Mksap Pulmonary

 MKSAP 15 Pulmonary Pulmonary and an d Critical and Care Medicine Questions  Answers & Critiques CD1

Question 1  A 28-year-old 28-year-old man is evaluated for a 6-month history of episodic episodic dyspnea, cough, and and wheezing.  As a child, he had asthma and allergies, but he has been been asymptomatic since his early teenage years. His recent symptoms started after an upper respiratory tract infection, and they are often triggered by eercise or eposure to cold a ir. He is also awa!ened with asthma symptoms " or 6 nights a month. He is otherwise healthy and ta!es no medications. #n physical eamination, vital signs are normal. $here is scattered wheezing in both lung fields. %hest radiograph is normal. &pirometry shows an '() * of + of predicted with a *" improvement after inhaled albuterol. hich of the following is the most appropriate therapy for this patient/ 0A-Azithromycin 0B-Inhaled albuterol as needed 0C-Inhaled low-dose corticosteroids plus inhaled albuterol as needed 0D-Long-acting β-agonist 0E-Long-acting β-agonist plus inhaled albuterol as needed

Question 1  A 28-year-old 28-year-old man is evaluated for a 6-month history of episodic episodic dyspnea, cough, and and wheezing.  As a child, he had asthma and allergies, but he has been been asymptomatic since his early teenage years. His recent symptoms started after an upper respiratory tract infection, and they are often triggered by eercise or eposure to cold a ir. He is also awa!ened with asthma symptoms " or 6 nights a month. He is otherwise healthy and ta!es no medications. #n physical eamination, vital signs are normal. $here is scattered wheezing in both lung fields. %hest radiograph is normal. &pirometry shows an '() * of + of predicted with a *" improvement after inhaled albuterol. hich of the following is the most appropriate therapy for this patient/ 0A-Azithromycin 0B-Inhaled albuterol as needed 0C-Inhaled low-dose corticosteroids plus inhaled albuterol as needed 0D-Long-acting β-agonist 0E-Long-acting β-agonist plus inhaled albuterol as needed

 Answer and Critique Critique 1 (Correct Answer: C) (ducational #b1ective $reat persistent asthma. Key oint oint 03nhaled corticosteroids are the cornerstone of therapy for persistent asthma. 0 Asthma  Asthma symptom s on 2 or more days a wee! 4or 2 or more nights a m onth5 is the defining characteris tic of persistent asthm a. 3nhaled corticosteroids are the cornerstone of therapy for persistent asthma. egular use of inhaled corticosteroids is associated with improved pulmonary function, reduced airway hyperresponsiveness, decreased asthma eacerbations, and reduced mortality. &ide effects of inhaled corticosteroids include oral candidiasis and dysphonia related to laryngeal muscle myopathy. &ystemic effects may occur with use of inhaled corticosteroids and are generally related to the dose and duration of use. 3n adults, these effects include osteopenia, s!in thinning, and increased ris! for cataracts or glaucoma. $herefore, the lowest dose consistent with disease control should be used. $he treatment should be reevaluated every 7 to 6 months in stable patients, and ad1ustments made to step-up or step-down therapy based on disease control and occurrence of eacerbations. 3n between visits, patients should use a written asthma management plan, devised by their physician, to guide potential changes to their treatment. 0 Albuterol  Albuterol should be used as needed in all patients with asthma, but by itself is not ade uate therapy because it does not affect the underlying airway inflammation. 9ong-acting :-agonists 4salmeterol and formoterol5 provide bronchodilation for up to *2 hours and are effective in preventing eercise-induced asthma. $hese drugs do not have a clinically significant anti-inflammatory effect; therefore, their use without concomitant administration of corticosteroids may mas! worsening of asthma control and lead to increased asthma-related complications, including the possibility of increased asthma-related deaths. $herefore, long-acting :agonists are not appropriate as monotherapy or in place of inhaled corticosteroids. $he use of antibiotics for atypical infections 486 mm Hg, the pulse rate is ?+>min, the respiration rate is 28>min, and the @min by face mas!, is ?. $here are dry crac!les at the lung bases etending half way up the chest bilaterally. %ardiac and abdominal eaminations are normal. ram stain of sputum is negative; culture is pending. %$ scan of the chest is negative for pulmonary embolism but shows new areas of alveolar infiltrates and consolidation superimposed on previous basilar, reticular, and honeycomb changes.

hich of the following is the most appropriate net test in the evaluation of this patient/ 0

A-Bronchoscopy with bronchoal!eolar la!age

0

B-"ungal serologies

0

C-#ight-heart catheterization

0

D-$wallowing e!aluation

 Answer and Critique 2 (Correct Answer:  A) Educational Objective: Evaluate an acute exacerbation of idiopathic pulmonary fibrosis. Key Point: 0@ronchoalveolar lavage is the diagnostic procedure to eclude opportunistic infection in an apparent acute eacerbation of idiopathic pulmonary fibrosis. 0$he two immediate diagnostic considerations in this patient are respiratory infection and an acute eacerbation of pulmonary fibrosis. @oth diagnostic possibilities may be evaluated by bronchoalveolar lavage with studies to detect bacterial organisms, opportunistic pathogens 4for eample, Pneumocystis jirovecii 5, and viral pathogens. outine sputum evaluation for ram stain and culture is not sensitive enough to detect opportunistic infectious organisms. Biagnostic criteria for an acute eacerbation of pulmonary fibrosis include eclusion of opportunistic respiratory infections via endotracheal aspiration or bronchoalveolar lavage as well as eclusion of pulmonary embolism, left ventricular failure, and other causes of acute lung in1ury. $he incidence of an acute eacerbation of idiopathic pulmonary fibrosis is not certain but li!ely ranges between " and =. 3n patients with pulmonary fibrosis admitted to the intensive care unit for respiratory failure, the incidence may be as high as 6 with a reported mortality rate between 8 and *. Co therapy has been shown to be beneficial. 0'ungal serologies may be helpful to diagnose opportunistic infection in this patient. However, bronchoalveolar lavage is more sensitive, and results more readily available for detecting other opportunistic pathogens that need to be ecluded in this immunosuppressed patient. ight-heart catheterization is not part of the initial evaluation of patients with a suspected acute eacerbation of idiopathic pulmonary fibrosis. Aspiration may cause acute lung in1ury and may trigger an acute eacerbation of idiopathic pulmonary fibrosis, but swallowing evaluation is unli!ely to be diagnostic in this patient with no history of aspiration.

