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Terms in this set (124)

Correct answer:
Distended neck veins with prominent a or v waves

Explanation
The correct response is distended neck veins with prominent a or v waves.

This patient's diagnosis is chronic cor pulmonale. Cor pulmonale is a change in the structure and function of the right ventricle and is caused by a primary disorder of the lungs or the pulmonary blood vessels. It 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, cardiomyopathy, ischemic heart disease, and congestive heart failure. As opposed to the pulmonary nature of Cor Pulmonale, in heart failure the etiology is cardiac. The heart doesn't pump blood with normal efficiency; subsequent organ congestion is caused by cardiac-related elevation of pulmonary or systemic venous pressures.

The finding of a systolic ejection murmur located at the aortic valve area most likely suggests a diagnosis of aortic stenosis.
Correct answer:
Echogenic bowel

Explanation
Echogenic bowel, which is sometimes seen on antenatal ultrasound (US) that is done during the 2nd trimester, may be a marker for aneuploidy, congenital infections such as toxoplasmosis and cytomegalovirus, rare intestinal disorders, and cystic fibrosis. However, most infants who exhibit this finding are normal, and most will pass stool by 48 hours. A delay should prompt a repeat exam on abdomen and rectum as well as assessment of adequacy of feeding. If the delay persists beyond 48 hours, a barium enema to evaluate for Hirschsprung's disease is indicated, and a consult for rectal biopsy should be considered. Newborns with cystic fibrosis also may present with meconium plug syndrome, a usually transient disorder of the newborn colon causing delayed passage of meconium and intestinal dilatation, although meconium ileus is more frequent and characteristic

Choroid plexus cysts may be commonly seen on US prior to 24 weeks' gestation. There is a possible association of ultrasound detected choroid plexus cyst and chromosomal problems, such as trisomy-18 and trisomy-21. If it is not associated with other anomalies, they are unlikely to be of any significance. Follow-up is not needed if results of the physical examination are normal.

An echogenic intracardiac focus or a bright spot seen near the papillary muscle of the left ventricle has a correlation with trisomy-21, but it is a normal finding in most cases. If there are no clinical findings suggestive of Down's syndrome, or there is a normal antenatal karyotype, the family can be reassured that no further testing is needed.

Mild enlargement or asymmetry of the cerebral ventricles might have some significance. The obstetric service will usually have performed serial US, karyotype, and cytomegalovirus and toxoplasmosis testing. A physical exam and cranial US should be done after delivery to document the brain anatomy and size of the ventricles. Any abnormal findings should be referred for consultation. A mild degree of ventricular enlargement often represents normal variation; however, if enlarged or asymmetric, it may also place an infant at risk for neurodevelopmental problems.

Hydronephrosis is found on 4.5% of pregnancies. Generally accepted criteria for fetal hydronephrosis are greater than 4 mm anteroposterior diameter renal pelvic dilatation in the second trimester, and greater than 7 mm dilation in the third trimester. Postnatal US should be done on infants who meet the criteria, although normal findings do not rule out vesicoureteral reflux. 10-30% of newborns with antenatal hydronephrosis have vesicoureteral reflux and are at risk of renal scarring from urinary tract infection.
Correct answer:
Erythromycin

Explanation
The correct response is erythromycin.

A clinical syndrome that includes low-grade fever, malaise, and nonproductive cough is characteristicof atypical pneumonia. The highest prevalence of infection is in the young, with children older than 5 years and young adults at most risk. Atypical pneumonia is most often caused by infection with Mycoplasma pneumoniae, Chlamydia pneumoniae, and Legionella pneumophila. Outpatients without coexisting illness, or patients over 60 years of age, are unlikely to have pneumonia caused by these 3 organisms. Macrolide antimicrobials provide suitable empiric therapy for atypical pneumonia.

Erythromycin and tetracycline have been shown in clinical trials to be efficacious for the treatment of pneumonia caused by M. pneumoniae. Fluoroquinolones have been shown effective in M. pneumoniae infections. β-Lactams are ineffective therapies because M. pneumoniae lack a cell wall.

C. pneumoniae should be suspected in atypical pneumonia, particularly when accompanied by pharyngitis. Large, controlled trials comparing antimicrobial agents have not been conducted, but erythromycin and other macrolides, tetracycline, and fluoroquinolones all appear effective.

L. pneumophila causes 1% to 15% of atypical pneumonia cases. The presentation of pneumonia caused by this agent is varied and nonspecific. Determination of L. pneumophila susceptibility to antimicrobials has been hindered by a lack of suitable assays for intracellular pathogens. Erythromycin, which has been the drug of choice for treatment of atypical pneumonia caused by L. pneumophila, has limited effectiveness in some cases. Azithromycin and the quinolones, with superior intracellular levels, have proven effective by experience. The efficacy of azithromycin, erythromycin, and quinolones has not been compared in clinical trials of L. pneumophila pneumonia.

Streptomycin, not part of routine antimicrobial therapy for pneumonia, is part of combined therapy of tuberculosis; it may be used in combination with other agents for treatment of endocarditis, plague, tularemia, and brucellosis. Trimethoprim-sulfamethoxazole, sometimes used to treat acute exacerbations of bronchitis, is not effective against these organisms. Like amoxicillin, the cephalosporins have no activity against Mycoplasma.
Correct answer:
Skin sweat test

Explanation
The criterion standard for diagnosing cystic fibrosis (CF) is the skin sweat test. An elevation of the sweat chloride >60 mmol/L is typical of CF, but the diagnosis requires positive results on 2 separate occasions in a patient with a CF phenotype, or who has a sibling with CF. DNA analysis is currently not used for primary diagnosis due to the large number of mutations associated with CF. With so many variations, patients are most commonly diagnosed in infancy during the first 24 hours of life or the first 1-2 years, but a significant number are diagnosed after the age of 18. In recent years, every state has begun screening newborns for cystic fibrosis. If a genetic test or blood test suggests CF, the diagnosis is confirmed using a sweat test.

Pulmonary function testing in CF reveals increased residual volumes (RV) with decreased forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1). These changes are seen in obstructive disease and are not specific to CF.

CF is an obstructive lung disease that is progressive over time. Changes on chest X-ray seen in CF include hyperinflation in early-stage disease and bronchiectasis in late-stage disease. Although these changes are typical, they are not diagnostic for CF. Chest radiograph would identify an LRI, which is secondary to the primary disease of CF.

Sputum for culture and sensitivity would identify Pseudomonas aeruginosa, which is the typical organism seen in lower respiratory infections (LRI) associated with CF, and Burkholderia cepacia, which is pathogenic for CF when identified. These organisms identify LRI, which must be treated aggressively, but these occur secondary to the underlying etiology of CF, which is a mutation of the CF gene affecting the exchange of ions across epithelia in the airways, intestines, pancreas, and sweat ducts.

Alpha-1-antitrypsin deficiency (α1AT) is a genetic risk factor for early-onset COPD, but it would not account for the gastrointestinal pathology associated with CF.
Correct answer:
Supplemental oxygen and supportive therapy

Explanation
Respiratory syncytial virus (RSV) is the most important etiology of viral lower respiratory disease in infants and children; bronchiolitis is the most common manifestation. Infection is initiated in the upper respiratory tract and may spread to the lower tract; this causes obstruction of smaller airways by edema, necrotic tissue, and inflammatory cells. This leads to the typical findings of nasal discharge, cough, retractions, and wheezing or rhonchi. The peak incidence is during the first 2 years of life. In the United States, it occurs during the winter months in an epidemic fashion.

RSV is a member of the Paramyxovirus family. Complications include apnea in the very young and premature infant, pneumonia, croup, respiratory failure, otitis media, and dehydration.

The severity of bronchiolitis is diagnosed and assessed based on history and physical examination. Infants with mild bronchiolitis can be treated symptomatically at home. The following are the criteria for hospitalization in RSV bronchiolitis:

Age less than 12 weeks
Gestational age at birth of less than 34 weeks
Cardiopulmonary disease or immunodeficiencies
Wheezing and respiratory distress associated with oxygen saturation below 92 percent on room air (hypoxemia)
History of significant apnea before assessment.
The management in moderate-to-severe bronchiolitis involves supplemental oxygen therapy and supportive measures such as prevention of dehydration and respiratory support. Supplemental oxygen is the single most useful therapy, usually delivered via nasal prongs when oxygen saturation (SpO2) falls persistently below 90% in previously healthy infants. Mechanical ventilation may be needed for respiratory failure or severe apnea.

Ribavirin, an antiviral agent, has not been shown to reduce hospital stays or mortality. The agent is very expensive and may be teratogenic in humans. It may still have a place for treatment of infants at significant risk for complications (i.e., those with cardiopulmonary disease). Bronchodilators, such as albuterol, have not been shown to be effective; however, their empirical use in the setting of the hospitalized patient is still commonly seen. Likewise, corticosteroids are not effective, and their use is contraindicated. Antibiotics, such as ampicillin and cefotaxime, are not routinely used in the majority of patients, but they are indicated in cases of clinical evidence of superimposed bacterial pneumonia or acquired nosocomial infection during the hospitalization.
Correct answer:
You obtain a specimen for a Gram-stain and sputum cultures and prescribe erythromycin 500mg PO q6h for 14 to 21 days for an empiric therapy against Mycoplasma pneumoniae.

Explanation
The patient presents with symptoms of community-acquired pneumonia, not classic symptoms of a S. pneumonia infection. He is most likely immunocompetent, and your patient does not show the classical symptoms of being critically ill. Hospital admission is unnecessary.

In such a case, the best course of action is to obtain a Gram-stain and sputum cultures before any treatment is initiated. Then, an empiric treatment is started immediately with erythromycin; treatment should be focused on the most likely non-viral pathogen, which is either S. pneumoniae, S. aureus, or Mycoplasma pneumoniae. Erythromycin is effective against both pathogens, as well as other atypical organisms such as Chlamydia and Legionella. Have the patient drink plenty of fluids for adequate hydration, and supply oxygen if needed. This treatment should improve the patient's condition within a few days. If not, you will have the results of the sputum cultures available to adjust the antibiotic therapy accordingly. Fluoroquinolones are used if there are underlying comorbidities such as chronic lung disease. Doxycycline is another first-line choice in uncomplicated CAP.

Starting an empiric therapy immediately is wrong because cultures have to be collected before antibiotic treatment is initiated, or they may be useless. Vancomycin as the antibiotic of choice is wrong because an empiric therapy should be focused on the most likely pathogen. Erythromycin should be prescribed for Mycoplasma pneumoniae, and penicillin G or erythromycin (for penicillin-allergic patients) should be prescribed for the treatment of S. pneumoniae.
orrect answer:
Respiratory syncytial virus

Explanation
Respiratory syncytial virus (RSV) is the major cause of lower respiratory tract illness (i.e., pneumonia, bronchiolitis, and tracheobronchitis) in young children. The virus belongs to the Paramyxoviridae family and to the genus Pneumovirus. The virus is very contagious, and illnesses tend to peak in the winter or spring. Wheezing, rhonchi, rales, interstitial infiltration, and hyperinflation may be present on chest radiographs. Fevers are usually seen and range between 38° to 40° C. Hypoxemia may be profound in children. There is a need for the measurement of an infant's arterial oxygen saturation because clinically the illness is difficult to assess. Because of its contagious nature, infants in hospitals are placed in isolation in order to prevent outbreaks within the hospital. The diagnosis of RSV can be made with reasonable accuracy based on the clinical and epidemiologic findings. Confirmation can be made by viral isolation (unfortunately this is time consuming) or by rapid diagnostic tests based that are available and provide results in about 10 minutes. Ribavirin, an antiviral agent, is available for treatment of RSV in infants where the diagnosis is made in the early stages of the disease. It has been shown to decrease viral shedding and increase oxygenation.

