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Terms in this set (192)

cystic fibrosis
Cystic fibrosis (CF) is a major cause of gastrointestinal and pulmonary morbidity in children due to mutations in the CF genes. The mutations lead to a deficiency in cystic fibrosis transmembrane conductance regulator protein that controls movement of salt and water into and out of epithelial cells and results in production of abnormally thick mucus. About 15% of patients with CF present with meconium ileus at birth. This is typically treated with enema for disimpaction and rarely surgery. Approximately half of the infants with CF will present with failure to thrive, which is diagnosed by lack of growth for 2 consecutive months in patients younger than 6 months of age. They may also present with respiratory compromise. However, not all patients present in childhood. Diagnosis of CF is confirmed by a sweat chloride level above 60 meq/L or with genetic testing. Treatment for patients with CF is mainly symptomatic therapy for obstructions of the digestive and respiratory tract. In addition, there is pancreatic enzyme supplementation to aid in digestion and vitamin and calorie supplementation for deficiencies in the diet. Gene therapy is now being looked at for future treatment. Intussusception (telescoping of the small intestine) typically presents in an infant with paroxysmal abdominal pain, vomiting, and diarrhea that may progress into bloody stools. Volvulus is normally the result of intestinal malrotation that causes occlusion of the superior mesenteric artery and eventual bowel necrosis. Infants typically present within 3 weeks of life with bile-stained vomiting and bowel obstruction. Wilson's disease is the defect in the ability to excrete copper in the bile that results in accumulation of copper in the liver.
A Pulmonary alveoli collapse, also known as atelectasis, occurs during operative procedures for a variety of reasons, including decreased clearance of secretions and decreased intra-alveolar pressure. Postoperatively, often due to pain, patients may not mobilize secretions appropriately, also contributing to atelectasis. Atelectasis is the most common postoperative pulmonary complication, and is often associated with emergent and prolonged surgeries, especially those of the thorax and abdomen. Atelectasis is associated with fever, an increased respiratory rate, an increased pulse, and lung exam findings ranging from normal to rales and decreased breath sounds. Symptoms usually present within the first 48 hours postoperatively.

Pulmonary aspiration pneumonitis, although possible, is less likely due to appropriate preoperative and intraoperative measures being utilized to decrease risk. Pneumonia is also a common postoperative complication, due to the same contributing factors as atelectasis. Physical exam findings may also be similar. However, postoperative pneumonia is likely to become evident between 24 and 96 hours postoperatively.
A postoperative pleural effusion may form, due to free peritoneal fluid as well as a complication of atelectasis, but has a lower incidence of occurrence than atelectasis alone. Patient symptoms will be based upon the size of the effusion, associated inflammation, and whether or not the effusion is infectious. Consideration must be given to pulmonary emboli for any post-surgical patient with tachypnea, tachycardia, and dyspnea. Pulmonary emboli may occur at any point postoperatively, but atelectasis remains a more common cause of postoperative fever and respiratory changes.
In the hypertensive patient with sudden loss of consciousness and decerebrate response (extensor posturing), you should consider brain stem hemorrhage. Abnormal breathing patterns can be observed in both pontine and medullary lesions; they sometimes can be prognostic. Her breathing pattern is apneustic. Apneustic breathing pattern characterizes deep, gasping inspiration with a pause at full inspiration followed by a brief, insufficient release and the end-inspiration pause before expiration. Lesion in the pons or upper medulla causes the removal of input from the vagus nerve and the pneumotaxic center. Normally, apneustic center of the lower pons promotes inspiration by stimulation of the dorsal respiratory center in the medulla to delay the 'switch off' signal of the inspiratory ramp provided by the pneumotaxic center of pons. Therefore, athe pneustic center controls the intensity of breathing. Apneusis is an ominous sign, with a generally poor prognosis.

Cheyne-Stokes respiration is an abnormal pattern of breathing characterized by repetitive progressively deeper and sometimes faster breathing, followed by a gradual decrease in breathing that results in temporary apnea. Cycles usually take 30 seconds to 2 minutes. Increased CO2 during the period of apnea causes compensatory hyperventilation. Hyperventilation in turn causes the decrease in CO2, which causes apnea and the cycle to restart. Causes include CNS dysfunction, cardiac failure with low cardiac output, sleep, hypoxia, or profound hypocapnia.

Ataxic breathing (Biot's respiration) is an abnormal pattern of breathing characterized by groups of quick, shallow inspirations followed by regular or irregular periods of apnea. It is generally a poor prognostic sign. Biot's respiration is caused by the medullary lesion due to strokes or trauma or by pressure on the medulla due to uncal or tentorial herniation.

Kussmaul breathing is a deep, labored, and gasping breathing pattern seen often in severe metabolic acidosis (diabetic ketoacidosis, renal failure). In metabolic acidosis, breathing is first rapid and shallow, but later on, as acidosis worsens, breathing gradually becomes Kussmaul breathing.

Central neurogenic hyperventilation is a very deep and rapid pattern of breathing, usually seen in the lesions of the midbrain and upper pons. Respirations are generally regular, and the PACO2 decrease due to the hyperventilation.

Cluster breathing is irregular breathing with periods of apnea that occurs at irregular intervals. It is generally seen in lesions in the low pons or upper medulla. It differs from Cheyne-Stokes pattern because there is no increasing and decreasing depth of respirations.
A 72-year-old man presents due to worsening shortness of breath, orthopnea, and chest pain; symptoms have been occurring for the past few weeks. The patient admits to some chronic heart problems as well as fatigue, dyspnea, and a non-productive cough; however, he feels like symptoms have worsened recently. He denies fever, chills, and a productive cough.

On physical exam, the man has mildly increased respiratory effort, but he does not appear in distress. He is barrel-chested. His breath sounds are diminished bilaterally, with dullness to percussion over the right and left lower lungs. No pleural friction rub is noted. On cardiovascular exam, an S3 gallop and mild tachycardia (110 bpm) are noted. Clubbing of the fingers, dependent edema in the lower extremities, and jugular venous distention are also noted. His cardiac enzymes and electrocardiogram demonstrate no acute cardiac pathology.

Pleural fluid and cardiomegaly are found on the chest X-ray, and a thoracentesis is performed. The pleural fluid is generally clear in color, testing negative for chylomicrons and triglycerides. It has low levels of red blood cells, white blood cells, protein, and lactate dehydrogenase (LDH).

What is the likely underlying mechanism for the pleural effusion in this patient?

Answer Choices
1 Chylothorax from disruption of the thoracic duct
2 Empyema from infection in the pleural space
3 Exudates from local inflammation in capillary beds
4 Hemothorax
5 Transudates from increased hydrostatic pressure or decreased oncotic pressure
All of the listed choices are possible mechanisms for pleural effusions. According to this patient's history and physical exam, as well as the characteristics of the pleural fluid, it should be apparent that his pleural effusion stems from transudates from increased hydrostatic pressure or decreased oncotic pressure. He presents with typical chronic congestive heart failure symptoms. The pleural effusion occurs not from diseased pleura, but from the underlying illness. Transudative pleural effusions are the most common type, and heart failure is the most common underlying cause of pleural effusions.

Chylothorax from disruption of the thoracic duct is relatively uncommon. The primary distinguishing characteristics are seen in the milky appearance of pleural fluid, with a large amount of chylomicrons and triglycerides.

Empyema from infection in the pleural space might have been a cause if this patient had pneumonia or other severe pulmonary infection. This patient's clinical history is not suggestive of infection. The pleural fluid of empyema may be grossly purulent or milky white. If the milky white fluid is centrifuged, showing clear supernatant with a pellet of white cells below, empyema should be considered.

Exudates from local inflammation in capillary beds is a mechanism for pleural effusions secondary to pulmonary embolism, malignancy, as well as some infections, such as pneumonia. Exudates can be distinguished from transudates by a combination of history, appearance, and characteristics of the pleural fluid (using Light's criteria). Simplified, the pleural fluid with exudates may be cloudy in appearance, with relatively high levels of protein and LDH.

Hemothorax is blood in the pleural space. It can occur with trauma to the chest wall or other structures of the thorax. Typically, there would be a history of trauma. Hemothorax is unlikely to be bilateral, except in cases of significant trauma. Additionally, the pleural fluid may appear grossly bloody and demonstrate high numbers of red blood cells on analysis.
Daily low-dose inhaled budesonide
This patient is exhibiting persistent asthma symptoms. Using the step-wise 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 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 the 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.
Sarcoidosis is a multisystem granulomatous disease. Histologically, it is characterized by the presence of non-caseating granulomas, hence the diagnosis.