Question 3 0

 A 6"-year-old woman is evaluated in a follow-up eamination for dyspnea, chronic cough, and mucoid sputum; she was diagnosed with chronic obstructive pulmonary disease 7 years ago. $he patient has a =-pac!-year history of cigarette smo!ing, but uit smo!ing * year ago. &he is otherwise healthy, and her only medication is inhaled albuterol as needed.

0

#n physical eamination, vital signs are normal. @reath sounds are decreased, but there is no edema or 1ugular venous distention. &pirometry shows an '() * of 62 of predicted and an '()*>')% ratio of 6". %hest radiograph shows mild hyperinflation.

hich of the following is the most appropriate therapy for this patient/ A-Add a long-acting β %-agonist B-Add an inhaled corticosteroid C-Add an oral corticosteroid D-Add theophylline and montelu&ast E-Continue current albuterol therapy

 Answer and Critique 3 (Correct Answer:  A) Educational Objective: Manage moderate chronic obstructive pulmonary disease. Key Point: 0 3n patients with moderate chronic obstructive pulmonary disease, therapy with a long-acting : 2-agonist or a long-acting anticholinergic agent improves uality of life and pulmonary function compared with therapy with short-acting bronchodilators alone. 0$his patient has stage 33 chronic obstructive pulmonary disease 4%#DB5 as defined by the guidelines of the lobal 3nitiative for #bstructive 9ung Bisease 4#9B5. #9B stage 33 disease is defined by a postbronchodilator '() *>')% ratio less than + and an '() * less than 8 but more than " of predicted with or without chronic symptoms. 3n patients with #9B stage 33 disease, maintenance treatment with one or more long-acting bronchodilators such as a long-acting : 2-agonist 4salmeterol or formoterol5 is r ecommended, along with as-needed albuterol. Dulmonary rehabilitation can be considered in addition to medical treatment in symptomatic patients. &tarting or adding a long-acting anticholinergic agent would also be appropriate. 03nhaled corticosteroids, oral corticosteroids, theophylline, and montelu!ast would be inappropriate f or this patient. $heophyllineEs narrow therapeutic window and poor bronchodilator effect ma!e it a poor choice. #ral corticosteroids are not recommended routinely in %#DB because of their systemic side effects. or renal dysfunction, neither of which this patient has. 0%hurg-&trauss syndrome is associated with asthm a and, in the vasculitic stage, can cause wea!ness. However, the syndrome most often manifests as mononeuritis multiple rather than generalized wea!ness. 'urthermore, this patient does not have other manifestations of vasculitis such as rash or renal dysfunction.

Question 6 0

 A "=-year-old man is evaluated in the emergency department f or a *-hour history of c hest pain with mild dyspnea. $he patient had been hospitalized * wee! ago for a colectomy for colon cancer. His medical history also includes hypertension and nephrotic syndrome secondary to membranous glomerulonephritis, and his medications are furosemide, ramipril, and pravastatin.

0

#n physical eamination the temperature is 7+." J% 4* J'5, the pulse rate is *2>min, the respiration rate is 2=>min, the blood pressure is **>6 mm Hg, and the @min. %ardiac eamination shows tachycardia and an &=. @reath sounds are normal. %hest radiograph is negative for infiltrates, widened mediastinum, and pneumothora. &erum creatinine concentration is 2.* mg>d9 4*8".6 Kmol>95. (mpiric unfractionated heparin therapy is begun.

hich of the following is the best test to confirm the diagnosis in this patient/

 A-Assay )or plasma D-dimer  B-C( angiography C-Lower e3tremity ultrasonography D-4easurement o) antithrombin III E-5entilation6per)usion scan

 Answer and Critique 6 (Correct Answer: E) Educational Objective: #onfirm the clinical diagnosis of acute pulmonary embolism. Key Point: 0(ither ventilation>perfusion scanning or contrast-enhanced %$ scanning 4if not contraindicated5 performed with a specific protocol to detect pulmonary embolism is an appropriate noninvasive test to diagnose acute pulmonary embolism. 0$his patient is at high ris! for pulmonary embolism because of his recent hospitalization, cancer, and nephrotic syndrome. A positive ventilation>perfusion scan would confirm the diagnosis of pulmonary embolism in this patient with a high pretest probability for the condition, especially in the absence of parenchymal lung defects on chest radiograph. 0$he probability of pulmonary embolism was very high based on this presentation that included chest pain, dyspnea, recent hospitalization and surgery, active cancer, and a protein-losing nephropathy. A negative B-dimer test would not be sufficient evidence to rule out a pulmonary embolism under these circumstances, and a high B -dimer level would add little to the diagnostic wor!-up. Becreased antithrombin 333 levels may result from nephrotic syndrome, and levels are lowered during acute thrombosis, especially during treatment with heparin. $herefore, measuring antithrombin 333 would add little to the accuracy of the diagnosis of pulmonary embolism or have any implication for immediate management decisions. 9ower etremity ultrasonography can disclose asymptomatic deep venous thrombosis in a small percentage of patients presenting with symptoms of pulmonary embolism. However, the yield is relatively low and ventilation>perfusion scanning would have a much higher degree of accuracy. %$ angiography is an acceptable modality to diagnose acute pulmonary embolism but reuires a significant amount of contrast infusion 4as much as a pulmonary angiogram5 which would be contraindicated in a patient with an elevated serum creatinine level.