Bordetella pertussis is a fastidious Gram-negative bacillus that is the causative agent of whooping cough. The organism is difficult to recover in cultures; PCR testing and DFA are the tests of choice, especially because results are acquired within hours instead of days. The organism specifically binds to ciliated epithelial cells. Since the nasopharynx is lined with ciliated epithelial cells, specimens obtained from this site are more reliable at obtaining valid results then any other specimen source. The infection is limited to the upper airways and pneumonia is a rare occurrence; thus, chest radiographs are usually normal. Children with whooping cough have paroxysms of coughing. When they gasp for breath the sound of this inspiration is the 'whoop' of whooping cough. Abnormal oxygen exchange is common and can cause the child to turn red, and sometimes blue. Repetitive coughing can lead to vomiting or choking on respiratory secretions.

Haemophilus influenzae is a small Gram-negative coccobacillus that requires 2 supplements for growth (factor X and factor V); it is non-motile; grows best at 5 - 10% carbon dioxide, and grows on chocolate agar (because of the availability of X and V factors), but not on BAP or MacConkey agar. Pneumonia due to H. influenzae Type b typically occurs in patients between 4 months and 4 years old, patients with primary lung disease, and patients that are alcoholics. Radiologic findings are those of a segmented, lobar, bronchopneumonic, or interstitial pattern (listed in descending order of frequency). Cavitation is rare. Pleural effusion occurs in about 50% of cases, and the fluid is usually found to be sterile when cultured.

Streptococcus pyogenes is a Gram-positive coccus; it is catalase-negative, beta-hemolytic on blood agar, and appears as chains on Gram stain. Definitive identification to distinguish it from other beta-hemolytic streptococci is the detection of its specific 'A' antigen by latex agglutination techniques. Pneumonia with this organism is extremely rare; it is associated in children with streptococcal pharyngitis, scarlet fever, and streptococcal pyoderma. Bacteremia is uncommon. It is universally sensitive to penicillin.

Corynebacterium diphtheriae is an aerobic, Gram-positive rod that is club-shaped. It causes the disease diphtheria. Selective media (cystine tellurite agar) is used for the isolation and identification of the bacteria; the organism produces black colonies. The organism produces a toxin (diphtheria toxin) that is the major virulence factor; it enters the circulation and inhibits protein synthesis in a variety of tissues (with heart, nerves, and kidneys being particularly targeted). The disease will usually occur in individuals that have not been properly immunized (especially children). On physical examination, the patient will have a pseudomembrane formed at the back of the throat. This pseudomembrane is composed of bacteria, fibrin, dead epithelial cells, and red and white blood cells. Aspiration of this pseudomembrane can cause death by suffocation. In unvaccinated children, the mortality rate is approximately 20%. Treatment is usually with both antibiotics and diphtheria antitoxin.
Correct answer:
Long-term, regular exercise training should be initiated.

Explanation
It is now generally accepted that regular physical activity should be an integral component of the treatment plan for individuals with cystic fibrosis (CF). Benefits include improved clearance of pulmonary secretions, slower decline of pulmonary function (e.g., of forced vital capacity), and better glycemic control and bone mineralization. A regular, long-term exercise program can maintain fitness even in the face of a decline in pulmonary function. The degree of exercise should be tailored to the tolerance and pulmonary function of the individual patient. Muscle strengthening, including the muscles of the chest wall, should be highlighted. Limitation of maximal exercise tolerance by symptoms does not appear to be a factor in mild to moderate pulmonary disease; it is only a factor when severe disease is present. Exercise capacity can be increased by exercise training in CF patients even with severe disease.

CF itself is not a contraindication to competitive sports. The degree to which an individual with CF will be able to compete depends upon the nature of the sport and pulmonary function, among other factors.

As indicated above, there are numerous benefits of physical activity and exercise training in patients with CF.

Even with severe pulmonary disease, physical activity can be of benefit in CF.

Parental encouragement of and parental participation in physical activity is important in initiating and maintaining a physical activity program in young CF patients.
Correct answer:
Cor pulmonale

Explanation
The most appropriate diagnosis in this scenario is cor pulmonale, often referred to as pulmonary heart disease. It occurs due to right ventricle structure and/or function alteration due to chronic lung disease, and it's usually triggered by pulmonary hypertension. Symptoms that present are due to the underlying pulmonary pathology. Dyspnea is the most common symptom, as well as abdominal pain and ascites. Signs found on physical assessment include tachypnea, elevated jugular venous pressure, hepatomegaly, and lower extremity edema. Evidence of prominent V waves in the jugular venous pulse may be found, as well as an RV heave palpable along the left sternal border or even in the epigastrium. Cyanosis is a late finding in the course of cor pulmonale. Diagnostically, ECG findings will display P pulmonale, right axis deviation, and right ventricle hypertrophy.

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:
Chest X-ray

Explanation
Any PPD skin test should be performed and interpreted only in healthy, asymptomatic individuals, with a negative chest X-ray for active tuberculosis (TB). A positive result is considered if the transverse diameter of the induration is greater then 15 mm for low-risk individuals, 10 mm for high-risk individuals, and 5 mm for HIV-positive individuals or close contacts of patients with active TB. A health care worker is considered a high-risk individual. In this case, the next step in the management is performing a chest X-ray to look for old or active TB lesions.

Patients with active TB should be placed on respiratory isolation for 2 weeks, or until 3 negative sputum results are obtained. They should follow the standard medication regimen for 6 or 9 months.

Prophylaxis with isoniazid for 6, 9, or 12 months should be considered for any patient with suspicion of latent TB (positive skin test result), because the risk to develop active TB is about 10% for the next 2 years. Current indications for prophylaxis are:

Any recent converters in the last 2 years from a negative skin test to a positive skin test, regardless of the patient's age
Any patient with a positive skin test who had TB or has an abnormal chest X-ray with signs of old TB lesions, regardless of the patient's age
Any patient younger then 35 years with a positive skin test and a normal chest X-ray
Reassurance would not be appropriate before obtaining a chest X-ray that would orient the management toward a diagnostic workup; it is also not appropriate before treatment for active TB or isoniazid prophylaxis. Sputum examination is not appropriate for an asymptomatic patient
Correct answer:
Silicosis

Explanation
Silicosis is the chronic fibrosing disease of the lungs produced by the prolonged and extensive exposure to free crystalline silica dust. It is the most prevalent occupational disease in the world. It requires years of exposure to crystalline silica to develop, as is found in mining, glassmaking, sand blasting, sand cutting, ceramics, glass manufacturing, and brickyards. It has a latency period of 20-30 years.

Pulmonary function reveals a restrictive pattern with decreased DLCO. Small and discrete nodules are present in chest x-rays of simple silicosis. In advanced cases, large masses of dense, fibrous tissue are seen. In about 5-10% of cases, the nodes will calcify circumferentially, producing so-called "eggshell" calcification.

Silica ingested by alveolar macrophages renders them ineffective, so a +PPD in these patients makes the diagnosis of latent tuberculosis infection, which should be treated with anti-tuberculosis medications. All patients with suspected silicosis should have yearly TB skin test and screening for lung malignancy.

Sarcoidosis is an idiopathic inflammatory disorder that may cause noncaseating granuloma formation in the lungs. Some patients report symptoms such as cough but others are asymptomatic. Calcified lymph nodes are not a common finding of the disease. Sarcoid is less likely given that the patient has a history of silica exposure.

HIV infection may result in reduced immunity and pneumonia, but pneumonia would not explain the calcified hilar lymph nodes.

Mesothelioma is a malignancy involving the pleural lining of the lung. It is typically associated with asbestos, not silica exposure. Chest x-ray typically shows pleural thickening and effusions but not calcified hilar lymph nodes.

Tuberculosis of the lungs results in the formation of caseating granulomas. Hilar lymph node enlargement may occur with acute presentations. Cavitations may also be present. In acute infections, other symptoms such as cough and weight loss are present.
Correct answer:
Cystic fibrosis

Explanation
Cystic fibrosis is one of the most common genetic disorders in the Caucasian population, with a carrier rate of approximately 1 in 28 Caucasians (1 in 31 Americans). It is inherited in an autosomal recessive fashion, and it is caused by mutations in the CFTR gene, which is located on the long arm of chromosome 7. The protein regulates Cl and Na transport across the epithelial membrane, and disruption leads to the formation and excretion of abnormally thick mucous, leading to severe pulmonary and digestive difficulties. Presence of the mucous in the lung leads to small airway obstruction. Infection is secondary; eventually, the bronchial walls become thick, the patient suffers from chronic hypoxemia, and muscular atrophy occurs. Due to pancreatic insufficiency, which is manifested as frequent passage of greasy, foul-smelling stool, abdominal protuberance, and deficiency of fat-soluble vitamins, affected individuals suffer from inadequate weight gain (apparent as early as 4 to 6 weeks of age) and growth retardation; these symptoms occur despite a good appetite. Patients also suffer from excessive sweating when stressed, and 10% develop insulin dependent diabetes. Average life span is early thirties. Although mutations in the CFTR gene have been identified, they are too numerous to provide a feasible DNA diagnostic test. However, a sweat test is available diagnostically.

Alagille syndrome is a genetic disorder inherited in an autosomal dominant fashion. It is caused by mutations in the JAG1 gene, localized to chromosome 20p12. It is characterized by liver, heart, skeletal, and ophthalmologic abnormalities. Liver problems are usually observed within the first 3 months of life, and they include cholestasis, jaundice, pruritus, and paucity of bile ducts. Heart problems range from murmur to peripheral or pulmonic stenosis or tetralogy of Fallot. Skeletal and ophthalmologic abnormalities do not usually cause medical problems, but they can aid in diagnosis. They include butterfly vertebrae and posterior embryotoxon. A characteristic facies is also common among affected individuals. The face is shaped as an inverted triangle - prominent forehead, deep-set eyes, moderate hypertelorism, pointed chin, and a straight nose with a bulbous tip. These individuals also tend to show pancreatic insufficiency and growth failure. Diagnosis is based on clinical findings, although deletion studies are available clinically. Direct mutation analysis is available on a research basis only.

The most common symptoms seen in 22q11-deletion syndrome include congenital heart disease (particularly conotruncal malformations), palatal abnormalities, hypocalcemia, immune deficiency, characteristic facial features, and learning difficulties. Most patients affected with this deletion syndrome can be diagnosed with fluorescence in situ hybridization (FISH) using DNA probes from the DiGeorge chromosomal region (DGCR). 94 percent of patients with the deletion have had a de novo event. 6 percent of patients inherit the deletion from a parent.

Galactosemia is an autosomal recessive disorder of carbohydrate metabolism. Symptoms include hypoglycemia, jaundice, failure to thrive, vomiting, and sepsis. It is a progressive disorder if not diagnosed and treated, and it eventually leads to intellectual disability and death. In MOST states of the United States, this deficiency is included in newborn screening, and it can therefore be recognized before the infant leaves the hospital.