The mediastinal and superficial lymph nodes, lungs, liver, spleen, skin, eyes, parotid glands, and phalangeal bones are frequently involved; however, all tissues may be involved. Sarcoidosis is not fatal unless it affects the vital organs such as the heart or the central nervous system. Calcium metabolism may be disturbed, causing hypercalcemia and (rarely) nephrocalcinosis and renal failure.

30% of the patients of sarcoidosis are asymptomatic. The disease is commonly detected by an abnormal chest radiograph, which reveals bilateral hilar lymphadenopathy in an asymptomatic patient. However, with extensive lung involvement, there may be exertional dyspnea or cough.

The diagnosis can be made confidently from the clinical and radiological features. It can also be confirmed histologically from the biopsy of a superficial lymph node or skin lesion.

Kveim test is a useful diagnostic procedure for sarcoidosis. The antigen (0.1 ml) obtained from human sarcoid tissue is injected intradermally and a small nodule develops 4 weeks later. If the test is positive, biopsy of the nodule reveals typical sarcoid follicle.

The development of positive Kveim test is suppressed by corticosteroid therapy.

Asymptomatic cases resolve spontaneously, but patients with persistent erythema nodosum, pyrexia, and arthralgias require oral corticosteroid therapy for a short duration. Symptomatic pulmonary sarcoidosis and sarcoidosis involving the eye or other vital organs usually needs to be treated with corticosteroids for several years.

Adenocarcinoma will yield malignant cells, not granulomas, on biopsy.

Histoplasmosis is caused by dimorphic fungi, Histoplasma capsulatum, or a variant, Histoplasma duboisii, which is found in the soil. It is caused by inhalation of the infected dust, which produces lesions similar to tuberculosis and have granulomas with central caseation.

Interstitial pneumonitis is the end result of diffuse fibrosing alveolitis. Many cases are idiopathic. The end stage is a honeycomb appearance and no granulomas are seen.

Berylliosis produces sarcoid-like lesions, but there would have to be a history of exposure to beryllium. Exposure to beryllium is now extremely uncommon.
2 Initiate diuretics and refer to a pulmonologist this week
This patient with pulmonary hypertension (which may be progressing into cor pulmonale) needs specialist care. It would be most appropriate to initiate diuretics and refer her to a pulmonologist this week. Treatment medications for pulmonary hypertension are complex; they can include endothelin receptor antagonists, phosphodiesterase inhibitors, intravenous prostacyclins, and others.

This patient has presented to the outpatient clinic and is stable. If left untreated, her condition may deteriorate, but her current state is not an acute emergency requiring an ambulance for immediate transport to the emergency department.

If the patient had pneumonia (you would expect a history of cough, fever, chills, and physical exam findings suggesting infection), it would be appropriate to initiate a macrolide antibiotic and cough syrup as well as follow-up in 2 days. However, this patient's pulmonary condition is not secondary to infection.

If the patient's dyspnea was caused by asthma, it would be reasonable to prescribe an albuterol inhaler for use as needed when shortness of breath occurs. However, albuterol (a short-acting beta-agonist) will not address her pulmonary hypertension or the cor pulmonale.

As with the discussion above regarding emergency transport, this patient does not present with an urgent or emergent condition that requires inpatient management. If there were indicators that the patient would not be compliant with outpatient care, inpatient admission could be considered. However, this patient describes prior specialist care, and it can be presumed she has been compliant with appointments, testing, and her home oxygen.
Cor Pulmonale
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.
Oxygen therapy

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 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.

Anticoagulation with warfarin is recommended in patients at high risk for thromboembolism. The evidence of benefit has not been established in patients with secondary PAH. Therefore, anticoagulation therapy may be used in patients with cor pulmonale secondary to thromboembolic phenomena and with underlying primary pulmonary artery hypertension.

The use of cardiac glycosides, such as digitalis, in patients with cor pulmonale has been controversial, and the beneficial effect of these drugs is not as obvious as in the setting of left heart failure. This drug must be used cautiously and should not be used during the acute phases of respiratory insufficiency, when large fluctuations in levels of hypoxia and acidosis may occur. Patients with hypoxemia or acidosis are at increased risk of developing arrhythmias due to digitalis through different mechanisms, including sympathoadrenal stimulation.

Some classes of vasodilators, such as beta agonists, nitrates, and angiotensin-converting enzyme (ACE) inhibitors, have been tried, but, in general, vasodilators have failed to show sustained benefit in patients with COPD and are not routinely used.
Breast Cancer
A pleural effusion results from an abnormal accumulation of fluid in the pleural space. Transudative pleural effusions suggest the absence of localized pleural disease and are caused by increased hydrostatic pressure, as seen in heart failure, or decreased oncotic pressure, as seen in cirrhosis. Because there is no underlying pleural disease process, transudative pleural effusions are often bilateral. Exudative pleural effusions are indicative of an underlying pleural disease process, such as pneumonia or a malignancy. A diagnostic thoracentesis can help distinguish transudative from exudative pleural effusions. The fluid should be sent for a cell count, chemistry, and, if indicated, cytology or culture. A pleural effusion is diagnosed as exudative based on the chemistry when the pleural fluid meets 1 or more of the following criteria: "1) ratio of pleural fluid protein to serum protein greater than 0.5; 2) ratio of pleural fluid LDH to serum LDH greater than 0.6; and 3) pleural fluid LDH greater than 2/3 the upper limit of normal serum LDH."

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

A patient with chronic kidney disease, heart failure, or cirrhosis is more likely to have bilateral transudative pleural effusions. Hypertension by itself is not a risk factor for a pleural effusion.
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.
Normal Saline
The clinical presentation is suggestive of a hypercalcemia of malignancy. It is most frequently associated with multiple myeloma, breast, lung, and kidney cancers, as well as T-cell lymphomas. The hypercalcemia is usually a result of increased bone resorption by osteoclasts. Patients present with anorexia, nausea, vomiting, constipation, fatigue, and headache. When the plasma calcium level rises above 12 mg/dL, they may also present with confusion, delirium, and coma. Laboratory results may also reveal an elevated blood urea nitrogen and creatinine levels. The 1st step in the management of severe hypercalcemia of malignancy, when the renal function is normal, is aggressive rehydration with intravenous normal saline while closely monitoring urine output and electrolytes. The bisphosphonate pamidronate, which acts as a bone resorption inhibitor, can then be given intravenously in addition to the normal saline.

Calcitonin, another bone resorption inhibitor, is also used to manage severe hypercalcemia of malignancy when it is combined with prednisone; however, some patients are unresponsive to this combination.

Hemodialysis, along with a calcium-free dialysate and peritoneal dialysis, is also used to lower the calcium levels (especially in patients with renal failure).

Plicamycin, which inhibits bone resorption, is also effective in lowering plasma calcium; however, due to its potentially severe adverse effects, it should be given if there is insufficient response to the other medications.
This patient's most likely diagnosis is idiopathic pulmonary fibrosis (IPF). It is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). Patients with hypoxemia (PaO2 < 55 mmHg or oxygen saturation as measured using pulse oximetry [SpO2] < 88%) at rest or with exercise should be prescribed oxygen therapy to maintain a saturation of at least 90% at rest, with sleep, and with exertion.

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

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

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

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

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

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.
This patient has a presentation most consistent with cor pulmonale. Chronic obstructive pulmonary disorder is the most common cause of secondary cor pulmonale while primary pulmonary hypertension is idiopathic. 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.

Diuretics such as furosemide are used in the management of chronic cor pulmonale, particularly when the right ventricular filling volume is markedly elevated and in the management of associated peripheral edema. These agents may result in improvement of the function of both the right and left ventricles.

Propranolol is a nonselective beta adrenergic receptor blocker whose use is contraindicated in patients with bronchospastic disease (such as asthma), COPD and congestive heart failure. Beta-selective agonists, not antagonists, demonstrate the advantages of bronchodilator and mucociliary clearance effect in the treatment of cor pulmonale.

Warfarin is recommended in patients at high risk for thromboembolism. The beneficial role of anticoagulation in improving the symptoms and mortality in patients with primary PAH has been demonstrated in several studies. The evidence of benefit, however, has not been established in patients with secondary PAH. Therefore, anticoagulation therapy may be used in patients with cor pulmonale secondary to thromboembolic phenomena and with underlying primary PAH.