Question 7  A An *8-year-old man is evaluated in the emergency department after his mother found him unconscious in his bed at home. &he reported that her son had gone to a party two nights ago, but she was not sure when he returned home. hen she chec!ed on him, he was unarousable. He has no significant medical history and ta!es no medications. 3n the emergency department, he is afebrile, bl ood pressure is **>+ mm Hg, the pulse rate is ">min, and respiration rate is 6>min; he is intubated for airway protection. Laboratory studies78emoglobin +%9% g6dL +%% g6L0 0Leu&ocyte count ':;;6 d9 4*+= mmol>95. $oicology testing is positive for opiates and cocaine. @ladder catheterization reveals only 7 m9 of brown urine.

hich of the f ollowing is the most li!ely cause of the patientEs renal failure/ 0A-8emolytic anemia 0B-8emolytic-uremic syndrome 0C-8epatorenal syndrome 0D-#habdomyolysis 0E-$epsis

 Answer and Critique 7 (Correct Answer: D) Educational Objective: Diagnose rhabdomyolysis secondary to narcotic overdose. Key Point: 0Contraumatic causes of rhabdomyolysis include drug use, metabolic disorders, and infections. 0$his patient most li!ely has rhabdomyolysis, which is caused by s!eletal muscle damage that leads to release of intracellular components into the circulation, such as creatine !inase and lactate dehydrogenase, the heme pigment myoglobin, purines, and potassium and phosphate. $he syndrome was first identified in patients with traumatic crush in1uries, but t here are nontraumatic causes, such as alcohol 4due to hypophosphatemia5, drug use, metabolic disorders, and infections. $he classic triad of findings includes muscle pain, wea!ness, and dar! urine. $he diagnosis is based on c linical findings and a history of predisposing factors 4such as prolonged immobilization or drug toicity5 and confirmed by the presence of myoglobinuria, an increased serum creatine !inase level, and, in some c ases, hyper!alemia. $he disorder usually resolves within days to wee!s. $reatment consists of aggressive fluid resuscitation; fluids should be ad1usted to maintain the hourly urine output at least 7 m9 until the urine is negative for myoglobin. Acute !idney in1ury resulting from acute tubular necrosis occurs in approimately one third of patients. Bialysis is sometimes necessary. 0 Although fulminant hepatic failure may result in coma, dar! urine, and renal failure, other tests of synthetic liver function in this patient are normal. $here are no clinical features to suggest sepsis. $he patient has mild anemia, but the proportionate reduction in the leu!ocyte and platelet counts suggests alcohol-induced bone marrow suppression. Hemolytic anemia would not eplain the patientEs elevated creatine !inase level and usually does not cause renal failure. Hemolytic uremic syndrome is not c onsistent with the clinical findings of polysubstance overdose or the laboratory finding of the elevated serum creatine !inase level.

Question 8  A +-year-old man is evaluated in the emergency department for a 2-day history of dyspnea with eertion, orthopnea, and paroysmal nocturnal dyspnea. He has ischemic heart disease with left ventricular dysfunction and had c oronary artery bypass graft surgery 6 wee!s ago. His medications include aspirin, nitroglycerin, metoprolol, lisinopril, and furosemide.#n physical eamination, the patient is sitting upright and breathing with difficulty; the temperature is 7+ J% 4?8.6 J'5, the blood pressure is *">8" mm Hg, the pulse rate is *">min and regular, and the respiration rate is 28>min. #ygen saturation is 8? on ambient air. $here are fine crac!les at the lung bases bilaterally, and breath sounds are diminished at the right b ase. $here is a regular tachycardia and an &7 at the ape. $here is no 1ugular venous distention or peripheral edema. Hemoglobin is *2." g>d9 4*2" g>95, and the leu!ocyte count is *,">K9 4*." M * ?>95. %hest radiograph shows cardiomegaly and small bilateral pleural effusions, greater on the right than the left. $horacentesis is performed, and pleural fluid analysis shows 0Cucleated cell count=">K9 with 7 neutrophils, + lymphocytes, * macrophages, *" mesothelial cells, and 2 eosinophils. 0Dleural fluid to serum total protein ratio 09actate dehydrogenase 49BH5

."=

*2" F>9

0Dleural fluid to upper limits of normal serum 9BH ratio 0lucose

8 mg>d9 4=.== mmol>95

0$otal protein

7.+ g>d9 47+ g>95

0Dh +.=" 0 Albumin

*." g>d9 4*" g>95

0%holesterol

7" mg>d9 4.? mmol>95

0$he serumpleural fluid albumin gradient is *.+.

hich of the following is the most li!ely diagnosis/ 0A- 8eart )ailure 0B- arapneumonic e))usion 0C- ost1cardiac inury syndrome 0D- ulmonary embolism

."2

 Answer and Critique 8 (Correct Answer:  A) Educational Objective: %ecogni&e the effect of diuretic therapy on the pleural fluid analysis in patients with heart failure. Key Point: 0Biuretic therapy for heart failure c an result in either a protein- or l actate dehydrogenasediscordant eudative pleural effusion and, rarely, a concordant eudate. 0$he patientEs pleural fluid analysis shows a protein discordant eudate 4an eudate by protein criterion only5 with a pleural fluid to serum total protein ratio of ."= and a pleural fluid l actate dehydrogenase 49BH5 to upper limits of normal serum 9BH ratio of ."2. Dleural fluid findings may have eudative characteristics in patients with heart failure who are receiving diuretics. A serumpleural fluid albumin gradient greater than *.2 suggests a transudate in cases where the pleural fluid to serum total protein ratio or pleural fluid to serum 9BH ratio and pleural fluid 9BH to upper limits of normal serum 9BH ratio suggest an eudate, but the clinical findings suggest a transudate. $he increased pleural fluid to serum total protein ratio is the result of a diuretic effect, with more efficient clearance of pleural liuid than pleural protein. 0Datients with postcardiac in1ury syndrome typically present 7 wee!s 4range 7 days to * year5 after coronary artery bypass graft surgery; they usually have pleuritic chest pain and typically dyspnea, pleural or pericardial friction rub, fever, left lower lobe infiltrates, leu!ocytosis, and an increased erythrocyte sedimentation rate. $his patientEs findings are not compatible with postcardiac in1ury syndrome. $he absence of chest pain would be highly unli!ely with a pulmonary embolisminduced pleural effusion. Co consolidation was detected on chest radiograph, ma!ing pneumonia unli!ely. 'urthermore, a parapneumonic effusion is typically a concordant eudate 4both protein and 9BH in the eudate range5 with a neutrophil predominance, and a low pleural fluid 9BH is typically not associated with an acute parapneumonic effusion.