William's syndrome is a microdeletion syndrome caused by a contiguous gene deletion of a critical region on chromosome 7q11. Patients share a common phenotype that includes mild intellectual disability, cardiovascular disease, connective tissue abnormalities, and a distinctive facies. The most common cardiac finding is supravalvar aortic stenosis. Connective tissue abnormalities include joint laxity, loose skin, hoarse voice, and tendency for hernia. Affected individuals usually have bitemporal narrowing, periorbital fullness, a short nose with a bulbous tip, malar hypoplasia, long philtrum, full lips, wide mouth with a small lower jaw, and prominent earlobes. Teeth tend to be small and widely spaced. Affected individuals tend to be overly friendly, anxious, and have attention deficit disorder. Growth pattern shows prenatal growth deficiency, failure to thrive in the first 4 years, and a brief pubertal spurt. Average height as an adult is less than the third percentile.
Correct answer:
Ceftriaxone 1 g IV Q24H and azithromycin 500 mg IV Q24H

Explanation
The correct response is ceftriaxone 1 g IC Q24H and azithromycin 500 mg IC 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, 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, 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 ceftriaxone and azithromycin covers gram-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, but 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. It would not have been highly recommended, however, 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 (MRSA).
Correct answer:
NSAIDs (non-steroidal anti-inflammatory drugs)

Explanation
The patient has pleuritis without effusion. Pleuritis is inflammation of the pleura; it may occur with or without effusion and is characterized by a sharp pain worsened by cough and inspiration. The pain may radiate to the shoulder if the diaphragmatic pleura is affected, and pressure around the area of inflammation may produce pain. Friction rub is heard on auscultation. The pleuritic chest pain causes shallow rapid breaths. The presentation with flu-like illness, malaise, absence of history of collagen vascular disease, and CXR findings makes the most likely etiology viral in this case. Pleuritis is treated with NSAIDs. Ibuprofen can be given in the dose of 400 - 800 mg orally 3 times daily for pain management in cases of pleurisy due to viral etiology. Other examples of etiologic agents are rheumatoid arthritis, systemic lupus erythematosus, radiation, pneumonia, acute pulmonary embolism, and pneumothorax.

Although a pulmonary embolism can cause pleuritis, the D-dimer assay is normal in this patient; no further evaluation by imaging modalities, such as V/Q scanning or CT scanning, is warranted in a person at low risk of a pulmonary embolism. Common risk factors are prolonged immobilization, congestive heart failure, stroke, pelvic fracture, obesity, and malignancy. Patients present with sudden shortness of breath, tachycardia, tachypnea, hypotension (if massive), and low-grade fever. There is hypoxemia on blood gas, and CXR may be normal or show a region of oligemia and wedge-shaped density. Treatment is by anticoagulation.
Correct answer:
Reduced intravascular oncotic pressure

Explanation
Pleural effusion, 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:
Immediate intubation

Explanation
Epiglottitis, an infection most commonly caused by Haemophilus influenzae, is characterized by inflammation and rapidly progressive edema of epiglottis and contiguous tissue. Children of ages 2 to 7 years who missed some immunizations are prone to this infection during winter. The use of H. Influenzae vaccine caused a marked decrease in the incidence of this disease. Drooling, hoarseness, high fever, sore throat, the characteristic "sniffing dog" position and the rapidly progressive respiratory obstruction make the diagnosis a clinical one. Because of the rapid evolution to airway occlusion, a patient with suspicion of epiglottitis should receive immediate intubation under anesthesia in the operating room. Emergency cricothyrotomy is the alternative if there are no conditions to ensure a patent endotracheal airway. Intravenous ceftriaxone or cefuroxime provide an empiric antibiotic coverage.

Lateral neck X-ray or other radiological examinations for a patient with epiglottitis in respiratory distress are considered a delay in the management. The classic "thumbprint sign" indicates obstruction of the airway by the swollen epiglottis. Throat examination is contraindicated in epiglottitis because it precipitates a laryngospasm. A cherry-red, swollen epiglottis on direct visualization during the intubation confirms the diagnosis.

Aerosol ribavirin is used for patients with respiratory syncytial virus bronchiolitis, a severe infection in infants that can also progress to respiratory failure. Patients experience wheezing, rales, prolonged expiratory phase, rhinorrhea, nasal congestion, and respiratory distress. Antigen testing from nasal secretions provides a rapid diagnosis. Polyclonal and monoclonal antibodies are used for prophylaxis in wintertime.

Nebulized racemic epinephrine is indicated in croup, an upper respiratory infection caused by parainfluenza virus. Nebulized racemic epinephrine has no effect in epiglottitis.
Correct answer:
Voriconazole

Explanation
The patient's symptoms are most indicative of a pneumonia-like infection, most likely stemming from the fungus Aspergillus fumigatus.

Voriconazole is considered a first-line treatment for invasive aspergillosis. It is available in both oral and intravenous forms and has good bioavailability. It is hepatically cleared and has less toxicity compared to amphotericin B, which is also used to treat aspergillosis.

Vancomycin was first isolated from Streptomyces orientalis. It was introduced in 1958 for the growing problem of penicillin-resistant staphylococci. By 1960, it was superseded by Methicillin. In the last 10 years, the use of vancomycin has significantly increased due to the emergence of Methicillin-resistant Staphylococcus aureus (MRSA), Clostridium difficile, and enterococcal disease. The antibiotic complexes to the D-alanyl-D-alanine portion of the growing cell wall and inhibits peptidoglycan and transpeptidation. Vancomycin is poorly absorbed orally and thus is given by IV. It is widely distributed throughout the body. Adverse reactions are not common; however, ototoxicity is a serious concern and blood levels must be monitored.

Erythromycin is a macrolide and was originally isolated from a strain of Streptomyces erythreus in 1952 by McGuire, et. al. Erythromycin interferes with protein synthesis. Oral erythromycin base is destroyed by the acid in the stomach; as a result, it is manufactured with an acid-resistant coating that is dissolved in the duodenum, which is where it is liberated in its active form. The antibiotic is distributed widely in tissue and is concentrated in the liver and spleen. Levels of the antibiotic persist in tissue longer than in serum, and the antibiotic does not enter the CSF in the absence of inflammation. Toxicity is low. Erythromycin was formerly the drug of choice for the treatment of Legionella; but now azithromycin is preferred.

Tetracycline compounds were first described in the 1940s. Tetracyclines inhibit protein synthesis. They are usually used orally and are absorbed by the stomach. Dairy products and aluminium or magnesium salts such as antacids will negatively affect absorption by the stomach; therefore, they must be avoided. The antibiotic distributes widely in the body, with the exception being the central nervous system. Gastrointestinal disturbances can be a side effect of the antibiotic. However, a far more serious side effect occurs due to the ability of the antibiotic to bind to divalent cations such as magnesium and calcium. Because of this, they are rarely used in children. In children, they bind to developing teeth and bone, resulting in gray or brown teeth or affect long bone development. Tetracyclines are used for some "unusual" infections such as Mycoplasma, Ureaplasma, nocardiosis, plague, Rocky Mountain Spotted Fever, Scrub typhus, and Q fever.

Penicillin was first isolated from the fungus Penicillin notatum. Penicillin is the antibiotic that is considered to have ushered in the modern era of antimicrobials. The basic penicillin ring consists of a thiazolidine ring and a B-lactam ring, which is a 4-member ring with amide linkage joining carbonyl and nitrogen. The mechanism of action of penicillins is through penicillin-binding protein (PBP) binding with subsequent inhibition of transpeptidation reaction and activation of the autolytic enzymes. Administration is orally and IM or IV. Food interferes with absorption, so it should be given 1 hour before or 2-3 hours after meals. Penicillin is widely distributed in the body, but there is poor penetration in some spaces, such as the eye, pericardium, and cerebral spinal fluid. The antibiotic is considered non-toxic, but it can cause allergic reactions in 3-10% of the patient population. Penicillin is most active against Streptococcus spp., Neisseria gonorrhoeae, Treponema pallidum, and Listeria spp. It is used clinically to treat syphilis, Streptococcus pyogenes pharyngitis, and as a prophylactic agent after rheumatic heart disease.
Correct answer:
Order a CT angiography of the chest

Explanation
This patient's presentation suggests a pulmonary embolism. EKG findings include an S1Q3T3 pattern, sinus tachycardia, T wave inversion in leads V1 - V3, Right Bundle Branch Block, and low amplitude deflections.

Computed tomography angiography (CTA) is the initial imaging modality of choice for stable patients with suspected pulmonary embolism.The American College of Radiology (ACR) considers chest CTA to be the current standard of care for the detection of pulmonary embolism.

The echocardiogram generally has limited accuracy in the diagnosis of pulmonary embolism.

Few investigators have reported the feasibility of MRI in the evaluation of pulmonary embolism. However, the role of MRI is mostly limited to the evaluation of patients who have impaired renal function or other contraindications for the use of iodinated contrast material.

Prescribing a proton pump inhibitor is not appropriate at this time, as the clinical presentation suggests pulmonary embolism.

Thrombolytic therapy is not recommended for most patients. The role of thrombolytic therapy in the management of acute pulmonary embolism is controversial and has not demonstrated benefits in terms of reduced mortality rates or earlier resolution of symptoms when currently compared with heparin. The currently accepted indications for thrombolytic therapy include hemodynamic instability, such as systolic blood pressure less than 90 mmHg or systolic blood pressure drop of greater than 40 mmHg from baseline for at least 15 minutes, or right ventricular dysfunction demonstrated on echocardiogram. Thrombolytic therapy should not be used in patients with major contraindications due to bleeding risks.
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/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 identifying and avoiding the causative agent, 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, 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 2-4 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 pneumonia, 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:
Epiglottitis

Explanation
The most likely diagnosis is acute epiglottitis, as the child has presented with sudden high fever, sore throat, dysphagia, drooling, and dyspnea. Cough is usually absent in epiglottitis. The diagnosis is further supported by the presence of polymorphonuclear leukocytosis and lateral radiograph of the upper airway showing the "thumb sign," which is due to swelling and inflammation of the epiglottis and other supraglottic structures, especially the aryepiglottic folds.

Haemophilus influenzae type b is the most common cause of acute epiglottitis. Stridor is a late finding and suggests severe airway obstruction. Rapid airway management is essential and includes nasotracheal intubation and oxygenation. Examination of the throat with a tongue depressor should be avoided; it can precipitate a fatal laryngospasm.

Antibiotics commonly used for treatment are cefotaxime, ceftriaxone, or meropenem pending culture and sensitivity reports.

Laryngotracheobronchitis (Croup) usually starts with rhinorrhea, cough, and low-grade fever. After 2-3 days, the child develops the characteristic "barking" cough, hoarseness of voice, and a biphasic stridor. In epiglottitis, the stridor is inspiratory. Cough is usually absent in epiglottitis, as seen in the above patient. Temperature ranges from 102 degrees F to 104 degrees F. Croup is most commonly caused by viruses (influenza A and B, adenovirus, and respiratory syncytial virus). Throat examination shows moderately inflamed pharynx, rhinitis, and mild tachypnea with subcostal and intercostal retractions. Epiglottitis has a more acute and rapid course than croup. X-ray chest shows the characteristic narrowing of the subglottic region and is known as the "steeple sign."