Digitalis should not be used during the acute phases of respiratory insufficiency when large fluctuations in levels of hypoxia and acidosis may occur. Patients with hypoxemia or acidosis are at increased risk of developing arrhythmias due to digitalis through different mechanisms, including sympathoadrenal stimulation.

Vasopressin possesses both vasoconstrictor and antidiuretic properties, both of which contribute to elevating arterial pressure. Its use is not appropriate for cor pulmonale. Rather, vasodilators have been advocated in the long-term management of chronic cor pulmonale with modest results. For instance, calcium channel blockers (particularly oral sustained-release nifedipine and diltiazem) can lower pulmonary pressures.
Positron emission tomography (PET) scan
A chest CT scan is an important part of a solitary lung nodule. It is more sensitive than plain radiography and can determine whether a nodule identified on a chest film is indeed solitary. It provides better visualization of the nodule, is more sensitive for calcification, and can be used to guide thoracic needle aspiration biopsies.

After the CT of the chest, the next step would be the PET scan. This study uses uptake of 2-fluoro-dexy-D-glucose (FDG) to measure glucose metabolism. The uptake of FDG by lung tumors is greater than that of normal lung parenchyma. In a patient with a solitary nodule, the sensitivity and specificity of PET for malignancy are about 95% and 90% respectively. With nodules less than 1.5 centimeters, PET is less reliable and also produces a higher rate of false-negatives. PET is particularly useful in evaluating indeterminate lesions in patients who are a poor surgical risk. Other benefits of PET scanning are detection of occult metastases and improved staging. A CT-PET strategy has been shown to be more cost-effective and also prevents more unnecessary thoracotomies than conventional approaches. CT-PET can demonstrate nodule enhancement. PET scanning is also the preferred approach in patients who have a solitary lung nodule classified as "indeterminate" based on the initial chest film and CT scan. If the PET scan is negative, follow up with serial CT scans. A positive PET scan indicates that malignancy is likely. PET is particularly useful in evaluating lesions in patients who are a poor surgical risk. Other benefits include detection of occult metastases and improved staging.

A bone scan would be useful when the patient has symptoms of bone pain, mid-thoracic pain, or night fevers. An incidental finding of a high serum calcium level in this clinical setting may also warrant a bone scan.

Bronchoscopy and transthoracic needle aspiration biopsy (TNAB) are the traditional options. Bronchoscopy may be useful if the lesion is 2 centimeters or more in diameter and can be accessed via a bronchus. In other settings, the false-negative rate is high; thus, in most patients, bronchoscopy is of limited value and has a relatively low diagnostic yield. TNAB is considered when the solitary nodule is smaller than 2 centimeters. The diagnostic yield is high for peripheral lesions. A negative result on TNAB may reliably rule out pulmonary mycosis in patients who live in areas where the incidence of this infection is high. TNAB has a higher complication rates than bronchoscopy.

Although thoracotomy is the most definitive way to establish a diagnosis in patients with a solitary lung nodule, operative mortality is 3% to 7%. If it is video-assisted, then there is lower perioperative morbidity and a shorter hospital stay. In 25% of cases, a video-assisted thoracoscopic procedure needs to be converted to an open thoracotomy.
Ziehl-Neelsen stain
This patient has the clinical features of pulmonary tuberculosis; in the United States, TB is common in immigrants from developing countries. For rapid diagnosis of pulmonary TB, direct sputum examination with Ziehl-Neelsen stain is the best choice. The culture should be sent at the same time to identify the type of Mycobacterium and the sensitivity to the drugs. Mycobacterium are aerobic, acid-fast bacilli. They are neither Gram-positive nor Gram-negative, but stain red with Ziehl-Neelsen stain because of the N-glycolyl muramic (mycolic) acid in their cell walls. The lipid content of the cell wall is very high; therefore, Mycobacterium is hydrophobic and impermeable to basic aniline bacteriologic stains.

Gram staining is the most common stain used for most of the bacteria, but it is not used for Mycobacterium.

Giemsa stain is used for tissue, blood, or secretions to see the microorganisms. It is commonly used for Neisseria species, malaria, and other blood parasites.

Direct immunofluorescence is useful in the identification of many microorganisms such as Bordetella pertussis and Chlamydia trachomatis. Fluorescein-labeled monoclonal or polyclonal antibodies against specific microbial antigens are added to a tissue or cell suspension; this enables it to detect a specific microorganism.

Darkfield examination uses a microscope that illuminates the object over a dark background. It is used extensively to identify spirochetes, which are too thin to be seen easily by staining.
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 5mm, 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 (300mg/day) for a period of 12 months to any HIV-infected persons with positive TST (> 5 mm), along with supplemental pyridoxine (25 - 50mg/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

> 5mm
- HIV infection
- People 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

> 10mm
- 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

> 15mm
- Healthy persons without known risk factors

* 5 TU PPD
Wegener's Granulomatosis
Wegener's Granulomatosis is a small vessel vasculitis of unknown pathogenesis that causes inflammation and necrotizing granulomas in the upper and lower respiratory tract and kidneys, as well as rash; 15 - 50% of patients have cutaneous involvement (Brenner, chapter 31). Palpable reddish-purple lesions on the lower extremities should raise suspicion of vasculitis; macules and nodules, may also erupt. Some 50 - 95% of patients with Wegener's will eventually have renal involvement (Brenner), which may range from mild findings to fulminant dialysis-requiring nephritis. On renal biopsy, focal segmental necrotizing glomerulonephritis is noted; crescents may be seen. Immune staining is generally negative. Treatment involves immunosuppressive therapy, as is the case with SLE, and dialysis as needed. In some resistant cases, plasmapheresis may be helpful. Patients with Wegener's are often Anti-Neutrophil Cytoplasmic Antibody (ANCA) positive.

Patients with Goodpasture's syndrome typically present with a triad of glomerulonephritis, pulmonary hemorrhage, and anti-glomerular basement membrane (GBM), type IV collagen antibodies. Many patients with Goodpasture's syndrome are ANCA positive.

SLEis a systemic inflammatory disease that causes a variety of signs and symptoms affecting many organs. Renal involvement is frequent in SLE and may include a variety of glomerular diseases, causing hematuria, proteinuria, and urinary casts.

Henoch-Schönlein Purpura (HSP) is a vasculitic syndrome that is seen more often in children than adults; it may cause petechiae, purpura, gastrointestinal bleeding, and hematuria/glomerulonephritis. Complement counts and anti-double-stranded DNA are normal, and elevated IgA antibody levels may be seen.

Rheumatoid arthritis (RA) is an inflammatory arthritis that causes joint stiffness and swelling that takes a long time to loosen up after prolonged sitting or sleeping. Symmetrically swollen joints and rheumatoid factor may be noted, but vasculitis is uncommon in RA (Harrison's chapter 314).
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.
Malignancy is the correct response.

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

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

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

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

Congestive heart failure accounts for >90% of transudative pleural effusions. Nephrotic syndrome and cirrhosis with ascites can also lead to transudative pleural effusions. Bacterial pneumonia and cancer are the most common causes of exudative effusions.
Explanation History of previous Pneumocystis jiroveci pneumonia (PCP) infection is the correct answer. Patients who are HIV positive should take antibiotic prophylaxis for PCP if they have a history of PCP infection, if their CD4 cell count is below 200, or if they have evidence of immunodeficiency, such as oral candidiasis. Prophylaxis for PCP is typically daily trimethoprim/sulfamethoxazole double strength, but it can also be done with daily dapsone or monthly aerosolized pentamidine. If patients are on antiretroviral therapy and their CD4 cell count remains above 200 for at least 3 months, then they can stop prophylaxis. If their CD4 cell count drops below 200 again, they need to restart prophylaxis.

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

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

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

HIV viral load >100,000 copies/mL is not the correct answer. HIV viral loads over 100,000 warrant antiretroviral treatment of HIV, but PCP prophylaxis is not given based on viral load. Rather, it is given based on CD4 cell count, previous PCP infection, or evidence of immunodeficiency.
2 Lung resection including the middle and lower lobe
Occasionally, patients present with resectable localized lung cancer and evidence of solitary metastasis. These patients should be considered for combined approach in which the primary tumor and solitary metastasis are resected. Locations that are amenable to a combined approach include adrenal, brain, lung, and bone. Most brain metastases are histologically adenocarcinoma in this case. Cranial resection is done first and thoracotomy thereafter, unless symptoms from the primary tumor site dominate the clinical picture.