Question 9  A 2-year-old woman is ev aluated in the emergency department for an acute episode of wheezing and dyspnea without cough or sputum production. &he has had previous freuent evaluations in emergency departments and urgent care centers for similar episodes. 3n between these episodes, findings on physical eamination and pulmonary function testing, including methacholine challenge, have been normal. &he is otherwise healthy and ta!es no medications. #n physical eamination, the patient has inspiratory and epiratory wheezing and is in moderate discomfort. $he temperature is 7+.* J% 4?8.8 J'5, pulse rate is *>min, and the respiration rate is 2=>min; oygen saturation on ambient air is ?6. After receiving albuterol and intravenous corticosteroids, she continues to wheeze and is in moderate respiratory distress. #ygen saturation on ambient air remains at ?6. %hest radiograph shows decreased lung volumes.

hich of the following is the most appropriate management for this patient/ 0A-Chest C( scan 0B-Intra!enous aminophylline 0C-Intra!enous azithromycin 0D-Intra!enous terbutaline 0E-Laryngoscopy

 Answer and Critique 9 (Correct Answer: E) Educational Objective: Evaluate vocal cord dysfunction. Key Point: 09aryngoscopy during an eacerbation of vocal cord dysfunction shows adduction of the vocal cords during inspiration. 0$his patient li!ely has vocal cord dysfunction 4)%B5. Datients with )%B can have throat or nec! discomfort, wheezing, stridor, and aniety. $he disorder can be difficult to differentiate from asthma; however, affected patients do not respond to the usual asthma therapy. Biagnosing )%B is made more difficult by the fact that many of these patients also have asthma. $he chest radiograph in this patient showed decreased lung volumes, which is in contrast to hyperinflation that would be epected in acute asthma. #ygen saturation is typically normal in patients with )%B. 09aryngoscopy, especially when done while the patient is symptomatic, can reveal characteristic adduction of the vocal cords during inspiration. Another test that helps ma!e the diagnosis is flow volume loops, in which the inspiratory and epiratory flow rates are recorded while a patient is as!ed to ta!e a deep breath and then to ehale. 3n patients with )%B, the inspiratory limb of the flow volume loop is NflattenedO owing to narrowing of the etrathoracic airway 4at the level of the vocal cords5 during inspiration. ecognition of )%B is essential to prevent lengthy courses of corticosteroids and to initiate therapies targeted at )%B, which include speech therapy, relaation techniues, and treating such underlying causes as aniety, postnasal drip, and gastroesophageal reflu disease. 03ntravenous aminophylline is not recommended for treating either acute asthma or )%B. $herapy with intravenous terbutaline or other :-agonists for asthma eacerbations is associated with an unacceptably high rate of side effects. Azithromycin is a reasonable choice for acute bronchitis, but there is little evidence that this patient has acute bronchitis, which would manifest with cough, sputum production, and fever. $he chest %$ scan can be used to eclude parenchymal lung disease or evaluate the possibility of a pulmonary embolism; however, these disorders are unli!ely in this patient with previous normal pulmonary eaminations and radiographs and ecellent oygenation, and chest %$ scan is unli!ely to yield useful information.

Question 10 0

 A +2-year-old woman is evaluated for fatigue and decreased eercise capacity. $he patient has severe chronic obstructive pulmonary disease, which was first diagnosed * years ago, and was hospitalized for her second eacerbation * month ago. &he is a former smo!er, having stopped smo!ing " years ago. &he has no other significant medical problems, and her medications are albuterol as needed, an inhaled corticosteroid, a long-acting bronchodilator, and oygen, 2 9>min by nasal cannula.

0

#n physical eamination, vital signs are normal. @reath sounds are decreased, and there is *L bilateral pitting edema. &pirometry done * month ago showed an '() * of 28 of predicted, and blood gases measured at that time 4on supplemental oygen5 showed pH +.=*, D%# 2 =7 mm Hg, and D# 2 6= mm Hg; B9%o is 7 of predicted. $here is no nocturnal oygen desaturation. %hest radiograph at this time shows hyperinflation. %$ scan of the chest shows homogeneous distribution of emphysema.

hich of the following would be the most appropriate management for this patient/ 0

A-ung transplantation

0

B-Lung !olume reduction surgery

0

C-,octurnal assisted !entilation

0

D-ulmonary rehabilitation

 Answer and Critique 10 (Correct  Answer: D) Educational Objective: Prescribe pulmonary rehabilitation for a patient with severe chronic obstructive pulmonary disease. Key Point: 0Dulmonary rehabilitation in patients with advanced lung disease can increase eercise capacity, decrease dyspnea, improve uality of life, and decrease health care utilization. 0$his patient who is on maimum medical treatment for chronic obstructive pulmonary disease 4%#DB5 and is still symptomatic would benefit from pulmonary rehabilitation. %omprehensive pulmonary rehabilitation includes patient education, eercise training, psychosocial support, and nutritional intervention as well as the evaluation for oygen supplementation. eferral should be considered for any patient with chronic respiratory disease who remains symptomatic or has decreased functional status despite otherwise optimal medical therapy. 0Dulmonary rehabilitation increases eercise capacity, reduces dyspnea, improves uality of life, and decreases health care utilization. eimbursement for pulmonary rehabilitation treatment remains an impediment to its widespread use. 0$he effect of lung volume reduction surgery is larger in patients with predominantly nonhomogeneous upper-lobe disease and limited eercise performance after rehabilitation. $he ideal candidate should have an '() *between 2 and 7" of predicted, the B9%# no lower than 2 of predicted, hyperinflation, and limited comorbidities. $here is no indication for nocturnal assisted ventilation because she does not have daytime hypercapnia and worsening oygen desaturation during sleep. 9ung transplantation should be considered in patients hospitalized with %#DB eacerbation complicated by hypercapnia 4D%#2 greater than " mm Hg5 and patients with '() * not eceeding 2 of predicted and either homogeneous disease on high-resolution %$ scan or B9%# less than 2 of predicted who are at high ris! of death after lung volume reduction surgery. 9ung transplantation is, therefore, not an option for this patient.