Bacterial tracheitis is more commonly caused by Staphylococcus aureus. It often follows a viral upper respiratory tract illness. Mean age is between 5 and 7 years. After a few days, the child develops a brassy cough, biphasic stridor, high fever, and toxicity; unlike epiglottitis, the child can lie flat, does not drool, and does not have dysphagia. Bacterial tracheitis is a life-threatening emergency, as respiratory obstruction may develop due to mucosal edema and thick purulent secretions, which can be adherent and membranous. Bronchoscopy is both diagnostic and therapeutic because it shows the purulent secretion that can be mechanically debrided.

Retropharyngeal abscess occurs as a complication of bacterial infection of the upper respiratory tract and commonly occurs in children between 3 and 4 years of age. It occurs due to spread of infection from the upper respiratory tract to the retropharyngeal lymph nodes, which suppurate and form an abscess. It may present with fever, reduced mobility of the neck, stridor, dysphagia, and drooling. Child may have respiratory distress or obstructive sleep apnea. Physical examination can reveal bulging of the posterior pharyngeal wall. Cervical lymphadenopathy may also be present. Lateral neck radiograph shows a soft tissue bulge in the posterior pharyngeal wall.

Laryngomalacia is the most common congenital anomaly of the larynx. The infant commonly presents with inspiratory stridor, which is aggravated when the infant is crying, feeding, or is placed in supine position. It typically starts at 4 - 6 weeks of age. The infant is otherwise usually happy and thriving. Stridor is due to partial collapse of a flaccid supraglottic airway. Condition is self-limiting and resolves by 18 months of age.
Correct answer:
Compressive

Explanation
The correct response is compressive.

This patient has a clinical picture consistent with superior vena cava compression, which is not uncommon in the presence of a right upper lobe tumor.

Because the superior vena cava has a thin wall coupled with a low intravascular pressure, and is surrounded by rigid structures (i.e., sternum, trachea, right bronchus, aorta, pulmonary artery, and perihilar and paratracheal lymph nodes), it is relatively easy to compress. The subsequent obstruction to flow causes an increased venous pressure which results in interstitial edema and retrograde collateral flow.

An immunological reaction is not likely the cause of symptoms that are strongly suggestive of a well-defined compressive lesion.

This patient has a well-defined syndrome pointing towards specific organ involvement. Constitutional symptoms (e.g., fatigue, malaise, weight loss, fever, chills, and night sweats) are nonspecific manifestations of a vast number of diseases and conditions.

In the presence of a nearby primary tumor, it is unlikely that a metastatic tumor cell from a distant location would cause superior vena cava compression. Correct answer:
Compressive

Explanation
The correct response is compressive.

This patient has a clinical picture consistent with superior vena cava compression, which is not uncommon in the presence of a right upper lobe tumor.

Because the superior vena cava has a thin wall coupled with a low intravascular pressure, and is surrounded by rigid structures (i.e., sternum, trachea, right bronchus, aorta, pulmonary artery, and perihilar and paratracheal lymph nodes), it is relatively easy to compress. The subsequent obstruction to flow causes an increased venous pressure which results in interstitial edema and retrograde collateral flow.

An immunological reaction is not likely the cause of symptoms that are strongly suggestive of a well-defined compressive lesion.

This patient has a well-defined syndrome pointing towards specific organ involvement. Constitutional symptoms (e.g., fatigue, malaise, weight loss, fever, chills, and night sweats) are nonspecific manifestations of a vast number of diseases and conditions.

In the presence of a nearby primary tumor, it is unlikely that a metastatic tumor cell from a distant location would cause superior vena cava compression.
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:
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:
Pulmonary embolism

Explanation
Pulmonary embolism pain is often abrupt in onset and pleuritic in nature. It can last several minutes to a few hours. Cough and hemoptysis may also be present. There may be predisposing factors such as deep venous thrombosis or long periods of immobilization. Orthopedic surgery is also a risk factor for pulmonary embolism. On examination, patients are dyspneic and tachypneic with a tachycardia. Chest radiograph findings that are suggestive of a pulmonary embolus are the Hampton hump and the Westermark sign. The Hampton hump is a wedge-shaped opacity that has a tip pointing towards the hilus. The Westermark sign is decreased vascularity. D-dimer is a measure of fibrin degradation products. A normal value reliably rules out the diagnosis of pulmonary embolism, while an elevated value suggests the diagnosis of pulmonary embolism.

Pneumothorax pain is usually described as the sudden onset of pleuritic chest pain. Other signs and symptoms of pneumothorax such as dyspnea, tracheal deviation, and decreased breath sounds may also be present. Chest radiograph findings of pneumothorax include a mediastinal shift and the presence of air without lung markings in the affected side.

Esophageal reflux pain is a substernal pain usually described as burning. It may last for up to 1 hour. It is relieved by antacids and may be aggravated by alcohol and postprandial recumbency.

Aortic dissection pain is of abrupt onset and usually described as severe and tearing. It may radiate from the anterior chest wall posteriorly to the interscapular region. On exam, blood pressure may be elevated and a pericardial rub and diastolic murmur of aortic insufficiency radiating along the right sternal border may be auscultated.

Unstable angina pain is usually described as tightness or pressure on the chest and lasts for 10-20 minutes. It is usually left-sided or retrosternal and may radiate to the jaw and neck. It often occurs at rest, but it may be precipitated by physical exertion and emotional stress. On examination, a systolic murmur of mitral regurgitation may be auscultated during an episode of pain.
Correct answer:
Pneumothorax

Explanation
This patient is presenting with a spontaneous primary pneumothorax. A finding of a pleural line on chest x-ray (CXR) is diagnostic for this condition. A pneumothorax is a condition in which air is introduced into the lung cavity, either spontaneously or by trauma. In young people without a known pulmonary pathology (more common in smokers especially), a pneumothorax may occur spontaneously. In older patients, other causes, such as a lung tumor or severe pulmonary disease, can lead to the collapse of the lung. As the lung collapses, the line from the edge of the pleura may be visible on CXR.

Bronchiectasis is a chronic condition of the large bronchi associated with coughing, dyspnea, and wheezing. Cystic fibrosis is a common cause of bronchiectasis. This patient does not have a cough, wheezing, or a history of chronic pulmonary disease.

Bronchitis is a rather vague term referring to acute or chronic inflammation of the bronchi. A viral infection is a common cause of acute bronchitis. Chronic obstructive pulmonary disease (COPD) is a common cause of chronic bronchitis. This patient's history and physical do not support any bronchial inflammation; they are not suggestive of an acute infection.

A foreign body in the bronchus can cause acute shortness of breath, chest pain, and some of this patient's physical findings. Typically, an adult patient should be able to provide a history that no foreign bodies were aspirated; an x-ray would usually identify a foreign body. Furthermore, a foreign body would not produce the pleural line unless it caused a pneumothorax; therefore, an airway foreign body is unlikely in this patient.

Pneumonia can cause chest pain and shortness of breath. However, the patient would likely feel more ill; there would be a history of malaise, fatigue, fever, chills, cough, and possibly nausea. The patient's x-ray and physical exam findings are not found with pneumonia. If pneumonia causes percussive changes on the lung exam, it produces dullness to percussion, not hyperresonance.
Correct answer:
Evaluation by a pulmonologist and lung transplantation specialists should be made.

Explanation
Any patient suspected of having idiopathic pulmonary fibrosis, interstitial lung disease, or any another idiopathic interstitial pneumonia should be referred to a pulmonologist for further evaluation and management. Any patient diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis should be referred for lung transplantation evaluation, regardless of the vital capacity.

The goal of any disease management strategy should include assessment and treatment of comorbid medical conditions. Common comorbid medical conditions found in patients with idiopathic pulmonary fibrosis (IPF) include chronic obstructive pulmonary disease, obstructive sleep apnea, gastroesophageal reflux disease, and coronary artery disease. Therefore, if any of these comorbid illnesses are present, they should be managed according to current practice guidelines.

Vaccination against influenza and pneumococcal infection should be encouraged in all patients with idiopathic pulmonary fibrosis.

Any patient with idiopathic pulmonary fibrosis who is overweight should be encouraged to meet with a nutritionist and make dietary changes to achieve ideal body weight. Maintaining adequate nutritional intake is important for quality of life in patients with idiopathic pulmonary fibrosis.

Improving quality of life is an important goal in disease management. Deconditioning and subsequent functional impairment is a common problem in patients with idiopathic pulmonary fibrosis and negatively impacts quality of life. Patients with idiopathic pulmonary fibrosis should be evaluated for pulmonary rehabilitation and encouraged to participate in regular exercise to maintain a maximal degree of musculoskeletal conditioning.
Correct answer:
Metastatic infiltrating ductal carcinoma

Explanation
Metastatic infiltrating ductal carcinoma is the correct response.

A blood-tinged effusion in anyone past the age of 40 should put neoplasia into the differential diagnosis. Neoplastic pleural invasion usually involves both the visceral and the parietal surfaces and can cause a bloody pleural effusion. Breast cancers often metastasize to the lung and pleura. Carcinomas of the lung and breast are the most common primary sites from which pleural metastases arise. Stomach and ovarian carcinomas are next in frequency for the development of malignant pleural effusion. Spread of malignancy to the pleura from breast, stomach, and ovarian cancers usually occur indirectly from hepatic metastases. However, contiguous spread through the chest wall in breast cancer, and through the diaphragm in ovarian or stomach cancer, can occur occasionally. When malignancy is suspected, cytological examination of the fluid helps in establishing the diagnosis. Pleural biopsy, or pleural fluid immunocytochemistry, is used as an adjunct to cell morphology to aid diagnosis.

Cor pulmonale results in peripheral edema, including pleural effusion. These are usually serous effusions, which are transudative in nature.

Systemic lupus erythematosus (SLE) can be associated with exudative pleural effusions, but they are usually serous and bilateral.

Bacterial sepsis due to S. aureus could lead to pleuritis with purulent exudates.

Pulmonary embolism (a common cause of pulmonary infarction) results in bloody pleural effusions; however, the cytological examination will be negative.
Correct answer:
Spironolactone

Explanation
This patient has a presentation most consistent with cor pulmonale secondary to chronic obstructive pulmonary disease (COPD). The electrocardiogram demonstrates right axis deviation, right ventricular hypertrophy, and right atrial enlargement. The chest x-ray indicates cardiac enlargement, with prominence of the pulmonary artery, right atrium, and right ventricle. Treatment should center upon treatment of the underlying contributory disorder. For patients with COPD, bronchodilation and infection treatment should be considered. Oxygen therapy is of great importance in patients with underlying COPD,especially when administered on a continuous basis. Oxygen therapy relieves hypoxemic pulmonary vasoconstriction, which then improves cardiac output, lessens sympathetic vasoconstriction, alleviates tissue hypoxemia, and improves renal perfusion.

Prostaglandin I2 (prostacyclin) is the main product of arachidonic acid in the vascular endothelium, has relatively specific vasodilatory effects on the pulmonary microvasculature along with antiplatelet effects, and, over time, may also promote vascular remodeling. Prostacyclin has been the cornerstone in the medical treatment of IPAH but is ineffective in the larger group of patients with severe COPD or interstitial pulmonary fibrosis or when hypoxemia is the cause for PAH (pulmonary artery hypertension).