The best surgical treatment for this particular lung cancer is lower and middle lobectomy. Lower lobectomy alone would leave a cancerous middle lobe and offer no survival advantage. Pneumonectomy would be an unnecessary extensive resection in view of the bronchoscope findings. Also, a pneumonectomy would have a higher mortality rate without the benefit of a higher cure rate.

For patients who are unable or unwilling to undergo surgery, radiation can be used with curative intent. The long-term survival rate for patients with early stage resectable disease is from 17%-33%. Results of curative photodynamic therapy are usually for microinvasive carcinoma, defined as lesions less than 10 mm. In 1997, a report documented 9 of 21 patients had no evidence of disease at a mean of 68 months. Currently, there is a limited role for photodynamic therapy with primary respectable lung cancer due to higher survival rates with surgical removal.
Avoid aspirin
Your patient most probably has aspirin-exacerbated respiratory disease. It is chronic sinusitis characterized by nasal polyposis, nonallergic induced asthma, and aspirin sensitivity. The condition is sometimes called aspirin triad. It commonly starts in the third decade. Clinical symptoms of aspirin-sensitive patients are characterized by mucosal inflammation and rhinitis, severe asthma precipitated by aspirin ingestion, and aggressive bilateral nasal polyposis. The rhinitis is persistent and difficult to manage, and the rhinorrhea is thin and nonpurulent (nonallergic rhinitis with eosinophilia syndrome). Asthma usually appears an average of 2 years after rhinitis, followed by the intolerance to aspirin and the co-occurrence of nasal polyps. Severe acute asthma attack can occur within a few minutes and up to 3 hours after ingestion of aspirin. Aspirin challenge can be used to confirm a diagnosis of aspirin sensitivity in these patients.

Antihistamines are commonly used for the relief of allergies caused by intolerance of proteins. They will not help your patient.

Inhaled cromolyn or nedocromil, inhaled corticosteroids, long-acting bronchodilators, and other drugs are used to control asthma, but they will not to prevent asthmatic attack in this patient.

Albuterol and other quick relief medications used to relieve acute asthma exacerbations, to prevent exercise-induced asthma symptoms, and to speed recovery from acute exacerbations, but albuterol will not prevent aspirin-exacerbated respiratory disease.

Influenza vaccine is useful to prevent influenza and the eventual need for the use of nonsteroidal anti-inflammatory drugs. It will not, however, prevent aspirin-exacerbated respiratory disease.
Metastatic infiltrating ductal carcinoma
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 occurring 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 is transudative in nature.

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

Bacterial sepsisdue 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.
Asthma is the correct answer. The vignette describes a child with atopic dermatitis, one of the most common childhood dermatological conditions. It usually begins before 2 years of age and displays a chronic relapsing course. Typical sites of involvements are the cheeks, flexural aspect of the elbows, natal cleft, and the hands. Clinical examination usually reveals xerosis, lichenification, and eczematous lesions. Atopic dermatitis is commonly associated with other atopic conditions, such as hay fever, extrinsic asthma, allergic conjunctivitis, and allergic rhinitis. A high IgE level and eosinophilia are common to all of these.

Gluten enteropathy is incorrect. Gluten enteropathy or celiac disease may be associated with skin manifestations, namely dermatitis herpetiformis. The typical patient is middle aged and develops crops of fluid filled blisters resembling herpetic lesions (hence the name herpetiformis). The face, hairline, and shoulder are common sites. IgE levels are usually normal. The association with celiac disease is strong; associated symptoms of abdominal pain, nausea and bloating may be contributory findings.

Vitamin A deficiency is incorrect. Vitamin A deficiency may result in dry, thickened 'toad skin'; it may also be associated with frequent skin infections. Eye manifestations, such as night blindness, corneal ulcers, and xerophthalmia, are more common.

Psoriasis is incorrect. Psoriasis usually causes discrete scaly lesions and is more common in older age groups, except for Guttate psoriasis. It shows a predilection for extensor surfaces and the scalp, as opposed to atopic dermatitis, which favors the flexural aspects.

Raised Dengue titers is incorrect. Dengue fever is an arboviral infection that may cause a fine maculopapular rash throughout the body. Dengue hemorrhagic fever may be associated with a low platelet count and petechial rashes. They are never associated with chronic discrete rashes.
Mediastinum shifted to the left is the correct answer. Patients with a pleural effusion that is over 1000mL can experience a shift in the mediastinum away from the side with the larger effusion. A pleural effusion is an accumulation of fluid within the pleural cavity, which is the area between the chest wall and the lung. The increased pressure within the affected side of the chest causes the mediastinum, including the trachea, to shift away from the side with the larger amount of fluid (and therefore larger amount of pressure). In this case, the mediastinum would shift towards the left, as the right side has almost twice the amount of fluid as the left.

Decreased tactile fremitus is not the correct answer. Tactile fremitus is vibration felt on a patient's chest while the patient vocalizes at low frequency. The patient is asked to repeat the same phrase over and over while the examiner moves their palm throughout the various lung fields. Tactile fremitus can be decreased in cases of any pathology separating the lung tissue from the chest wall. Pleural effusion is a common cause of decreased tactile fremitus. It is incorrect in this case because a physical examination is very nonspecific. The examiner would not necessarily know from assessing tactile fremitus the amount of pleural effusion or the differentiation between the sides.

Mediastinum shifted to the right is not the correct answer. If the patient's mediastinum was shifted toward the right in this case, then it would be shifted toward the affected side. In patients with large pleural effusions, the side with the higher amount of pressure pushes the shift away from the affected side. Patients with atelectasis, pleural fibrosis, or who are status post-pneumonectomy would experience a decreased pressure within the pleural cavity on 1 side; therefore, the mediastinum would be shifted toward that affected side.

Pleural friction rub is not the correct answer. A pleural friction rub is heard on cardiac auscultation and is the result of the pleural linings rubbing together. In cases when the pleural linings are inflamed (such as in pneumonia, pleural effusion, or pulmonary embolism), a pleural friction rub can be heard. The finding is nonspecific in terms of the amount of fluid or differentiation between sides; therefore, it would not necessarily be indicative of these particular thoracentesis findings.

Bilateral inaudible breath sounds is not the correct answer. Due to the increased fluid and pressure associated with pleural effusion, the breath sounds may not be heard during physical examination. However, not hearing the sounds on either side does not give any indication as to how severe the pleural effusion is (although this typically happens in the larger effusions) and certainly does not show the differentiation between sides.
Croup, which is caused by the parainfluenza virus, is an upper respiratory infection with a characteristic brassy cough, hoarseness, and respiratory stridor. The typical clinical picture has a variable onset, from a progressive fever, sore throat, and cough for a couple of days to a sudden midnight onset. The severity varies from stridor only (with mild agitation) to stridor at rest to airway obstruction. Treatment involves humidified air, inhaled or oral corticosteroids, and nebulized racemic epinephrine.

Bronchiolitis, caused by the respiratory syncytial virus (RSV) and other respiratory viruses (influenza, parainfluenza, rhinovirus), is a severe infection in infants that can progress to respiratory failure. Premature infants and infants with heart and lung disease have severe forms of bronchiolitis. The patients have wheezing, rales, prolonged expiratory phase, rhinorrhea, nasal congestion, fever, tachypnea, and respiratory distress. Antigen testing for RSV from nasal secretions provides a rapid diagnosis. Ribavirin administered through aerosol may shorten the clinical course. Polyclonal and monoclonal antibodies are used for prophylaxis in wintertime.

Epiglottitis, which is caused by Haemophilus influenzae, is characterized by inflammation and rapidly progressive edema of epiglottis and contiguous tissue. Children ages 2 to 7 years who missed some immunizations are prone to this infection during winter. Drooling, hoarseness, high fever, sore throat, the characteristic 'sniffing dog' position, and the rapidly progressive respiratory obstruction make the diagnosis a clinical one. Immediate intubation and intravenous ceftriaxone or cefuroxime are recommended for treatment.