Question 11 0

 A +*-year-old woman is evaluated for a 7-wee! history of mild pain in the shoulders and thighs and wea!ness when rising from a seated position and getting out of bed. &he also has a new rash on her hands. (ight m onths ago she was evaluated for dyspnea and new interstitial infiltrates that resulted in a lung biopsy and a diagnosis of idiopathic nonspecific interstitial pneumonia. &he was treated with prednisone, 6 m g>d, for * month; the dose was then tapered to * mg>d. Her symptoms had been st able on that dose until her new complaints.

0

#n physical eamination, there are swelling and discoloration of the eyelids and an erythematous scaly rash over the etensor surfaces of interphalangeal 1oints of both hands. $here is symmetric wea!ness of the proimal hip fleors and shoulder girdle muscles; hand s trength is normal. 9aboratory studies show antinuclear antibodies positive at a titer of **28 4previously negative5, serum creatine !inase *2+ F>9, and erythrocyte sedimentation rate 6 mm>h; serum electrolytes and complete blood count are normal. %hest radiograph shows bilateral reticular and alveolar abnormalities in the lower- and mid-lung zones.

hich of the following is the most appropriate management for this patient/ 0

 A-Electromyography and muscle biopsy

0

B-#epeat lung biopsy

0

C-$&in biopsy

0

D-(aper prednisone dosage

 Answer and Critique 11 (Correct  Answer: A) Educational Objective: Diagnose and manage dermatomyositis presenting as interstitial lung disease. Key Point: 0Fp to 7 of patients with dermatomyositis and polymyositis present with single-organ involvement of the lungs indistinguishable from idiopathic interstitial lung disease. 0$his patientEs symmetric proimal muscle wea!ness and pain with an erythematous, scaly rash over the interphalangeal  1oints 4ottron sign5, coupled with a positive antinuclea r antibody titer and elevated creatine !ina se level, suggest dermatomyositis. (lectromyography and muscle biopsy will establish the diagnosis of inflammatory myopathy. 3nflammatory myopathy must be distinguished from corticosteroid-induced myopathy because treatment of dermatomyositis reuires increased corticosteroids, whereas corticosteroid-induced myopathy is treated with withdrawal of prednisone. %orticosteroidinduced myopathy is not associated with elevated antinuclear antibodies, creatine !inase, or erythrocyte sedimentation rate. Fp to 7 of patients with dermatomyositis and polymyositis present without muscle, s!in, or 1oint involvement and have single-organ involvement of the lungs indistinguishable from idiopathic interstitial lung disease. 3nterstitial lung disease associated with inflammatory myopathy often occurs in the contet of antisynthetase antibodies 4for eample, anti-Po-*5 and the antisynthetase syndrome 4acute onset, constitutional symptoms, aynaud phenomenon, NmechanicEs hands,O arthritis, and interstitial lung disease5. 0epeat lung biopsy is unli!ely to yield new diagnostic information in this patient with stable chest radiograph and no new respiratory symptoms. A s!in biopsy is li!ely to reveal non specific findings that will not h elp diagnose the muscle symptoms.

Question 12  A +=-year-old man is evaluated for a "-year history of gradually progressive dyspnea and dry cough without wheezing or hemoptysis. 'or the past 2 years he has had pain and occasional swelling in both !nees. He has not had fever or lost weight. He smo!ed one pac! of cigarettes a day from the age of *8 to 6 years. He wor!ed as an insulator for = years. Dhysical eamination shows no digital clubbing or cyanosis. Auscultation of the lungs reveals bilateral end-inspiratory crac!les. Dulmonary function testing shows 0$otal lung capacity 0esidual volume 0')% 0'()*

6+ of predicted +2 of predicted

6" of predicted +" of predicted

0'()*>')% ratio 8? 0B9%# "2 of predicted 0His chest radiograph is shown

hich of the following is the most li!ely diagnosis/ 0A-Asbestosis 0B-Idiopathic pulmonary )ibrosis 0C-#heumatoid interstitial lung disease 0D-ulmonary sarcoidosis

 Answer and Critique 12 (Correct  Answer: A) Educational Objective: Diagnose asbestosis. Key Point  0Dleural plaues are focal, often partially calcified, fibrous tissue collections on the parietal pleura and are a mar!er of asbestos eposure. 0$he diagnosis of asbestosis is based on a convincing history of asbestos eposure with an appropriately long latent period 4* to *" years5 and definite evidence of interstitial fibrosis without other li!ely causes. $his patient wor!ed as an insulator when asbestos eposure was still widespread and is at ris! for asbestos-related lung disease. $he most specific finding on chest radiograph is bilateral partially calcified pleural plaues. Dleural plaues are focal, often partially calcified, fibrous tissue collections on the parietal pleura and are considered a mar!er of asbestos eposure. 0heumatoid lung disease has many manifestations, including an interstitial lung disease, which is most common in patients with severe rheumatoid arthritis. $his patientEs occasional swelling in both !nees is not compatible with the diagnosis of rheumatoid arthritis. 0&arcoidosis occurs most commonly in young and middle-aged adults, with a pea! incidence in the third decade. 8" mm Hg, and the @min, and the respiration rate is 22>min. $he lungs are clear, heart sounds are normal, and there is no evidence of bleeding on pelvic eamination. %omplete blood count on admission revealed a h ematocrit of 7= and a platelet count of *",>K9 4*" M *?>95. %hest radiograph is normal. )e )entilation>pe ntilation>perfusion rfusion scan shows mismatched perfusion defects in 2 of her lung volume.