Current guidelines suggest low-level graded aerobic exercise, being careful to avoid heavy physical exertion or isometric exercises because they may cause syncope. PAH and cor pulmonale patients should follow a sodium-restricted diet of less than 2400 mg per day.

Immunosuppressive therapy is often administered to patients with connective tissue diseases and those with interstitial lung disorders, although only a minority of patients in the latter category respond. Although there are reports of improvements in pulmonary function and oxygenation in patients who are receiving interferon-γ1b injections, recently published trials failed to show any improved survival with this therapy.

To manage right ventricular volume overload, patients often respond well to a combination of a loop diuretic, such as furosemide, and the potassium-sparing diuretic spironolactone
Correct answer:
Anticoagulation

Explanation
This patient is presenting with a likely pulmonary embolism (PE). Acute dyspnea, cough (with or without hemoptysis), chest pain, and tachycardia/tachypnea are common presenting signs/symptoms of a PE. If further testing was to be done, a CT pulmonary angiography could be done. However, with a strong clinical suspicion, the diagnosis can be made and anticoagulation, the cornerstone of PE treatment, can be initiated. There are options in prediction rules for PE. This patient would score "high" on the Wells criteria, with clinical signs/symptoms of deep venous thrombosis -3.0 points; alternative diagnosis less likely than PE - 3.0 points; heart rate >100 beats/minute -1.5 points; and hemoptysis - 1.0 points. Unfractionated heparin and low molecular weight heparin are the most common choices. The patient's tender leg should also be evaluated for a possible deep venous thrombosis, although it would be treated with the same anticoagulation as the PE. Depending on the patient's hemodynamic stability and extent of PE, fibrinolytic therapy (thrombolysis) may be considered.

Chest tube decompression may be used to treat a pneumothorax. A pneumothorax may present with chest pain and dyspnea, but the D-dimer would not be elevated. The lung exam would be expected to be abnormal, and a pleural line may have been shown on the chest x-ray.

Hyperbaric oxygen therapy is useful for a variety of conditions, ranging from non-healing skin wounds to carbon monoxide poisoning and air embolism. It is not a primary treatment for PE.

Inferior vena cava filters can be used in the management of PE, mostly in individuals with recurrent PE, to prevent future embolism. This patient did not have a prior history of PE. The anticoagulants are first-line treatment.

Percutaneous coronary intervention (PCI) is a treatment for acute ST-elevation myocardial infarction (STEMI), especially if it can be performed very rapidly after symptom onset. It does not have a role in the treatment of PE. Myocardial infarction (MI) would have initially been on the differential for a patient with chest pain and dyspnea, but the tests have helped rule MI out.
Correct answer:
Influenzae and pneumococci

Explanation
The correct response is influenzae and pneumococci.

It is very likely that the patient described above has an occupation pulmonary disease process, most likely coal worker's pneumoconiosis, or black lung disease. Most patients that develop this disease tend to have worked around coal dust for several years, if not decades. For this reason it is commonly diagnosed in patients who are over the age of 50. Smoking does not increase the risk of developing pneumoconiosis. The chronic ingestion of inhaled coal dust by macrophages in the alveoli leads to the formation of coal macules, usually 2 - 5 mm in diameter. These areas then appear on a chest radiograph as opacities, especially in the upper lung.

Simple coal work's pneumoconiosis commonly is asymptomatic; at times a cough or even varying degrees of shortness of breath may be present. The patient will typically not have any significant abnormal pulmonary function results. More severe cases of this pathology are usually referred to as progressive massive fibrosis.

There is no cure for coal worker's pneumoconiosis; therefore, prevention of excessive and long-term exposure is necessary. Once a patient has developed symptoms of this disease, such as the patient above, the healthcare provider must ensure the patient is up to date with all potential vaccinations to avoid any pulmonary co-morbidities. This should be viewed as a critical component of health maintenance. The influenzae and the Streptococcivaccines would be crucial for this patient to receive; consequences of these infections could be extremely dangerous. A zoster vaccination may be considered, but would not necessary be the best answer. This patient receiving a varicella vaccine would not be recommended at this time.
Correct answer:
Bronchial carcinoid tumor

Explanation
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:
Control of his heart failure to prevent pulmonary complications

Explanation
Preventive medicine is often classified into primary, secondary, and tertiary methods. Primary methods are meant to prevent disease occurrence altogether. Secondary methods are intended to detect disease at a very early stage, before complications. Tertiary methods may occur after the patient already has some significant illness, and they are aimed at preventing further complications from the disease. In this patient's case, control of his heart failure to prevent pulmonary complications is a recommended tertiary preventive medicine. This patient's chronic heart failure has caused a pleural effusion. Better control of his heart condition will prevent pulmonary complications.

Administration of the influenza vaccine is a recommended primary method of preventive medicine for this patient because it prevents the illness altogether. It is recommended for almost all adults, and it is especially recommended for the patient in this case, but it is not classified as tertiary prevention.

Checking a prostate-specific antigen (PSA) to detect prostate cancer at an early stage is considered a secondary level of prevention; it is aimed at detecting the illness at a very early and treatable stage. PSA screening for prostate cancer is controversial, and because this patient is much more likely to succumb to complications from his heart failure and pleural effusion than prostate cancer, it should not be recommended at this time.

Daily low-dose aspirin has been often recommended as primary prevention of stroke and myocardial infarction in high-risk individuals as well as secondary prevention. While aspirin therapy may offer some mild benefit to this patient (who has a history and physical consistent with chronic congestive heart failure), it is a not a top priority.

The provider should not recommend calcium supplementation to reduce risk of fractures in this patient. Calcium supplementation has recently been linked to an increased risk of cardiovascular events. Except for specific conditions, widespread calcium supplementation is not recommended. It would also be a primary prevention technique, intended to prevent bone loss and fracture before disease was present.
Correct answer:
Pleural fluid N-terminal pro-brain natriuretic peptide levels greater than 1500 pg/mL

Explanation
This patient's presentation is most consistent with a pleural effusion. This patient is demonstrating pleuritis, which is caused by damage and inflammation to the pleura of the lung. The most common cause of pleural effusion is left ventricular failure, which causes a transudative effusion.

The characteristics of pleural fluid transudate are a yellow appearance, specific gravity of less than 1.016, absolute protein of less than 3 grams per 100 mL, a pleural protein to serum ratio of less than 0.5, a pleural to serum LDH ratio of less than 0.6, a serum to pleural glucose ratio of less than 1, and very low WBCs. A pleural fluid N-terminal pro-brain natriuretic peptide (NT-proBNP) >1500 pg/mL is virtually diagnostic of an effusion secondary to congestive heart failure.

The earliest sign of pleural effusion on the frontal view of a chest radiograph is a blunting of the lateral costophrenic angle. A large free pleural effusion appears as a dependent opacity with lateral upward sloping of a meniscus-shaped contour. The diaphragmatic contour is partially or completely obliterated, depending on the amount of fluid (silhouette sign).

Lobar consolidations with air bronchograms, patchy airspace opacities, or diffuse alveolar or interstitial opacities are common radiographic findings consistent with pneumonia.

Hyperinflation, hyperlucency, and depressed diaphragms upon chest X-ray assessment suggest asthma. Increased lung volumes and increased retrosternal airspace may also be observed.
Correct answer:
Talc sclerotherapy

Explanation
The correct response is Talc sclerotherapy.

The clinical scenario is highly suggestive of a (recurrent) secondary pneumothorax. Components that lead to this diagnosis include unilateral, sudden chest pain with dyspnea and minimal physical exam findings, in this case, the mild tachycardia and diminished breath sounds. The above scenario most likely fits what is considered a secondary pneumothorax, which usually occurs as a complication of various pathologies, including COPD, asthma, cystic fibrosis, tuberculosis, Pneumocystis pneumonia, and various interstitial lung diseases. Diagnosis is usually confirmed with chest radiograph.

Treatment is typically observation and potential surgical intervention after about 5 days if the pneumothorax fails to resolve itself. However, the ability of the patient to withstand a surgical intervention also must be considered. There are various thought processes in terms of management of recurrent pneumothorax especially. The correct option out of the above is the talc sclerotherapy; this is commonly reserved for patients who may not necessarily be surgical candidates. This is also known as pleurodesis and is performed to obliterate the pleural spaced to prevent recurrent pleural effusions or persistent pneumothorax. Talc is an effective sclerosant is instilled into the pieural space to cause inflammation and fibrosis. Intravenous antibiotics would be correct if the pneumothorax were actually caused by the sequelae of staphylococcal pneumonia. Oral antibiotics, short-acting β2-agonist, and inhaled corticosteroids are inappropriate in the management of a secondary pneumothorax
Correct answer:
Pleural effusion

Explanation
The correct response is pleural effusion.

The key findings are absent breath sounds, specifically in the lower lung field during auscultation, percussion that produces a dull note in the same area, and tactile fremitus absent over that location. All these signs indicate that fluid accumulation is highly likely in the pleural space in that area (which separates the air-filled lung from the chest wall) and blocks the transmission of sound. Another sign is a notable fever (in an elderly patient) indicating some type of infectious process. Symptoms further lead to respiratory or cardiology involvement.

During auscultation, breath sounds are many times obscured by wheezing; percussion will reveal resonant and hyper-resonant notes. Tactile fremitus will be absent. Fever is not a feature of asthma.

Chronic obstructive pulmonary disease (COPD) is not the correct choice. Although during auscultation breath sounds would be potentially decreased to absent, these will be diffusely noted (not just concentrated in 1 area). There also may be crackles, wheezes, or rhonchi heard with this particular diagnosis. When percussion is performed in a patient with COPD, there will be diffusely hyper-resonant percussion notes produced. Tactile fremitus would be decreased, but this would be throughout the lung fields. Fever could potentially be an associated finding in COPD.

Early left-sided heart failure is not the correct choice. In this pathology, there is increased pressure in the pulmonary veins which causes congestion and interstitial edema around the alveoli; bronchial mucosa may also become edematous. For this reason, auscultation will potentially reveal vesicular breath sounds; there may also be late inspiratory crackles in dependent portions of the lung fields (and some wheezing). Tactile fremitus will be normal; percussion will reveal resonant notes. Fever is not a sign of this diagnosis.

Pneumothorax occurs when there is air leaking into the pleural space, typically unilaterally, causing the lung to recoil away from the chest wall. Pleural air blocks the transmission of sound (as in this patient), but will produce different physical examination findings; during percussion, the affected area with produce a hyper-resonant (or even tympanic) note over the affected area, auscultation will be absent, but tactile fremitus may be decreased or potentially absent. Fever is not a common finding.
Correct answer:
Haemophilus influenzae, Streptococcus pneumoniae, Moraxella catarrhalis

Explanation
Haemophilus influenzae, Streptococcus pneumoniae, and Moraxella catarrhalis are the most predominant pathogens involved in the acute exacerbation of chronic bronchitis; they are responsible for up to 60% of the cases and are the most common pathogens to be cultured from sputum in these patients. Acute exacerbations of chronic bronchitis are marked by an increase in the volume or purulence of sputum and a worsening of cough or dyspnea. Gram stain of the sputum usually shows a mixture of organisms; they are often gram-positive diplococci, Streptococcus pneumoniae, and gram-negative rods, Haemophilus influenzae.