Foreign body aspiration presents with complete or partial airway obstruction localized at a different location in the respiratory tract. The acute presentation after a choking episode provides the diagnosis; however, without a witnessed episode of choking, clinical presentation may take a couple of days after the aspiration. Tracheal obstructions present with acute asphyxia in total obstruction and with stridor in partial obstruction. Localized signs, such as wheezing, rhonchi, and decreased breath sounds, are found in lower respiratory tract obstruction. The treatment of choice is extraction with rigid bronchoscope.

Pneumonia in children may be a difficult clinical diagnosis. The immune status, the age, and risk factors may help in suspicion of the etiologic organism. Profuse crackles and rales after an upper respiratory infection may suggest a viral pneumonia. A localized consolidation syndrome is more characteristic for a bacterial origin of pneumonia. The characteristic findings on chest radiography also suggest the etiology of the pneumonia.
Tracheomalacia is a common cause of persistent wheezing in early infancy, with male preponderance in the ratio of 2:1. In primary tracheomalacia, there is insufficient cartilage to maintain the patency of the airway throughout the respiratory cycle. It commonly occurs in premature infants. Secondary tracheomalacia occurs when trachea is compressed by structures like vascular rings or deficient cartilage due to tracheoesophageal fistula.

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

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

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

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

Vocal cord paralysis is the 2nd most common cause of neonatal stridor. It is often associated with central nervous system lesions like meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. Bilateral vocal cord paralysis typically produces airway obstruction manifested by high-pitched inspiratory stridor. Unilateral paralysis causes aspiration, coughing, and choking. Cry is weak. Other symptoms of airway obstruction are less common. Diagnosis of vocal cord paralysis is made by awake flexible laryngoscopy with ultrasonography, which is a useful adjunctive examination.
Maternal smoking during pregnancy
SIDS is considered the sudden death of an infant under the age of 1 year that is unexplained by thorough case investigation, autopsy, and review of case history. The occurrence peaks between 2 - 3 months of age. Since 1994, when the Back to Sleep campaign began recommending placing infants in a supine position, there has been a consistent decrease in SIDS rate; however, SIDS is still responsible for more infant deaths in the United States than any other cause of death during infancy beyond the neonatal period. The predominant hypothesis concerning the etiology remains that certain infants may have a maldevelopment or delay in maturation of the brainstem neural network responsible for arousal, and this affects the physiologic response to life-threatening challenges during sleep.

Independent risk factors for SIDS that have been consistently identified across studies are prone sleep position, sleeping on a soft surface, overheating, late or no prenatal care, young maternal age, preterm birth and/or low birth weight, male gender, and maternal smoking during pregnancy. Almost every epidemiologic study has shown maternal smoking during pregnancy to be a major risk factor. Smoking after birth has emerged as a separate risk factor, but separating this variable from maternal smoking before birth is difficult.

Bed sharing is highly controversial. Although it facilitates breastfeeding and enhances the infant-maternal bond, it can be hazardous under certain conditions. SIDS risk seems to be particularly high with multiple bed sharers, especially if alcohol is consumed. It can be extremely hazardous when sleeping with an infant on a couch. The longer the duration of bed sharing is during the night, the higher the risk. There is, however, evidence that room sharing without bed sharing is the most protective sleep setting for the 1st 6 months and should be recommended.

Approximately 20% of SIDS deaths occur while in the care of a non-parental caregiver. Many of these deaths have been associated with the prone sleep position, especially when the infant is accustomed to sleeping supine. This has shown to actually increase the risk of SIDS 18-fold. Some centers are unaware of the dangers of sleeping prone and/or are misinformed of the risks and benefits of sleep positions.

Studies have shown that infants dying of SIDS were more likely to have used a pillow or soft mattress, and they were found with their nose and mouth completely covered by bedding or assumed a facedown posture. Soft objects such as stuffed toys, quilts, blankets, and loose bedding should be kept out of the crib. Blankets should be tucked in around the crib mattress.

Several new studies have reported an actual protective effect of pacifiers on the incidence of SIDS. The mechanism is unclear, but lowered arousal threshold among users has been proposed. Others theorize that pacifier use may enhance an infant's ability to breathe through the mouth if the nasal airway becomes obstructed. Retroposition of the tongue can lead to obstructive apnea and asphyxiation; sucking of the pacifier requires forward positioning of the tongue, decreasing risk of oropharyngeal obstruction..

There has also been a concern raised about an approximate 1 - 2 fold increased risk of otitis media associated with pacifier use, but this incidence is generally lower in the 1st year of life, especially in the 1st 6 months, which is when the risk of SIDS is highest.
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.
The diagnosis is pulmonary embolism, which should always be suspected if the patient suddenly collapses 1 - 2 weeks after surgery. Pulmonary embolism develops due to blockage of blood flow to the lungs caused by venous thrombi from deep vein thrombosis passing into the pulmonary circulation. It can be prevented by mobilizing the patient early following surgery and considering prophylaxis with heparin. Classically, it presents around 10 days post-op with collapse and sudden breathlessness while straining at stool; however, it may occur after any period of immobility or with no predisposing factors. Other features include pleuritic pain, dyspnea, shock, tachypnea, cyanosis, hemoptysis, gallop rhythm, pleural rub, right ventricular S3 gallop, and loud P2. Chest X-ray is often normal and sometimes with decreased vascular markings. Diagnosis is usually made clinically and confirmed by ventilation-perfusion mismatch on lung VQ (ventilation-perfusion) scan. An isotope lung scan using the macroaggregated albumin followed by ventilation scan using 133 Xenon can provide complementary evidence that the perfusion problem is due to pulmonary emboli.

Initial management of this patient is adequate heparinization. Most patients with pulmonary emboli would resolve on adequate anticoagulation. The initial dose would be given intravenously and could be up to 10,000 units.

Pulmonary embolectomy is indicated in only a very small group of patients who have massive embolus and are in a critical state.

Exploratory laparotomy would be fruitless, as there will be no pathology inside the abdomen because the cause of this incident lies in insufficient physical mobilization after the operation.

Treatment with streptokinase or urokinase is not recommended in this postoperative patient.

Vena cava filter is indicated in patients who continue to throw emboli in spite of adequate anticoagulation or in whom heparinization is not advisable.
Influenza vaccine
Chronic obstructive pulmonary disease (COPD) affects approximately 32 million Americans and is the 4th leading cause of death in the US (1). The main cause of COPD is smoking. For this reason, COPD is considered a preventable disease.

"The Global Initiative for Chronic Obstructive Lung Disease (GOLD) guidelines define COPD as a disease state characterized by airflow limitation that is not fully reversible, is usually progressive, and is associated with an abnormal inflammatory response of the lungs to inhaled noxious particles or gases" (Mosenifar, 2011, Overview).

Treatment of COPD is aimed at limiting exacerbations and progression of disease. Smoking cessation is an important component of the treatment of COPD, but it will only be effective if the patient is willing to attempt to quit. Nicotine patches or gum can be used as nicotine replacement therapy to aid in smoking cessation. The dose of nicotine is gradually decreased. Bupropion (Zyban) is an antidepressant that can be used in conjunction with behavioral therapy to help in smoking cessation (1).

Pharmacological treatment of stable COPD includes treating the airflow restriction and inflammation. Inhaled bronchodilators, such as beta-agonists and anticholinergic agents, are the mainstay of therapy. "Tiotropium is the only long-acting muscarinic agent available at this time and has become a first-line therapy in patients with persistent symptoms" (Mosenifar, 2011, Treatment).

Combination therapy of inhaled beta-agonist and corticosteroids or inhaled anticholinergic and corticosteroids are indicated when a patient's symptoms are not controlled by monotherapy alone (1). In this case, the patient is controlled on tiotropium, so the addition of fluticasone and salmeterol (corticosteroid and long-acting beta-agonist) is not warranted.

Pulmonary infections can lead to a COPD exacerbation; therefore, patients should routinely receive pneumococcal and influenza vaccines. The CDC recommends adults receive 1 dose at age 65 years. In the case presented, the patient had his vaccine at age 65 and does not need another one. The current recommendation for the influenza vaccine is that everybody receives an annual dose (2). Therefore, the patient in this case should be given an influenza vaccine at this visit.
Small cell carcinoma
Small cell carcinoma is most frequently a central lesion not associated with cavitation. A hilar mass is associated with small cell carcinoma. Small cell carcinoma is associated with paraneoplastic syndromes. Cushing's syndrome secondary to ectopic ACTHproduction can sometimes be seen with small cell carcinoma, as can syndrome of inappropriate antidiuretic hormone production (SIADH).