hich of the following would be an acceptable therapy for this patient/ 0A-n)erior !ena ca!a )ilter  0@@-Intra!enous Intra!enous argatroban 0C-Intra!enous low-molecular-weight heparin 0D-$ubcutaneous un)ractionated heparin

 Answer and C Critique ritique 21 (Correct  Answer: D) Educational Objective: 'reat stable acute pulmonary embolism. Key Point  0 Acute  Acute pulm onary embolism can be treated initially with subcutaneous unfrac tionated heparin, low-m olecular-weight heparins, or fondaparinu without the need for dosage ad1ustment. 0$his patient has had an acute pulmonary embolism * day post partum. $he patient has no evidence of active bleeding, and there is no increased ris! for bleeding from anticoagulation. &ubcutaneous administration of unfractionated heparin, low-molecular-weight heparins, and fondaparinu are all safe and effective for the treatment of acute pulmonary embolism. A recent clinical trial showed that high-dose subcutaneous unfractionated heparin, administered without dose ad1ustment guided by the activated partial thromboplastin time, was as safe and effective as low-molecular-weight heparin administered in the same fashion. 03ntravenous argatroban, a direct thrombin inhibitor, might be useful in the setting of heparin-induced thrombocytopenia. However, the patientEs platelet count is normal. 88 mm Hg, pulse rate is ?>min, and respiration rate is 2>min. # ygen saturation with the patient at rest and breathing am bient air is 86. Pugular venous distention and a loud D 2 are present. $he chest is hyperinflated and breath sounds are dim inished. $here is *L pedal edema. Hemoglobin concentration is *6." g>d9 4*6" g>95. Arterial blood gases show pH +.7", D%# 2 "" mm Hg, and D# 2 "" mm Hg on ambient air. &pirometry shows an '() * of 2" of predicted. %hest radiograph shows hyperinflation but no infiltrates.

hich of the following is the most appropriate therapy for this patient/ 0A-Continuous o3ygen 0B-,octurnal o3ygen 0C-3ygen as needed 0D-3ygen during e3ercise

 Answer and Critique 22 (Correct  Answer: A) Educational Objective:%ecogni&e indications for continuous oxygen therapy in patients with chronic obstructive pulmonary disease. Key Point  03n a patient with severe chronic obstructive pulmonary disease, at-rest oygen saturation less than or eual to 88 is an indication for long-term continuous oygen therapy. 0$he long-term administration of oygen for more than *" hours per day to patients with chronic obstructive pulmonary disease 4%#DB5 increases survival, and may also improve hemodynamics, hematologic characteristics, eercise capacity, lung mechanics, and mental status. 3ndications for continuous long-term oygen therapy for patients with %#DB include 0D#2 less than or eual to "" mm Hg or oygen saturation less than or eual to 88 0Do2 less than or eual to "? mm Hg or oygen saturation less than or eual to 8? if there is evidence of cor pulmonale, right heart failure, or erythrocytosis 4hematocrit greater than ""5. 0$his patientEs resting oygen saturation is 86 and his D# 2 is "" mm Hg, and, therefore, continuous long-term oygen therapy is indicated. 0%hronic hypoemia leading to the development of cor pulmonale portends a poor prognosis. Cocturnal oygen therapy is better than no oygen therapy at all, but continuous therapy is better than nocturnal therapy in severely hypoemic patients with erythrocytosis, elevated pulmonary artery pressures, and respiratory acidosis. Co study has shown a survival benefit when oygen is prescribed for eercise-induced oygen desaturation or when used as needed for symptoms of breathlessness.

Question 23  A 27-year-old man see!s medical advice for an upcoming mountain epedition. A year earlier, a planned ="-day tre! to 9hotse in Cepal 4elevation 8"*6 m Q2+,?= ftR5 was cut short when he developed severe dyspnea and cough productive of blood-tinged, frothy sputum shortly after leaving the base camp 4elevation =?7 m Q*6,*+= ftR5. hen his symptoms persisted despite oygen therapy, he was aided down the mountain. He plans to return to the high Himalayas for another attempt to climb the 9hotse summit.

hich of the following would be appropriate prophylais for this patient/ 0A-Acetazolamide 0B-De3amethasone 0C-8ydrochlorothiazide 0D-4etoprolol 0E-,i)edipine

 Answer and Critique 23 (Correct  Answer: E) Educational Objective: %ecogni&e prophylaxis for high-altitude pulmonary edema. Key Point  0Cifedipine is used both to prevent and to treat high-altitude pulmonary edema. 0@oth the occurrence and severity of respiratory symptoms at high altitude are affected by the degree of elevation, rapidity of ascent, altitude during sleep, comorbid cardiovascular and respiratory disorders, physical eertion at altitude, and individual variations in tolerance to altitude 4for eample, altitude illness is more common in those with inadeuate hypoic ventilatory drive, prior history of altitude illness, and residence below an altitude of ?*" m Q7 ftR5. 0High-altitude pulmonary edema 4HAD(5 is a form of noncardiogenic pulmonary edema due to lea!age of fluid and hemorrhage into the alveolar spaces. $he most effective preventive measure for HAD( is an appropriately gradual ascent to altitude 4not greater than 7 to " m Q?8= to *6= ftR daily above an altitude of 2 m Q6"62 ftR, with scheduled rest days every 7 or = days5. Cifedipine is used to prevent and to treat HAD(. 0 Acetazolamide is used as prophylais for periodic breathing related to high altitude and acute m ountain sic!ness 4A95, cholesterol 2 mg>d9 4".2 mmol>95, and triglycerides * mg>d9 4*.*7 mmol>95. $horacentesis yields " m9 of pleural fluid, and analysis shows 0%ell count (rythrocytes 7>K9; leu!ocytes 8?>K9 4.8? M *?>95 with 6" lymphocytes, 22 neutrophils, 8 mesothelial cells, and = eosinophils 0$otal protein