Other organisms associated with acute exacerbations include M. pneumoniae, Legionella pneumophila, atypical bacterium (e.g., Chlamydia pneumoniae), and viruses (e.g., influenza and adenovirus). C. pneumoniae is the most common atypical bacterium implicated in exacerbations and approximates 10% of the cases. Viruses have been implicated in the remaining 25-30% of the cases. Pseudomonas aeruginosa is associated with severe exacerbations that require mechanical ventilation. The less frequent cause of exacerbation includes M. pneumoniae and L. pneumophila.

Sputum production in chronic bronchitis is insidious in onset, initially occurring only in the morning. The sputum is usually mucoid, but it becomes purulent during an exacerbation. The patient may also be wheezing, dyspneic, and febrile during these exacerbations. These acute exacerbations can be triggered by exposure to tobacco smoke, air pollution, viral infections, and allergies. As chronic obstructive pulmonary disease (COPD) progresses, the intervals shorten. Patients with chronic bronchitis may also present with hemoptysis, which is usually caused by mucosal erosion.

On examination, there may be expiratory wheezes and crepitations. CXR reveals irregular bronchovascular markings due to repeated inflammation and scarring. Patients with acute exacerbations of chronic bronchitis should be treated with trimethoprim/sulfamethoxazole or amoxicillin/clavulanate combinations. They should also receive an annual influenza vaccination.
Correct answer:
Reduced inspiratory patency of the upper airway due to relaxation of the airway muscles

Explanation
This patient's history and physical exam are remarkable for obstructive sleep apnea/hypopnea syndrome (OSAHS), which is caused by the airway being sucked closed on inspiration during sleep. This occurs as the upper-airway dilating muscles relax during sleep. In patients with OSAHS, the dilating muscles fail to oppose negative pressure within the airway during inspiration. Although these patients have narrow upper airways already during wakefulness, increased muscle dilating activity helps to maintain airway patency. However, during sleep, muscle tone falls and the airway further narrows; snoring may commence before the airway occludes, and apnea results. Apneas and hypopneas terminate when the subject arouses (i.e., briefly wakes up).

When it occurs in the setting of breast cancer, lung cancer, or lymphoma, the presence of dyspnea; facial, neck, trunk, or extremity swelling; headache; and venous distension, this suggests the possibility of superior cava syndrome.

In Hashimoto's thyroiditis, there is a marked lymphocytic infiltration of the thyroid with germinal center formation, atrophy of the thyroid follicles accompanied by oxyphil metaplasia, absence of colloid, and mild to moderate fibrosis.

The predominant pathologic event of chronic bronchitis is an inflammatory process in the airways, with mucosal thickening and mucus hypersecretion, resulting in diffuse airflow obstruction.
Correct answer:
Hospice care with the diagnosis of end stage HIV/AIDS disease

Explanation
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/ml from 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:
Malignancy

Explanation
The correct response is malignancy.

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. The differential diagnosis of bilateral pleural effusions includes any cause of a transudative effusion. The most common of these is cardiac, renal or liver failure, and hypothyroidism. It also includes certain causes of exudative effusions, such as malignant disease, pneumonia, and tuberculosis. 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.

As defined by Light's Criteria, a pleural effusion is an exudate that has 1 or more of the following laboratory features:

Ratio of pleural fluid protein to serum protein >0.5;
Ratio of pleural fluid LDH to serum LDH >0.6; and
Pleural fluid LDH >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 this patient 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:
Erythema nodosum

Explanation
This patient presents with classical findings of sarcoidosis. Erythema nodosum (EN) may occur in up to 39% of sarcoidosis cases, characterized by tender erythematous nodules most commonly located on the anterior tibial areas. EN is usually related to the acute presentation of systemic sarcoidosis (fever, malaise, polyarthralgias, and bilateral hilar lymphadenopathy) and has a self-limited course of about 1 month.

EN is a non-specific skin lesion that is also observed in rheumatic fever, inflammatory bowel disease, infections (coccidioidomycosis, histoplasmosis, α-hemolytic streptococci, tuberculosis, and syphilis), patients with hypersensitivity reactions to drugs (oral contraceptives, NSAIDs), and pregnancy. Despite all these associations, EN is typically idiopathic in approximately 55% of the cases. An EN biopsy should be avoided, as histopathology shows non-specific subcutaneous inflammation and vasculitis.

While lupus pernio is a skin manifestation of sarcoidosis, it is unusual on the legs; it is a specific cutaneous manifestation of chronic systemic sarcoidosis. Lupus pernio is characterized by red-to-purple indurated papules, plaques, or nodules; they are most commonly seen on the mid-face, particularly the alar rim of the nose and the hands. Biopsy shows granulomas and is diagnostic of sarcoidosis.

Erythematous patches and plaques can be seen in multiple situations, including systemic lupus erythematosus (SLE) and dermatomyositis (DM). The classical SLE rash has a butterfly shape and is localized on the cheeks and the nose ridge. In DM, the rash is localized on the face, neck, back, and shoulders (shawl sign).

Like erythema nodosum, pretibial myxedema is usually located on the anterior tibial areas. Pretibial myxedema is characterized by areas of a waxy, discolored induration resultant of glycosaminoglycans accumulation in Grave's disease.

Erythema multiforme presents with multiple types of lesions, including macules, papules, and vesicles; however, the classic description is the "target lesion," which is characterized by multiple erythematous rings around a dusky blue center (commonly located on the trunk or extremities). It is usually seen in drug reactions (penicillin, sulfa drugs, phenytoin, and aspirin), but it can also be associated with infections (Mycoplasma pneumoniae, HSV), cancers (lymphoma, leukemia), and autoimmune conditions (collagen diseases, vasculitis). It is part of a spectrum that includes Steven-Johnson syndrome and toxic epidermal necrolysis.

Sarcoidosis most commonly affects people of African and Northern European ancestry.
Correct answer:
Pulmonary embolism

Explanation
This patient has a presentation most consistent with pulmonary embolism. Nearly all PEs arise from deep venous thrombosis (DVT) in the lower extremity or pelvic veins. Risk factors for DVT and PE are similar in children and adults and include conditions that impair venous return, conditions that cause endothelial injury or dysfunction, and underlying hypercoagulability disorders. Bed rest and confinement without walking, even for a few hours, are common precipitators. Specific risks include an age over 60, atrial fibrillation, cigarette smoking (including passive smoke), estrogen receptor modulators, exogenous estrogens, and progestins (including oral contraceptives and estrogen therapy), extremity or pelvic trauma, heart failure, hypercoagulability disorders, immobilization, indwelling venous catheters, myeloproliferative disorders, nephrotic syndrome, obesity, pregnancy and postpartum states, sickle cell anemia, recent surgeries, and prior venous thromboembolism. Larger emboli cause acute dyspnea, pleuritic chest pain, or both. Dyspnea may be intermittent or occur only with exercise. Less common symptoms include cough and hemoptysis.

The most common signs of PE are tachycardia and tachypnea. Less commonly, patients have hypotension, a loud second heart sound (S2) due to a loud pulmonic component (P2), and crackles or wheezing. In the presence of right ventricular failure, distended internal jugular veins and a right ventricular heave may be evident, and right ventricular gallop (third and fourth heart sounds [S3 and S4]), with or without tricuspid regurgitation, may be audible. ECG most often shows tachycardia and various ST-T wave abnormalities, which are not specific for PE. An S1Q3T3 or a new right bundle branch block may indicate the effect of abrupt rise in right ventricular pressure on right ventricular conduction; these findings are moderately specific but insensitive for PE.

Pulmonary edema typically causes severe dyspnea, the production of pink and frothy sputum, and diaphoresis and cyanosis. Rales are present in all lung fields, as are generalized wheezing and rhonchi. The chest X-ray usually indicates vascular redistribution, blurriness of vascular outlines, increased interstitial markings, and, characteristically, the butterfly pattern of distribution of alveolar edema.

The patient's younger age, quality of chest pain, lack of gastrointestinal and skin findings, and absence of characteristic EKG findings make acute myocardial infarction a less likely diagnosis. The rapid onset of symptoms and denial of fever, cough, and sputum suggest a diagnosis other than bacterial pneumonia, as does the absence of an allergic history and adventitious lung sounds.
Correct answer:
Emphysema

Explanation
The findings described suggest that the patient is suffering from emphysema. Emphysema is a chronic obstructive airway disease; it is characterized by abnormal, permanent enlargement of the airways distal to the terminal bronchioles and destruction of their walls. Cigarette smoking is a major contributor in the development of emphysema. Other causes include air pollution, alpha-1 antitrypsin deficiency, and occupational exposure. The classical symptomatology includes prolonged progressive dyspnea with late-onset non-productive cough, occasional mucopurulent relapses, and eventual cachexia and respiratory failure. The patients are usually thin and have a barrel-shaped chest. There is tachypnea with pursed-lip breathing and use of accessory muscles; they may also adopt a tripod sitting position. A chest X-ray shows hyperinflated lungs with bullae, tubular heart, and flattened diaphragm. Forced expiratory spirometry quantifies airway obstruction. Pulmonary function tests (PFT) reveal a decrease in forced expiratory volume in 1 second (FEV1) along with reduction of FEV1/FVC (forced vital capacity) ratio. Treatment includes general measures, such as cessation of smoking, breathing exercises, symptomatic treatment like antibiotics to control secondary infection, bronchodilators, corticosteroids, and oxygen therapy. Lung volume reduction surgery and lung transplantation may be required depending on the severity.

Congestive cardiac failure presents with dyspnea, cough, orthopnea, weakness, abdominal discomfort, and edema in lower portions of the body. The chest X-ray may show cardiac enlargement. There may be pulmonary congestion with audible rales. Echocardiography confirms the diagnosis.

Chronic bronchitis produces obstructive changes and bronchial inflammation. Chronic bronchitis is characterized by chronic productive cough for ≥3 months in each of 2 successive years for which other causes, such as infection with Mycobacterium tuberculosis, carcinoma of the lung, or chronic heart failure, have been excluded. Chest X-ray shows increased bronchovascular markings and cardiomegaly.

Bronchiectasis is a condition characterized by abnormal permanent distortion of the conducting bronchi or airways, most often secondary to an infectious process. Patients with bronchiectasis often present with chronic cough with mucopurulent sputum production that lasts months to years. CT, especially HRCT (high-resolution computed tomography), helps in confirming the diagnosis.

Diffuse alveolar damage, or adult respiratory distress syndrome, is characterized by fulminant interstitial and alveolar edema that develops after an initial lung injury. It results from increased alveolar-capillary permeability that is not cardiogenic in origin. Injury can occur directly or as part of a generalized systemic acute inflammatory process.
Correct answer:
Pneumococcal polysaccharide vaccination

Explanation
Normally recommended for all individuals 65 and over, the pneumococcal polysaccharide vaccination is recommended for younger patients who have chronic lung diseases, such as this patient.

Blood pressure screening of adults is recommended by multiple organizations. A recommended interval is every 2 years for those with normal blood pressures and every year for those with mildly elevated blood pressures (systolic 120-139 mm Hg and/or diastolic 80-89 mm Hg). This patient has a systolic blood pressure of 140 mm Hg and other cardiovascular complications, however, so he should be monitored more frequently. If high blood pressure remains elevated, he should be treated.