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

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

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

Squamous cell carcinoma is centrally located. Cavitation can also sometimes be seen with X-ray. Keratin formation, keratin pearl formation, and intercellular bridges can be seen with squamous cell carcinoma.
Mediastinum shifted to the left
Mediastinum shifted to the left is the correct answer. Patients with a pleural effusion that is over 1000mL can experience a shift in the mediastinum away from the side with the larger effusion. A pleural effusion is an accumulation of fluid within the pleural cavity, which is the area between the chest wall and the lung. The increased pressure within the affected side of the chest causes the mediastinum, including the trachea, to shift away from the side with the larger amount of fluid (and therefore larger amount of pressure). In this case, the mediastinum would shift towards the left, as the right side has almost twice the amount of fluid as the left.

Decreased tactile fremitus is not the correct answer. Tactile fremitus is vibration felt on a patient's chest while the patient vocalizes at low frequency. The patient is asked to repeat the same phrase over and over while the examiner moves their palm throughout the various lung fields. Tactile fremitus can be decreased in cases of any pathology separating the lung tissue from the chest wall. Pleural effusion is a common cause of decreased tactile fremitus. It is incorrect in this case because a physical examination is very nonspecific. The examiner would not necessarily know from assessing tactile fremitus the amount of pleural effusion or the differentiation between the sides.

Mediastinum shifted to the right is not the correct answer. If the patient's mediastinum was shifted toward the right in this case, then it would be shifted toward the affected side. In patients with large pleural effusions, the side with the higher amount of pressure pushes the shift away from the affected side. Patients with atelectasis, pleural fibrosis, or who are status post-pneumonectomy would experience a decreased pressure within the pleural cavity on 1 side; therefore, the mediastinum would be shifted toward that affected side.

Pleural friction rub is not the correct answer. A pleural friction rub is heard on cardiac auscultation and is the result of the pleural linings rubbing together. In cases when the pleural linings are inflamed (such as in pneumonia, pleural effusion, or pulmonary embolism), a pleural friction rub can be heard. The finding is nonspecific in terms of the amount of fluid or differentiation between sides; therefore, it would not necessarily be indicative of these particular thoracentesis findings.

Bilateral inaudible breath sounds is not the correct answer. Due to the increased fluid and pressure associated with pleural effusion, the breath sounds may not be heard during physical examination. However, not hearing the sounds on either side does not give any indication as to how severe the pleural effusion is (although this typically happens in the larger effusions) and certainly does not show the differentiation between sides.
Hepatojugular reflux
This patient is presenting with a progression of a primary pulmonary disease (pulmonary hypertension) into cor pulmonale, which is also known as pulmonary heart disease. Cor pulmonale, when moderate to severe, will present with signs and symptoms of right heart failure, such as hepatojugular reflux, peripheral edema, prominent P2, hepatomegaly, cyanosis, wheezing, and ascites. The ECG findings indicate right ventricular hypertrophy, which is a complication of progressing primary lung disease.

Depending on the location in which the bruit was auscultated, an abdominal bruit might indicate atherosclerosis in the renal arteries, aorta, or iliac arteries. Atherosclerosis is typically seen in much older patients. This patient's pulmonary hypertension is not directly associated with atherosclerosis, and bruits would not be expected with her physical exam.

Absent breath sounds are a finding associated with several pulmonary conditions, such as pneumothorax or a severe pneumonia; they are also seen in patients with a history of pneumonectomy. A pneumothorax or pneumonia might have some aspects in common with this patient's history (such as dyspnea), but they would not be associated with the fluid retention and ECG changes.

Dry mucus membranes and reduced skin turgor are indicators of severe dehydration. Nothing about this patient's history is consistent with dehydration. On the contrary, this patient is in a fluid-overload state.

Virchow's node enlargement indicates left-sided supraclavicular lymphadenopathy. This finding is a harbinger for malignancy of the thorax. The patient's symptoms do not suggest malignancy. If malignancy was present, depending on the primary type of cancer, the patient might exhibit unexplained weight loss and possibly night sweats.
3 He 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 and when placed correctly it should produce a wheal of 6 - 10 mm in diameter. The skin test results should be read 48 - 72 hours after administration of the PPD.

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

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 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 vaccine.

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

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

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

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

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

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

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

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

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

Glucocorticoid therapy is recommended for symptomatic interstitial lung disease (ILD) patients with eosinophilic pneumonias, cryptogenic organizing pneumonia, connective tissue diseases, sarcoidosis, hypersensitivity pneumonitis, acute inorganic dust exposures, acute radiation pneumonitis, diffuse alveolar hemorrhage, and drug-induced ILD. In organic dust disease, glucocorticoids are recommended for both the acute and chronic stages.
Acute respiratory distress syndrome
Acute respiratory distress syndrome (ARDS) is an acute lung injury in which the ratio of PaO2/FIO2 is 200 or less and there is bilateral interstitial infiltrates with no evidence of raised left atrial pressure. Common etiologies are aspiration, sepsis, pancreatitis, toxic inhalation burns, multiple blood transfusions, drugs, and trauma.

It is characterized by dyspnea, tachypnea, rales, bilateral infiltrates, marked hypoxemia that is not corrected by O2, and leukocytosis. Multi-organ failure may also be present. The patient in question has all of these features, and his PaO2/FIO2 ratio is 100. Treatment of ARDS includes identifying and treating the underlying condition as well as cardiopulmonary support. No pharmacological agents have been shown to be beneficial. Adding positive end-expiratory pressuremay improve oxygenation by opening the fluid-filled alveolar units (alveolar recruitment). Other modalities that may improve oxygenation include prone positioning and inverse ratio ventilation.

Bacterial and viral pneumonia presents as cough with or without sputum production, fever, and leukocytosis. It does not present with this degree of shunt hypoxemia. It also does not lead to such rapid deterioration. Myocardial infarction may be precipitated by cocaine use. For it to have caused such infiltrates, however, there would have also been signs of right heart failure and hypotension; it would not have caused so much shunt hypoxemia. Pulmonary embolism can present acutely. It has to be massive to present this way, in which case the patient would be hypotensive, and CXR would not show bilateral infiltrate.

Increasing the tidal volume or respiratory rate will not improve his oxygenation since he has very high minute ventilation. An emergent thoracentesis will not improve the patient's status.
The correct answer is phenoxybenzamine. The patient's young age suggests that she is suffering from a form of secondary hypertension. Given her paroxysmal headaches, diaphoresis, and palpitations accompanied by hypertension, she is likely suffering from a pheochromocytoma. Pheochromocytomas are benign tumors that arise from the adrenal glands. The tumors secrete norepinephrine, which leads to hypertension, tachycardia, headache, and diaphoresis. Diagnosis is made based on elevation in urinary and plasma metanephrines and catecholamines. and it is followed by tumor location on CT or MRI. The patient requires removal of the pheochromocytoma, but preoperative control of her blood pressure is necessary. Alpha blockers, such as phenoxybenzamine or prazosin, are the preferred antihypertensive agents in pheochromocytoma.

Lisinopril is an ACE inhibitor and would not be considered first-line treatment in the control of hypertension in pheochromocytoma.

Metoprolol is a beta blocker. Beta blockers may only be used in the treatment of pheochromocytoma after the patient has received an alpha blocker. If a patient with pheochromocytoma receives a beta blocker as first-line treatment, the patient will develop unopposed alpha agonism, causing worsened hypertension.

Lasix is a diuretic and is not recommended as first-line treatment in the control of hypertension in pheochromocytoma.

Hypertension should not be treated at this time is an incorrect answer; the patient's blood pressure is severely elevated and she requires control of her blood pressure prior to the removal of her pheochromocytoma.
Adenocarcinoma is the correct answer. Adenocarcinoma accounts for about 40% of all lung cancers. While smoking is a strong risk factor, it is the most common lung malignancy in non-smokers. It is seen more commonly in women, and it starts typically at an earlier age. Formerly known as bronchoalveolar carcinoma, adenocarcinoma sometimes produces mucus or other such substances. The peripheral location is also typical, as compared to the more central squamous cell carcinoma.

Small cell carcinoma is incorrect. Also known as oat cell carcinoma, it is extremely unusual in non-smokers, and it accounts for about 10% to 15% of lung cancers. They are typically central in location, and they respond poorly to treatment.

