset of copious watery diarrhea. Within 24 - 48 hours, dysentery occurs as a result of mucosal invasion. Shigella sonnei is the most common etiologic agent of shigellosis in industrialized countries.

Yersinia enterocolitica is transmitted by milk, water, or solid foods; it is often outbreak-associated. Patients experience diarrhea, fever, and abdominal pain 16 - 72 hours after consuming food. Y. enterocolitica may mimic acute appendicitis as a result of mesenteric adenopathy or terminal ileitis. Refer to the information below.

Incubation period (hours); clinical syndromes; likely organisms:

1 - 8; nausea and vomiting; Staphylococcus aureus, Bacillus cereus (emetic toxin), and Norwalk virus

8 - 12; diarrhea and abdominal cramps; Bacillus cereus (enterotoxin) and Clostridium perfringens

12 - 72; diarrhea, fever; mucus, dysentery, as well as neurologic and extraintestinal symptoms; Salmonella enteritidis, Escherichia coli, Campylobacter jejuni, Shigella, Clostridium botulinum, and Listeria monocytogenes
Correct answer: Positive transglutaminase antibodies

Dermatitis herpetiformis produces erythematous, pruriginous bullous lesions on the extensor surfaces of the elbows, knees, scalp, neck, and buttocks. These lesions, sometimes called skin celiac disease, are found in 10% of patients with celiac disease, and are highly specific for it; nearly all patients with dermatitis herpetiformis have histologic evidence of celiac disease on small-bowel biopsy. However, in many cases the enteropathy is subclinical. Histopathologic examination of the bullae reveals deposits of anti-gliadin IgA antibodies in the basal membrane of the epidermis.

In most cases, the skin lesions involute after the patient is put on a gluten-free diet. However, this can take several months. In patients with severe pruritus, the mainstay of treatment is dapsone, which is highly effective in relieving this symptom. Second-line options include sulfasalazine and sulfapyridine.

Celiac disease can present with isolated nutrient deficiencies (e.g. iron, folate, B-complex vitamins), and therefore could explain this patient's iron deficiency anemia. As transglutaminase antibodies are found in 70% of cases of celiac disease, there is a high probability that this assay will be positive in this patient, whether or not the anemia is caused by celiac disease.

The more florid presentation of celiac disease - with overt steatorrhea, weight loss, and malnutrition - is becoming less common in the United States. Therefore, the physician must be aware of the possibility of celiac disease in patients with milder symptoms, such as occasional diarrhea with bulky stools, bloating, or more rarely, isolated micronutrient deficiencies.

Anti-Saccharomyces (ASCA) antibodies are associated with Crohn's disease (60-70% of patients are positive). Lesions in these patients include oral aphthous ulcers, pyoderma gangrenosum, and rheumatoid nodules, as well as signs of non-cutaneous disease (e.g. enterocutaneous fistulas, malnutrition, micronutrient deficiencies).

A 99mTc-red cell scintigraphy could be positive because occult gastrointestinal bleeding is still a possibility. Causes of a negative iron balance include:

Deficient intake, which is most prevalent in poor countries.
Pregnancy.
Chronic bleeding (gynecologic, gastrointestinal, hematuria).
Iron deposition in tissues (e.g., cardiac hemosiderosis)
The deficiency in hemoglobin synthesis in the bone marrow tends to cause a compensatory rise in erythropoietin levels, which produces erythroid hyperplasia in the bone marrow. Other findings include absence of sideroblasts and stainable iron in the bone marrow's reticular network. However, bone marrow biopsy has been largely abandoned for diagnosis of iron-deficiency anemia because of interobserver variability and the availability of less invasive tests of iron status, particularly serum ferritin and total iron binding capacity (TIBC).