Colonoscopy is a colon cancer screening technique routinely recommended for all men and women 50 and over; it may be recommended earlier if a family history of colon cancer is present. If he has not yet had one, this patient should undergo a screening colonoscopy, but this does not directly relate to his presenting condition (emphysema/chronic obstructive pulmonary disease, progressed into cor pulmonale). Preventive services are most effective when tailored to the patient's specific needs. In the patient's case, a pneumococcal vaccination is a higher priority preventive service than colonoscopy.

Prostate-specific antigen (PSA) testing for prostate cancer has been controversial. Currently, the US Preventive Services Task Force is neither for nor against PSA screening. Given his severe pulmonary disease, PSA testing is not a high priority for this patient.

This patient's current BMI is over 30, indicating obesity, but the patient's history reveals very recent weight gain attributable to fluid retention. Before any aggressive weight loss is recommended, the edema should be addressed through sodium and fluid restriction and most likely diuretic medications. Furthermore, aggressive physical activity for weight loss would likely exacerbate his condition, so it is not recommended.
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.
Case Ico-delete Highlights
A 47-year-old Caucasian woman, previously healthy, presents for evaluation of a 4-week history of dyspnea. Her symptoms started approximately 1 month ago when on vacation in Colorado. She initially attributed these symptoms to the altitude; however, upon return to her hometown, she continued to have shortness of breath with mild activity, such as walking more than 100 feet, walking upstairs 1 flight, vacuuming, or sweeping. Her symptoms resolved with rest. She also reports mild exertional chest tightness and easy fatigability. She denies paroxysmal nocturnal dyspnea, orthopnea, edema, palpitations, and syncope.
Past medical history includes the usual childhood illnesses. She has no previous surgeries and no known allergies. Medications include a daily multivitamin and occasional Tylenol for headaches. She is a non-smoker, rarely uses alcoholic beverages, and denies the use of illegal or illicit drugs.
Physical exam reveals an alert white woman in no acute distress. Her vitals reveal temp 96.9°F, pulse 80 and regular, respirations 16, and BP 136/82. O2 sat is 96% on room air. There is no obvious jugular venous distention. Respirations are non-labored. Lung fields are clear to auscultation and percussion. No rhonchi, rales, or wheezes are present.

Heart shows RV heave present, normal S1 with fixed, split S2 with prominent P2 component. Grade II/VI systolic murmur is present at the left upper sternal border at the second intercostal space. The remainder of a complete physical examination is within normal limits.
CBC and BMP are unremarkable. Free T4 and TSH are within normal limits. EKG shows normal sinus rhythm with right ventricular hypertrophy, right atrial enlargement, and right axis deviation. There is an RSR in leads v1 and v2.

Question
What do you suspect is the primary cause of her dyspnea?
Correct answer:
Pulmonary hypertension

Explanation
The correct answer is pulmonary hypertension. The reduced oxygen concentration present at high altitudes can trigger (or worsen) the development of pulmonary hypertension in patients who were previously asymptomatic. Symptoms of pulmonary hypertension include dyspnea, associated chest tightness, and easy fatigability. Palpitations or dizziness may be associated symptoms. With more severe pulmonary hypertension, symptoms of right-sided heart failure (pedal or peripheral edema, ascites, hepatomegaly, or jugular venous distention) may develop.

Angina secondary to coronary artery disease is incorrect. Angina most commonly presents as exertional chest tightness alleviated by rest, but it often presents as exertional dyspnea. This patient has both of these symptoms; however, angina alone would not account for the abnormalities on her physical exam (cardiac murmur, widely fixed and split S2) and EKG (right ventricular hypertrophy, right atrial enlargement, right axis deviation, and incomplete right bundle branch block). This patient also does not have typical risk factors for CAD, such as hypertension, diabetes, high cholesterol, or smoking.

Aortic stenosis is incorrect. Aortic stenosis can cause exertional dyspnea and typical exertional angina; however, aortic stenosis would not cause right ventricular hypertrophy. It could, however, cause left ventricular hypertrophy.

Atrial septal defect is incorrect. Atrial septal defect is a congenital heart defect present at birth. If an atrial septal defect were the principal cause of her dyspnea, the patient would have had dyspnea her entire life. This patient did not develop dyspnea until four weeks ago, due to the development of pulmonary hypertension after high-altitude exposure. Atrial septal defect is associated with a widely-fixed split S2 and cardiac murmur and could cause right ventricular hypertrophy and right atrial enlargement. Atrial septal defect can cause long-standing right ventricular volume and pressure overload, which can certainly be contributing factors to the development of pulmonary hypertension. cause right ventricular hypertrophy and right atrial enlargement.

Idiopathic pulmonary fibrosis is incorrect. Idiopathic pulmonary fibrosis could certainly cause dyspnea and fatigue; however, it typically would be associated with bibasilar inspiratory lung crackles on physical examination. This patient had normal auscultation of the lungs. Idiopathic pulmonary fibrosis rarely affects patients younger than 50.
Correct answer:
He was given BCG vaccination earlier in life.

Explanation
The patient was given BCG vaccination earlier in life.

The Mantoux tuberculin skin test (TST) is performed to determine whether the person is infected with Mycobacterium tuberculosis. The TST is performed by injecting 0.1 mL of tuberculin purified protein derivative (PPD) into the inner surface of the forearm. Skin test results should be read 48-72 hours after administration of the PPD.

Generally, in immunocompetent individuals, any palpable induration ≥10 mm is considered a positive reaction. In the case of tuberculosis suspects or close contacts of individuals with tuberculosis, an induration ≥5 mm should be interpreted as a positive reaction.

An induration measuring 5-9 mm is considered a doubtful reaction. In case of doubtful reaction, the possibility of skin sensitivity due to previous immunization or atypical mycobacterium exposure should be considered. Though prior BCG vaccination increases the risk of a reactive PPD, this effect is known to be inconsistent. Studies have shown that reactions >10 mm should not be attributed to prior BCG vaccination. Induration ≥5 mm should be considered positive in HIV-positive patients, patients with known contact with active TB, patients with a history of organ transplant on immunosuppressive therapy, and patients with fibrotic lesions on chest X-ray.

Positive tuberculin test indicates exposure of the immune system to tuberculous protein, either in the form of BCG vaccine, an active tuberculous infection, or a chronic tuberculous infection.

Proper vaccination history could have unmasked the cause of the indeterminant reaction to tuberculin test in this patient. Other causes, such as a wrong sputum and chest X-ray report, inadequate sputum samples, chronic cavitary lesion, or active tubercular infection, are not the cause of doubtful reaction in this patient.
Correct answer:
Start isoniazid+rifampicin+pyrazinamide+ethambutol immediately and reassess the patient's treatment after the culture result.

Explanation
The choice for tuberculosis treatment is always multiple drug therapy to prevent resistance during the long treatment (6-9 months). Isoniazid (INH), rifampicin, pyrazinamide, and ethambutol is the most used combination to start in newly diagnosed TB patients. The patient described in this question has all the symptoms of pulmonary TB. His PPD skin test is 15 mm, and his sputum is also positive for acid-fast bacilli.

PPD is used as an antigen in the tuberculin test, which is a delayed hypersensitivity reaction. The standard tuberculin test is performed by injecting 0.1 mL of diluents containing 5 TU (tuberculin unit) of PPD. It is injected intradermally, and induration is checked in 48-72 hours. 0-5 mm is accepted as negative; 5-10 mm can be due to a previous BCG vaccination. If a patient has not had the vaccine and was exposed to TB, however, 5-10 mm indicates they are probably infected. In AIDS patients, a 5 mm reaction is considered positive. Even if they had the BCG vaccine previously, patients with 15 mm or more induration are assumed to be infected with M. tuberculosis.

This patient's PPD is 15 mm, and his sputum is positive for AFBs, so the proper approach should be to send a culture to identify the type of Mycobacterium, but treatment should not be delayed waiting for culture results. Multiple drug therapy should be started and the patient's treatment should be assessed after receiving the culture result. If it is not a resistant strain, pyrazinamide and ethambutol can be stopped after 2 months. The patient should continue with INH and rifampin and complete 6 months of treatment.
Correct answer:
Oxygen

Explanation
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 undergone appropriate trials to indicate a benefit for treating idiopathic pulmonary fibrosis. Current guidelines recommend against using corticosteroid therapy alone.

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:
Pneumonia

Explanation
Pneumonia often causes a sharp pain that is aggravated by inspiration. Other signs and symptoms of pneumonia (e.g., cough, fever, dyspnea, and rales on auscultation) may also be present. A consolidation on chest X-ray is also consistent with the diagnosis of pneumonia.

Esophageal reflux pain is a substernal pain that is usually described as burning. It may last for up to 1 hour. It is relieved by antacids and may be aggravated by alcohol and postprandial recumbency. There are typically no X-ray findings associated with reflux.

Aortic dissection pain is of abrupt onset; it is usually described as severe and tearing. It may radiate from the anterior chest wall posteriorly to the interscapular region. On examination, the blood pressure may be elevated. A pericardial rub and the diastolic murmur of aortic insufficiency radiating along the right sternal border may be auscultated. The mediastinum may be widened on chest X-ray.

Pulmonary embolism pain is usually of abrupt onset and pleuritic in nature. It has a duration of several minutes to a few hours. There may be predisposing factors, such as deep venous thrombosis or long periods of immobilization. Patients may also report hemoptysis. On examination, the patients are dyspneic and tachypneic with tachycardia.

Unstable angina pain is usually described as tightness or pressure on the chest; it lasts for 10-20 minutes. It is usually left-sided or retrosternal, and it may radiate to the jaw and neck. It often occurs at rest, but it may be precipitated by physical exertion and emotional stress. On examination, a systolic murmur of mitral regurgitation may be auscultated during the pain.
Correct answer:
Endotracheal intubation

Explanation
The clinical picture is suggestive of epiglottitis due to H. influenzae type B infection. Hib immunization is not routine in many parts of the world. The illness is characterized by a very sore throat, dysphagia, toxaemia, and rapidly progressive airway obstruction. Total airway obstruction is imminent by the time stridor appears. The child's appearance is most deceptive. The child does not appear to be in grave danger because the airway obstruction is masked by measures the child adopts to protect the airway; the child sits, the head is kept still and jaw pushed slightly forward, there is reluctance to open the mouth wide, and breathing is shallow with slow deliberate inspirations. The child will also be reluctant to speak (speech is soft), reluctant to swallow, and may drool.

This is a medical emergency and no effort should be made to see the epiglottis, as it may precipitate complete obstruction and the patient may choke to death. There is no role for conservative therapy unless proper airway is maintained. The examination should only be done during operation under general anesthesia. After confirmation of epiglottitis, an endotracheal tube is passed, which is kept in situ for 24 to 72 hours until the fever and sore throat subside under proper antibiotic therapy, steroids, and oxygen. Once the airway is maintained, a blood culture and blood count can be taken, and intravenous chloramphenicol can be administered. Ampicillin may be substituted if the blood culture is sterile or grows sensitive organisms. Oral steroid (prednisolone) is given until the tube is out. If the child becomes gravely ill, or if he undergoes respiratory arrest, tracheostomy may be required.
Correct answer:
Plasmapheresis

Explanation
Lambert-Eaton syndrome is caused by antibodies to the presynaptic calcium channel, which decreases the release of acetylcholine. Repeated stimulation of the nerve ending increases the intracellular calcium concentration, which allows enough acetylcholine to be released to cause muscle contraction. Of the treatments listed, plasmapheresis would be most effective at removing the circulating antibodies. Other therapies include immunosuppressants (e.g., prednisone or azathioprine).