Large cell carcinoma is incorrect. It accounts for about 10% to 15% of lung cancers. It can occur in any part of the lung, and it tends to grow and spread rapidly. Being more uncommon than adenocarcinoma, in this patient, the likelihood of it being a large cell tumor is lower.

Mesothelioma is incorrect. Mesothelioma is a pleural malignancy associated strongly with asbestos exposure. They typically present as pleural effusions with pleural plaques. Smoking is not strongly associated with mesothelioma.

Squamous cell carcinoma is incorrect. It accounts for about 25 to 30% of all cases of lung cancer, and it is strongly associated with smoking. Squamous cell carcinoma tends to be more central in location and is most commonly associated with para-neoplastic syndromes.
Reduced intravascular oncotic pressure
Pleural effusions, an abnormal collection of fluid in the pleural space, is related to an underlying pathology causing excess fluid production or decreased absorption. Effusions are classified as transudative or exudative depending on the characteristics of the fluid.

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

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

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

Reduced pressure in the pleural space prevents full lung expansion, allowing pleural fluid accumulation. Causes of this include atelectasis (transudative) or mesothelioma (exudative).
Cystic Fibrosis
ystic fibrosis is an inherited disease that affects the respiratory and digestive systems. It affects the exocrine (mucus and sweat) glands of the body and is caused by a defective gene. It is the most common cause of chronic lung disease in children and young adults. Thick mucus is formed in the bronchial tree, which predisposes the person to chronic lung infections. Symptoms include no meconium stool in the first 24 hours of life, stools (pale or clay colored, foul-smelling, and floating), skin may taste salty (infants), persistent respiratory infections, coughing or wheezing, weight loss, clubbing of the fingers or toes, diarrhea, delayed growth, easy fatigue, and splenomegaly. Tests should include sweat chloride test, a fecal fat test (positive), bone X-ray, chemistry panel, upper GI, small bowel series, as well as immunoreactive trypsinogen. Treatments include antibiotics for respiratory infections and pancreatic enzymes to replace the missing enzymes. Mucomyst may be used on occasion to thin secretions. The use of ibuprofen has been shown to slow lung deterioration in some children. Other treatments include postural drainage, chest percussion, and other breathing treatments. Lung transplant may be considered in some cases. About 50% of patients with cystic fibrosis live beyond age 20. Few patients live beyond 35. Death occurs from pulmonary (lung) complications.

Pulmonary tuberculosis is a contagious bacterial infection caused by Mycobacterium tuberculosis (TB). The lungs are primarily involved, but the infection can spread to other organs. The disease is characterized by the development of granulomas (granular tumors) in the infected tissues. Primary infection is usually asymptomatic. Symptoms may include initially but are not limited to minor cough and mild fever, fatigue, weight loss, coughing up blood, slight fever and night sweats, wheezing, rales, excessive sweating, joint pain, hearing loss, diarrhea, chest pain, breathing difficulty, positive Babinski's reflex, and clubbing of the fingers or toes.

Pulmonary histiocytosis is characterized by inflammation of the small airways (bronchioles) and the small blood vessels in the lungs. This inflammation leads to stiffening (fibrosis) and destruction of the walls of the alveoli. Systemic involvement may cause rashes, pulmonary problems, enlargement of the spleen and liver, anemia, and death. In children, symptoms can include failure to thrive, weight loss, irritability, fever, seborrheic dermatitis of the scalp, generalized rash (petechiae or purpura), and chronically draining ears; bone pain may or may not be present.

Pulmonary aspergilloma is a fungal mass that grows in pre-existing lung cavities (or can cause new lung cavities). The preexisting cavities may have been caused by a previous infection of histoplasmosis, tuberculosis, sarcoidosis, lung abscess, cystic fibrosis, or previous lung cancer. Symptoms include cough, coughing up blood, weight loss, and fever. Tests include bronchoscopy or bronchoscopy with lavage (BAL), chest X-ray, and sputum culture. The most effective therapy is a surgical resection (cutting out the fungus growth). If life-threatening bleeding occurs, emergency surgery may be the only choice of treatment.

Pulmonary nocardiosis is an infection of the lung (pneumonia that is caused by a fungus-like bacterium). The causative organism is present throughout the world, and the infection is acquired through inhalation, causing a pneumonia-like illness. The disease can spread to any part of the body, but brain lesions and subcutaneous (under the skin) lesions are most common. Symptoms include cough with sputum production, progressive difficulty breathing, general discomfort, uneasiness or ill feeling (malaise), weight loss, fever (intermittent), night sweats, chest and joint pain, as well as liver and spleen enlargement (hepatosplenomegaly).
Pseudomonas aeruginosa is a Gram-negative, oxidase-positive rod that does not ferment glucose, with a typical metallic sheen of the growth on TSI agar, coupled with the blue-green pigment on ordinary nutrient agar and a fruity aroma. P. aeruginosa produces 2 pigments: (1) pyocyanin which can color the pus in a wound blue, and (2) pyoverdin (fluorescein), a yellow-green pigment that fluoresces under ultraviolet light. P. aeruginosa is the only species of Pseudomonads that synthesizes pyocyanin. Pseudomonads are able to grow in water containing only traces of nutrients, and they have a remarkable ability to withstand disinfectants. Strains of P. aeruginosa isolated from cystic fibrosis patients have a prominent slime layer (glycocalyx), which gives their colonies a very mucoid appearance. P. aeruginosa is primarily an opportunistic pathogen that causes infections in hospitalized patients, those with chronic respiratory disease in whom the normal clearance mechanisms are impaired, immunosuppressed patients with neutrophil counts of less than 500/μL, and patients with indwelling catheters. P. aeruginosa can cause infections virtually anywhere in the body, but urinary tract infections, pneumonia (especially in cystic fibrosis patients) and wound infections (especially burns) predominate.

Haemophilus influenzae is a small Gram-negative rod with a polysaccharide capsule. It is the leading cause of meningitis in young children, and it is an important cause of upper respiratory tract infections and sepsis in children; also, it causes pneumonia in adults, particularly those with chronic obstructive lung disease.

Bordetella pertussis is a small, coccobacillary, encapsulated Gram-negative rod. It causes whooping cough (pertussis).

Legionella pneumophila is Gram-negative rod that stain faintly with the standard Gram stain. Sputum Gram stains reveal many neutrophils, but no bacteria. The organism fails to grow on ordinary media in a culture of blood or sputum, but it will grow on medium supplemented with iron and cysteine. L. pneumophila causes atypical pneumonia, both in the community and in hospitalized immunocompromised patients.

Streptococcus pneumoniae causes pneumonia, bacteremia, meningitis and infections of the upper respiratory tract. Pneumococci are Gram-positive, lancet-shaped cocci arranged in pairs or short chains.
pulmonary htn
The patient above is most likely suffering from restrictive lung diseasedue to the lupus, and specifically signs and symptoms of pulmonary hypertension. Pulmonary hypertension is a lung disorder the pulmonary arteries become narrowed. As a result, the blood pressure in these arteries rises above normal levels. The high pressure strains the right ventricle of the heart, causing it to expand in size. Overworked and enlarged, the right ventricle gradually becomes weaker and loses its ability to pump enough blood to the lungs. This can lead to the development of right heart failure.

Symptoms of pulmonary hypertension do not usually occur until the condition has progressed. The initial symptom is usually shortness of breath with everyday activities, such as climbing stairs. Fatigue, dizziness, and fainting spells also can be symptoms. Edema in the ankles, abdomen, or legs may be present; bluish lips may also be present. Chest pain may occur as strain on the heart increases.

Causes of pulmonary hypertension may include, but are not limited, to dexfenfluramine and phentermine (fen-phen has been taken off the market, but former fen-phen users have a 23-fold increase risk of developing pulmonary hypertension, possibly years later), liver diseases, rheumatic disorders (which include systemic lupus erythematosus), other lung conditions, heart diseases, and low-oxygen conditions; it may be idiopathic in origin.

Congestive heart failure is usually a disease state that initially affects the left side of the heart, causing signs and symptoms relating to that.

Pulmonary embolism is incorrect; it usually presents with dyspnea and pain on exertion, possible tachypnea, and (potentially) even hemoptysis or syncope. It is also usually seen in a more acute presentation scenario.