7." g>d9 47" g>95

09actate dehydrogenase 2" F>9 0Dh +." 0 Amylase 2" F>9 0$riglycerides

*=" mg>d9 4*.6 mmol>95

0%holesterol

78 mg>d9 4.?8 mmol>95

0%ytology, ram stain, acid-fast bacilli stain, and bacterial culture are negative.

hich of the following is the most li!ely diagnosis/ 0A-Chylothora3 0B-Lymphomatous pleural e))usion 0C-arapneumonic e))usion 0D-(uberculous pleural e))usion

 Answer and Critique 24 (Correct  Answer: A) Educational Objective:Diagnose chylothorax. Key Point  0$he most common causes of chylothora are cancer and trauma; other causes are pulmonary tuberculosis, chronic mediastinal infections, sarcoidosis, lymphangioleiomyomatosis, and radiation fibrosis. 0%hylothora is drainage of lymphatic fluid into the pleural space secondary to disruption or bloc!age of the thoracic duct or one of its lymphatic tributaries. d9 4*.2= mmol>95 and occurs in association with a low pleural fluid cholesterol concentration. 3f the triglyceride level is less than " mg>d9 4."6 mmol>95, chylothora is unli!ely. hen the pleural fluid triglyceride concentration is between " and ** mg>d9 4."6 and *.2= mmol>95, a lipoprotein analysis should be done and the presence of chylomicrons would confirm the diagnosis in such cases. 0%hylothora can also occur in association with pulmonary tuberculosis and chronic mediastinal infections, sarcoidosis, lymphangioleiomyomatosis, and radiation fibrosis. 0 A lymphomatous pleural eff usion is always a consideration in p atients with a history of lymph oma; however, a lymphomat ous pleural effusion typically has an elevated lactate dehydrogenase level 4often greater than * F>95. Darapneumonic effusion is usually associated with a neutrophilic pleocytosis. Datients with tuberculous pleural effusion usually present with a nonproductive cough, chest pain, and fever. %hest radiograph usually shows a small to moderate effusion.

Question 25  A "6-year-old woman is evaluated for a 2-month history of a drooping eyelid, difficulty chewing food and swallowing, and slurred speech. $he symptoms are worse when she is tired. $he patient has a *"-pac!-year history of cigarette smo!ing but uit smo!ing * years ago. &he is otherwise healthy and ta!es no medications. #n physical eamination, the temperature is 7+. J% 4?8.6 J'5, the blood pressure is **8>6 mm Hg, the pulse rate is +2>min, the respiration rate is *6>min, and the @min. $here is significant rigidity of all his etremities.

hich of the following is the most appropriate therapy for this patient/ 0A-Alcohol sponge baths 0B-Ampicillin-sulbactam 0C-Corticosteroids 0D-Dantrolene 0E-$odium nitroprusside

 Answer and Critique 26 (Correct  Answer: D) Educational Objective:%ecogni&e and treat malignant hyperthermia. Key Point  0!g every " to * minutes until the symptoms resolve. esponse to dantrolene is not diagnostic of the disorder but is supportive if signs and symptoms resolve uic!ly. 'or those patients with a !nown history, pretreatment with dantrolene before the anesthetic agent is administered prevents the dev elopment of symptoms. 0 Alcohol sponge baths are generally not recommended as an augmentation of evaporative cooling in any hyperthermic patient, including malignant hyperthermia, owing to the possibility of substantial alcohol absorption through the s!in. 'urthermore, augmented coo ling 4typically accomplished with water misting and forced air circulation by fans5 may result in shivering which can increase body temperature unless it is s uppressed with benzodiazepine administration. Ampicillin-sulbactam might be a consideration if acute ascending cho langitis were suspected; however, this is unli!ely only hours after an elective cholecystectomy. 'urthermore, an infection cannot account for the patientEs muscular rigidity. %orticosteroids would be effective treatment f or an allergic reaction, but there are no symptoms suggesting an allergic reaction such as rash, urticaria, angioedema, or wheezing. &odium nitroprusside is indicated in patients with hypertensive emergencies. However, this patientEs blood pressure is elevated secondary to malignant hyperthermia, and treatment of the underlying disorder is the preferred therapy.

Question 27  A "6-year-old woman is evaluated for a 2-year history of episodic cough and chest tightness. Her symptoms began after a severe respiratory tract infection. &ince then, she has had cough and chest discomfort after similar infections, typically lasting several wee!s before resolving. &he feels well between episodes. &he is otherwise healthy and t a!es no medications. Dhysical eamination reveals no abnormalities, and spirometry is normal.

hich of the following is the most appropriate net step in the evaluation of this patient/ 0A-Bronchoscopy 0@-C( scan o) the sinuses 0C-E3ercise echocardiography 0D-4ethacholine challenge testing

 Answer and Critique 27 (Correct  Answer: D) Educational Objective:%ecogni&e indications for methacholine challenge testing in cases of suspected asthma. Key Point  0m9 suggests some bronchial hyperreactivity and is less specific for asthma. A D% 2 above *6 mg>m9 is considered normal. $he sensitivity of a positive methacholine challenge test in asthma is in the range of 8" to ?". 'alse-positive results can occur in patients with allergic rhinitis, chronic obstructive pulmonary disease, heart failure, cystic fibrosis, or bronchitis. 0@ronchoscopy to evaluate the trachea could be helpful if an anatomic lesion is suspected. However, the symptoms in patients with such lesions are persistent or progressive rather than intermittent. &ince this patient has intermittent symptoms, bronchoscopy is not indicated. (ercise echocardiography could help determine the presence of cardiac ischemia or myocardial dysfunction, the typical symptoms of which are dyspnea on eertion, chest tightness, or pain. %ough and wheezing can occur in coronary artery disease, particularly when associated with acute decompensation of the left ventricle, but this patientEs intermittent episodes of cough and wheezing are provo!ed by an upper respiratory tract infection, ma!ing the diagnosis of coronary artery disease unli!ely. Datients with rhinosinusitis have symptoms consisting of nasal congestion, purulent nasal secretions, sinus tenderness, and facial pain. adiography, including sinus %$ scan, is not indicated in the initial evaluation of acute sinusitis.