The Lambert-Eaton (or myasthenic syndrome) may occur as a paraneoplastic syndrome of small cell lung cancer. The presentation is similar to myasthenia gravis; however, in myasthenia gravis, the weakness is made worse by sustained movement while it is improved in the Lambert-Eaton syndrome. Myasthenia gravis is caused by antibodies to the acetylcholine receptor; consequently, the treatment of myasthenia gravis lies in increasing the acetylcholine concentration in the synaptic cleft using an acetylcholinesterase inhibitor (e.g., neostigmine). Acetylcholinesterase inhibitors are sometimes used to treat Lambert-Eaton syndrome, but they are rarely effective alone.

Atropine is a cholinergic antagonist at muscarinic, not nicotinic receptors; therefore it would not be useful in this case.

Amikacin is an aminoglycoside; it can cause weakness of skeletal muscles.

Cisapride is a promotility agent use for dysphagia or gastroesophageal reflux. It has been withdrawn from the United States market, and it is available only to patients who meet specific criteria.
Correct answer:
Idiopathic pulmonary fibrosis

Explanation
This patient's most likely diagnosis is idiopathic pulmonary fibrosis. Amiodarone, bleomycin, and nitrofurantoin are notable medications associated with pulmonary fibrosis.

Most patients with idiopathic pulmonary fibrosis present with a gradual onset, which is often greater than 6 months. 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. Associated constitutional symptoms are uncommon. The physical examination reveals fine bibasilar inspiratory crackles (Velcro crackles) and digital clubbing in 25 - 50% of cases. Typical chest X-ray findings include peripheral reticular opacities (netlike linear and curvilinear densities) that are predominantly located at the lung bases, honeycombing (coarse reticular pattern), and lower lobe volume loss.

The diagnosis of pulmonary embolism almost always occurs with underlying predisposing conditions present; venous thrombosis may result from a generalized hypercoagulable state, venous endothelial injury, or local stasis (Virchow triad). Most commonly, the initial manifestations of pulmonary embolism include an abrupt dyspnea and chest pain. Tachycardia and hypoxia are the most common clinical signs. Associated manifestations include fever, hypotension, cyanosis, pleural friction rub, and findings consistent with pulmonary consolidation. ECG most often shows tachycardia and various ST-T wave abnormalities, which are not specific for PE. Common chest radiographic abnormalities include atelectasis, pleural effusion, parenchymal opacities, and elevation of a hemidiaphragm. The classic radiographic findings of pulmonary infarction include a wedge-shaped, pleura-based triangular opacity with an apex pointing toward the hilus (Hampton hump) or decreased vascularity (Westermark sign).

Sarcoidosis is a multisystem inflammatory disease of unknown etiology that manifests as non-caseating granulomas, predominantly in the lungs and intrathoracic lymph nodes. The presentation commonly includes systemic complaints of fever, anorexia, and arthralgias, as well as pulmonary complaints such as dyspnea on exertion, cough, chest pain, and (rarely) hemoptysis. Extrapulmonary findings are common, and include erythema nodosum, lower-extremity panniculitis, lupus pernio, a facial violaceous rash, maculopapular plaques, granulomatous uveitis, conjunctival lesions, scleral plaques, cardiomyopathy, and cranial nerve palsies. The chest X-ray commonly reveals air trapping, lymphadenopathy, and infiltrates.

Goodpasture's disease is a condition of glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating anti-glomerular basement membrane (anti-GBM) antibodies. Constitutional symptoms, such as malaise, chills and fever, and/or arthralgias, may precede or be concurrent with pulmonary or renal manifestations. Hemoptysis, cough, dyspnea, and shortness of breath describe pulmonary involvement, while hematuria, edema, high blood pressure, and uremia signify renal affliction. Significant anemia and chest pain may also occur.

Wegener granulomatosis is a rare multisystem autoimmune disease of unknown etiology; its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels. A wide spectrum of extrapulmonary manifestations, which include recurrent respiratory infection in adults and upper and lower respiratory tract problems in children, is expected. Manifestations include constitutional complaints, conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, nasolacrimal duct occlusion, proptosis, chronic sinusitis, epistaxis, saddle nose deformity, serous otitis media, hearing loss, strawberry gingival hyperplasia, stridor, myalgias, arthritis, arthralgias, glomerulonephritis, and renal failure. Later-onset findings reveal neuropathic and cranial nerve abnormalities. Other manifestations include CNS small- to medium-sized vessel vasculitis, a palpable purpura, splanchnic vasculitis, myocardial infarction, and/or cardiac friction rubs.



References
Correct answer:
Daily low-dose inhaled budesonide

Explanation
This patient is exhibiting persistent asthma symptoms. Using the stepwise approach to treatment, the 2nd step (after using a short-acting beta-agonist, such as albuterol) is to add a low-dose inhaled corticosteroid. Alternatives include cromolyn, a leukotriene receptor antagonist, nedocromil, and theophylline. For this patient, daily low-dose inhaled budesonide would be most appropriate. He should then be monitored for response; if necessary, the medication should be adjusted. The patient and his mother should receive patient education on asthma, the use of peak flow meters, and the proper use of medications.

A burst of oral prednisone can be useful in the management of an acute exacerbation of asthma. However, due to the side effects of systemic steroids, its use is discouraged. This patient is not in acute distress and should start with inhaled steroids, which pose fewer side effects than oral/systemic steroids.

Daily-inhaled salmeterol, a long-acting beta-agonist (or LABA), should be added to an inhaled corticosteroid if low-medium doses of the inhaled steroid alone are unable to control symptoms. The salmeterol is not recommended as a stand-alone therapy, and it should only be used with other asthma control medications.

Daily oral zileuton, a 5-lipoxygenase inhibitor, is extremely expensive and is dosed 4 times daily. It should not be recommended initially when the preferred agent (low-dose inhaled corticosteroid) has not yet been tried. Additionally, compliance in teens can be difficult, so a 4-times-daily medication is not ideal.

Subcutaneous injections of omalizumab, which is an immunomodulator, are an option in step 5 and 6 patients, who have failed to achieve symptom control with multiple and high-dose medications. Omalizumab is not appropriate in this patient's case.
Correct answer:
Pleural line on chest X-ray (CXR)

Explanation
This patient is presenting with a spontaneous primary pneumothorax. A finding of a pleural line on chest x-ray (CXR) is diagnostic for this condition. A pneumothorax is a condition in which air is introduced into the lung cavity, either spontaneously or by trauma. In young people without known pulmonary pathology (especially more common in smokers), a pneumothorax may occur spontaneously. In older patients, other causes, such as a lung tumor or severe pulmonary disease, can lead to the collapse of the lung. As the lung collapses, the line from the edge of the pleura may be visible on CXR.

Blunting of costophrenic angles on CXR would indicate some type of pleural effusion or fluid in the lungs. This patient's history and physical are not consistent with a pleural effusion (other than some dyspnea may be present). Specifically, percussion over a pleural effusion should produce a dullness, not a hyperresonance.

Increased pH on ABG can certainly be seen in cases of respiratory alkalosis, associated with a pneumothorax. However, increases in pH can occur with numerous other conditions; therefore, an increase is not diagnostic for tension pneumothorax.

Oxygen saturation may drop below 90% in cases of a pneumothorax; conversely, the oxygen saturation may be normal in milder cases of pneumothorax. However, as with the ABG, decreased oxygen saturation is a common finding with various pulmonary conditions; it alone is not diagnostic for pneumothorax.

A sputum smear positive for acid-fast bacilli indicates a Mycobacterium pulmonary infection, such as tuberculosis. Classically, tuberculosis does not produce tracheal shift or hyperresonance on exam. This patient's history is not consistent with a pulmonary infection either.
Correct answer:
Ethambutol

Explanation
The correct response is ethambutol .

This patient has all the signs and symptoms of pulmonary tuberculosis (TB). Direct sputum examination by Ziehl-Nielsen stain also helps the diagnosis, but it is still not confirmatory. Sputum needs to be cultured to check what kind of mycobacterium is causing this disease. It is important to start the treatment as soon as the culture is sent.

The standard treatment for adult respiratory/pulmonary TB includes a complete 6-month regimen comprising of 2 months initial phase with 4 drugs: rifampin, isoniazid, pyrazinamide, and ethambutol. This is followed by a 4-month continuation phase consisting of 2 drugs: rifampin and isoniazid. Irrespective of the bacteriological status of the sputum, this is the recommended standard treatment for respiratory tuberculosis (including isolated pleural effusion or mediastinal lymphadenopathy). The 4th drug, ethambutol, may be omitted in patients with a low risk of resistance to isoniazid.

Ethambutol should be started in individuals who are known or suspected to be HIV-positive, in those who have had previous treatment, and in immigrants and refugees of any ethnic group who are considered to have a significantly higher risk of resistance to isoniazid and other drugs.

Like most medications, antituberculosis drugs also have some side effects. Since treatment is long-term, it is essential that patients are warned about and checked for side effects. If side effects are not explained well to the patient, it will decrease the compliance. The adverse effect of ethambutol is retrobulbar neuritis.

The important side effects of anti-tubercular drugs are:

INH: hepatotoxicity, peripheral neuritis, cutaneous hypersensitivity, (rarely) optic neuritis

RMP: hepatotoxicity, nephrotoxicity, red discoloration of the body fluids, ''flu-syndrome', and thrombocytopenic purpura

PZA: hepatotoxicity, hyperuricemia

ETH: retrobulbar neuritis

STM: nephrotoxicity, ototoxicity
Correct answer:
5 mm

Explanation
The correct response is 5 mm.

Persons with HIV should be tested yearly for tuberculosis using the purified protein derivative (PPD) skin test, also referred to as the Mantoux test. In those with HIV, and in certain other cases (refer to the table), an induration of more than, or equal to, 5 mm is considered positive. Preventive therapy should be prescribed for all patients having a positive PPD. Those with a positive skin test (or high-risk exposure) should undergo prophylaxis. A common regimen consists of isoniazid (INH) and pyridoxine daily, usually for at least 1 year. CDC recommends INH (300 mg/day) for a period of 12 months to any HIV-infected persons with positive TST (>5 mm), along with supplemental pyridoxine (25 - 50 mg/day) to prevent peripheral neuropathy. Both isoniazid-resistant and multidrug-resistant strains of Mycobacterium tuberculosis are becoming more prevalent.

Classifying positive TST reactions:

Interpretation of the tuberculin skin test*:

Induration (dia.)

Positive in persons with

>5 mm

- HIV infection
- Persons with chest X-ray findings consistent with prior TB
- Close contacts of a person with infectious TB
- Patients with organ transplant and other immunosuppressed persons

>10 mm
- Medical risk factors such as chronic renal disease, diabetes, gastrectomy, and silicosis
- Residents/employees of high-risk congregate settings (jails, nursing homes, hospitals and other long-term facilities for elderly)
- IV drug users
- Mycobacteriology laboratory personnel

>15 mm
- Healthy persons without known risk factors

* 5 TU PPD