Pericarditis is incorrect, although may be extremely hard to differentiate from pathologies that cause right-sided heart failure. The cause of pericarditis is a major difference; etiologies include tuberculosis, radiation therapy, cardiac surgery, viral pericarditis, or histoplasmosis.

Dilated cardiomyopathy is incorrect; this condition will also occur in the left portion of the heart initially.Causes include (but are not limited to) ETOH abuse, catecholamine excess, and myocarditis; they can also be idiopathic.
Inhaled surfactant replacement
Respiratory distress syndrome (RDS) is also known as hyaline membrane disease (HMD) and is a condition that causes infants to need extra oxygen and assistance in breathing. It is one of the most common problems seen in premature infants; the more premature the baby, the higher the risk and the more severe the HMD. Many times HMD typically worsens over the first 48 to 72 hours after birth and then improves with treatment; more than 90 percent of babies with HMD survive.

HMD occurs when there is not enough surfactant in the newborn's lungs. Surfactant is made by the cells in the airways and consists of phospholipids and protein. It begins to be produced in the fetus at about 24 to 28 weeks of pregnancy and is found in amniotic fluid between 28 and 32 weeks. By about 35 week's gestation, most babies have developed adequate amounts of surfactant. In healthy lungs, surfactant is released into the lung tissues to help lower surface tension in the airways and this helps keep the lung alveoli open. When there is not enough surfactant, the tiny alveoli collapse with each breath. As the alveoli collapse, damaged cells collect in the airways and this makes it even harder to breath. The newborn works harder and harder to breath with each breath, trying to re-inflate the collapsed airways. This vicious cycle can eventually lead to a build-up of carbon dioxide, eventual acidosis, and/or eventual physical exhaustion from trying to attempt to take breaths.

The most common symptoms of HMD include difficulty breathing at birth that gets progressively worse, cyanosis, flaring of the nostrils, tachypnea, grunting sounds, and chest retractions. Chest radiographs will reveal a characteristic "ground glass" appearance".

Treatment of HMD consists of placing an endotracheal tube, supplemental oxygen, continuous positive airway pressure (CPAP), and inhaled surfactant replacement with artificial surfactant. This treatment has been shown to reduce the severity of HMD and is most effective if started within the first 6 hours of birth. Artificial surfactant comes as a powder that is mixed with sterile water and given through the ET tube.

RDS stemming from HMD is not a viral or bacterial infection; therefore, intravenous antivirals or antibiotics would be an inappropriate choice. This also is not specifically an issue with Hepatitis B infection, therefore, the Hepatitis B immunoglobulin answer is incorrect. Inhaled corticosteroids may give some minor symptomatic relief, but they are not an indicated or efficient treatment for patients with hyaline membrane disease or respiratory distress syndrome.
The clinical picture along with clubbing and the honeycomb appearance on chest X-ray is diagnostic of bronchiectasis.

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

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

Treatment is by antibiotics and assisting postural drainage.

Cystic fibrosis presents with associated symptoms of malabsorption and infertility.

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

Pneumonia will have an acute history, and recurring infections are not characteristic. X-ray will be diagnostic. Tuberculosis will also be diagnosed on the X-ray. Bronchiectasis may develop secondary to a tuberculous hilar lymph node obstructing a major bronchus.
Pulmonary embolism
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 of greater than 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 2nd 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 (3rd and 4th 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, 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.
squamous cell
Squamous cell carcinoma is the most likely diagnosis in this patient with signs and symptoms of hypercalcemia; it is a notorious paraneoplastic syndrome in pulmonary squamous cell carcinoma due to the secretion of parathyroid hormone related peptide (PTHRP). The patient also has a strong history of cigarette smoking, clubbing on physical examination, and a centrally-located cavitary lung lesion that resembles a pulmonary abscess; all findings suggest squamous cell carcinoma.

This question combines clinical features of both hyperparathroidism and squamous cell carcinoma. Hyperparathyroidism is a syndrome of hypercalcemia resulting from excessive release of parathyroid hormone and in most cases is due to adenoma in a single parathyroid gland. Parathyroid gland hypertrophy accounts for the remaining cases of hyperparathyroidism, and paraneoplastic syndromes such as those associated with squamous cell carcinoma are indeed rare. The symptoms of hyperparathyroidism are simple if you remember the mnemonic: "Painful bones (and tenderness), renal stones (nephrolithiasis), abdominal groans (abdominal pain), and psychic moans (changes in mental status)." This patient has abdominal pain, bone pain, a history of nephrolithiasis, and a recent change in mental states (new onset anxiety and depression). Combine these features with a high serum calcium, low serum phosphate, and high urinary cAMP (PTH exerts its effects though the second messenger cyclic AMP (cAMP)), and the diagnosis of hyperparathyroidism is assured.

Bronchoalveolar cell carcinoma is a subtype of adenocarcinoma, and although an association with smoking has not been established, a substantial percentage of patients have a significant smoking history. The incidence of bronchoalveolar cell carcinoma is increased in patients who have underlying interstitial lung disease, parenchymal scaring, and exogenous lipoid pneumonia. Bronchoalveolar carcinoma may appear as a solitary pulmonary nodule, multiple nodules, or consolidation.

Large cell pulmonary carcinomas account for only a small percentage of bronchogenic carcinomas and are also associated with cigarette smoking. The lesion occurs peripherally and grows rapidly, with early metastases and a poor outcome.

Small cell carcinomas also arise centrally in peribronchial locations and infiltrate the bronchial submucosa. Small cell carcinoma of the lung (Oat Cell Carcinoma) is strongly associated with cigarette smoking. Widespread metastases occur early in the course of the disease, with common spread to mediastinal lymph nodes, liver, bones, adrenal glands, and brain. Also, several paraneoplastic syndromes are associated with oat cell carcinoma secondary to oncogenic cell production of peptide hormones. The most common paraneoplastic syndromes are the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and the syndrome of ectopic adrenocorticotropic hormone (ACTH) production, which can lead to Cushing Disease.
erythrogenic toxin
Erythrogenic toxin (streptococcal pyrogenic exotoxin) is produced by Streptococcus pyogenes. The toxin is responsible for the rash of "scarlet fever". The toxin has been shown to exhibit pyrogenicity and cytotoxicity. It usually appears at the 2nd day of infection, on the upper part of the chest, spreading to the rest of the trunk out towards the rest of the body, with the palms, soles, and face being spared.

Exfoliative toxin, produced by Staphylococcus aureus, is responsible for scalded skin syndrome (SSS), wherein the patient appears to have acquired a burn of the skin. There is extensive scalding and flaking desquamation of the epidermis. The syndrome is especially common in infants and small children.

Elastase is an extracellular protease that is produced by Pseudomonas aeruginosa. The protease is associated with the organism's virulence due to tissue destruction and bacterial invasion. Elastase is necrotizing to the skin, cornea, and lung; it is capable of producing hemorrhage.

Enterotoxin is produced by various bacteria. In Clostridium perfringens, the production of this toxin causes the symptoms of food poisoning. Clostridium perfringens type A is most associated with this toxin production. Enterotoxin is thought to act as a superantigen, which causes a massive release of inflammatory mediators and induces a calcium ion-dependent breakdown of permeability.

Exotoxin (diphtheria toxin) is produced by Corynebacterium diphtheriae. The toxin inhibits protein synthesis in mammalian cells, but not bacteria. It affects all cells in the body; the heart, nerves, and kidney are impacted the most.
High resolution CT
This patient has a likely diagnosis of idiopathic pulmonary fibrosis (IPF). This is a relatively rare disease and can be difficult to diagnose. Patients typically have symptoms for 1 - 2 years prior to definitive diagnosis. IPF is a chronic, progressive restrictive pulmonary disease of the lung parenchyma. Unlike pneumoconiosis, there are no known occupational exposures (hence the term idiopathic). IPF presents with exertional dyspnea and a non-productive cough. A high-resolution computed tomography (CT) and/or a lung biopsy are the means for definitive diagnosis. The CT may show reticular opacities, honeycombing, and perhaps a 'ground glass' appearance in the lung tissue. There are heterogeneous areas of diseased lung interspersed with healthy tissue.

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

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

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

A ventilation-perfusion scan is a test that is primarily used in the diagnosis of pulmonary embolism (PE). This patient is not presenting with a history consistent with a PE (which may include acute dyspnea and chest pain).
supp O2 and supportive treatment
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.
idiopathic pulmonary fibrosis
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.