Question 28  A ""-year-old man with a +-year history of severe chronic obstructive pulm onary disease is evaluated after being discharged from the hospital following an acute eacerbation; he has had three eacerbations over the previous *8 months. He is a long-term smo!er who stopped smo!ing * year ago. He adheres to therapy with albuterol as needed and inhaled salmeterol and tiotropium and has demonstrated proper inhaler techniue. #n physical eamination, vital signs are normal. @reath sounds are decreased bilaterally; there is no edema or cyanosis. #ygen saturation after eertion is ?2 on ambient air. &pirometry shows an '()* of 72 of predicted and an '() *>')% ratio of =. %hest radiograph done in the hospital 7 wee!s ago showed no active disease.

hich of the following should be added to this patientEs therapeutic regimen/ 0A-An inhaled corticosteroid 0B-Ipratropium 0C-N -acetylcysteine 0D-ral prednisone

 Answer and Critique 28 (Correct  Answer: A) Educational Objective: %ecogni&e the role of inhaled corticosteroids i n severe chronic obstructive pulmonary disease. Key Point  03nhaled corticosteroids may offer significant benefit in patients with severe chronic obstructive pulmonary disease, with the benefit generally greater when an inhaled corticosteroid is com bined with a long-acting : 2-agonist. 0egular use of inhaled corticosteroids in patients with chronic obstructive pulmonary disease 4%#DB5 is associated with a reduction in the rate of eacerbations from *.7 to .? per year, and patients who have freuent eacerbations with more severe %#DB benefit most. 3n si placebo-controlled trials in *+=* patients over 6 months, inhaled corticosteroids reduced eacerbations by 2=. $herefore, the #9B guidelines recommend consideration of inhaled corticosteroids i n patients whose lung function is less than " and those who have eacerbations. hen inhaled corticosteroids are combined with a long-acting : 2-agonist, the rate of decline in uality of life and health status is significantly reduced and acute eacerbations are reduced by 2"; lung function is also improved and dyspnea is alleviated. $here does not appear to be a dose response to inhaled corticosteroids in %#DB, and the effects of combination therapy on mortality are uncertain. 0 Anticholinergic agents in %#DB are especially useful when combined with short-acting or long-acting : 2-agonists. $iotropium is effective in patients with stable %#DB for up to 2= hours and should not be combined with short-acting anticholinergic agents, such as ipratropium. 6 mm Hg, the pulse rate is *2>min at rest and *2>min after the patient wal!s across the room, the respiration rate is 2>min, and the @6 holosystolic murmur at the left sternal border near the fourth rib that increases with inspiration. $he lower etremities are edematous. $here is no cyanosis or clubbing. %omplete blood count and resting arterial blood gases are normal. (lectrocardiography shows a rightward S& ais and large  waves in ) *. &pirometry and plethysmography are normal. $he chest radiograph shows no infiltrates or masses.

hich of the following is the best net step in the evaluation of this patient/ 0A-Bronchoscopy and transbronchial lung biopsy 0B-4ethacholine challenge test 0C-#ight-heart catheterization and pulmonary angiography 0D-(ransthoracic echocardiography

 Answer and Critique 31 (Correct  Answer: D) Educational Objective:Evaluate pulmonary hypertension. Key Point  03n patients with suspected pulmonary hypertension, transthoracic echocardiography can suggest the presence of pulmonary hypertension and evaluate cardiac causes of elevated pulmonary artery pressure. 0$he patientEs progressive dyspnea, hemodynamic symptoms during eercise, and physical findings suggest right ventricular dysfunction and pulmonary hypertension. $ransthoracic echocardiography can confirm the presence of pulmonary hypertension and right ventricular dysfunction. (chocardiography is also useful to rule out left-sided heart disease and congenital heart disease as a cause of pulmonary hypertension. A ventilation>perfusion scan can also rule out potential causes. $ypically, the ventilation>perfusion scan in pulmonary arterial hypertension is either normal or shows a scattered, Nmoth-eatenO perfusion pattern in the peripheral lung zones. 0$he patient has no evidence of bronchospasm. (ercise-induced asthma is unli!ely because the symptoms begin immediately during mild eertion and subside rapidly upon rest. 'urthermore, eercise-induced bronchospasm cannot eplain the patientEs clinical findings of pulmonary hypertension. $herefore, a methacholine challenge test is not indicated. ightheart catheterization and pulmonary angiography might be necessary to confirm the diagnosis of pulmonary arterial hypertension but are not indicated before less invasive screening tests for pulmonary hypertension are done. @ronchoscopy and transbronchial lung biopsy may be indicated in patients with diffuse parenchymal lung disease, but this patientEs chest radiograph is normal, ma!ing parenchymal lung disease unli!ely.

Question 32  A 2-year-old male college student is evaluated for a 7-year history of persistent daytime sleepiness. He snores loudly but h as had no witnessed apneas or catapley. He has occasional episodes of sleep paralysis in which he cannot move for about a minute after awa!ening from sleep. He typically goes to bed at **7 D< on wee!days and at * A< on wee!ends. He falls asleep easily, sleeps uneventfully, and awa!ens at about 6 A< on wee!days and ** Amin, the respiration rate is 22>min, the blood pressure is *7>+8 mm Hg, and the @