B4 histopath quizzes

A 59-year-old man presented with concerns about high blood pressure. At a recent visit to his dentist he was told his blood pressure was high. He was reclining in the dentist's chair when his blood pressure was taken, but he did not remember the exact reading. He had no symptoms. He has never taken medications for high blood pressure. His blood pressure on right arm was 170/120 mm Hg. Chest x-ray showed cardiomegaly. Which of the following molecular events would most likely underlie this patient's cardiomegaly? A) activation of G-protein-coupled receptors B) activation of tyrosine kinase-coupled receptors C) decreased local production of angiotensin II D) increased production of insulin-like growth factor-1 E) inhibition of natriuretic peptide genes

Activation of G-protein coupled receptors

A 33-year-old military recruit presented with a 5-day history of unremitting substernal chest pain that radiated to his shoulders. The pain was worse when he was lying down and improved when he leaned forward. He reported having no dyspnea. He had no chest-wall tenderness and had not undergone any recent trauma. Laboratory studies revealed leukocytosis and elevated erythrocyte sedimentation rate. His serum levels of cardiac enzymes levels were normal, as was his chest CT scan for pulmonary thromboembolism. The next day, a friction rub was heard during cardiac auscultation. ECG showed PR-segment depression and ST-segment elevation in all leads. Transthoracic echocardiography disclosed a moderate-sized pericardial effusion but not left myocardial dysfunction. Which of the following is most likely to explain this patient's clinical and laboratory findings? A) acute pericarditis B) acute myocarditis C) unstable angina D)

Acute pericarditis

A previously healthy 11-year-old boy presented with rapid, irregular, aimless, involuntary movements of the limbs, neck, and trunk that resembled continuous restlessness. These uncontrollable movements had begun one month earlier, initially affecting only his left limbs but later extending to other parts of his body. The involuntary movements were severe enough to impair his daily activities such as writing, brushing teeth and holding a bowl. The patient reported having a sore throat four months before presentation. Physical examination showed that he was afebrile, alert and oriented. Large erythematous skin macules were present on the back, showing a pale center and serpiginous pink margin. Cardiovascular examination was remarkable for a grade 2/6 ejection systolic murmur could be heard over the left upper sternal border. Laboratory studies revealed a level of antistreptolysin O that was five times the normal level.

Acute rheumatic fever

An 11-year-old boy presented with fever, sore throat, and skin rash. His 6-year-old brother had previously been diagnosed with group A streptococcal pharyngitis. The patient was reported to have a significant penicillin allergy. After 7 days of illness, he was empirically treated with azithromycin and diphenhydramine for 10 days. While still taking azithromycin and approximately 2 weeks after initial symptoms, he developed fever of 38.5°C (101.3°F), ankle pain, redness and swelling. The pain, redness and swelling migrated at varying times to his knees and elbows, requiring the use of a wheelchair during a family vacation. Upon return home 18 days after the initial illness, he had a positive group A streptococcal rapid antigen-detection test from his pharynx, and azithromycin was continued. Upon presentation to a hospital at 21 days after initial symptom onset, he was afebrile with normal vital signs. On examination,

Acute rheumatic fever

A 26-year-old woman presented with pain in the fingertips of both hands on exposure to cold. She had had the same symptoms every winter for the past decade. She was a non-smoker, with an unremarkable cardiovascular history. Her physical examination was normal except for sharply demarcated pallor of her hands as shown in the attached figure. All of the laboratory findings were within normal ranges. Immunologic workup was completely negative. There were no signs or symptoms suggestive of systemic lupus erythematosus or scleroderma. Which of the following is most likely to explain the pathogenesis of this patient's vascular disorder?

Alpha 2-adrenergic receptor overactivity

A 6-year-old girl complained of left knee pain. On physical examination, her knee is red, swollen, and tender. Two weeks ago, she had a similar condition in her right ankle. A cardiac examination revealed a murmur. Subcutaneous nodules were noted on her fingers. One month ago, she had pharyngitis caused by group A streptococci. Which of the following is the most accurate statement about her current illness?

Antibodies against S. pyogenes antigens cross-react with normal tissue epitopes

A 34-year-old man presented complaining of chest pain and diaphoresis that have started two days earlier. He explained that his chest pain started after physical exertion. This pain radiated to the back and was of strong intensity. The patient reported that this was the first time to experience such pain. He was 190 cm (6.2 feet) tall and weighed 61 kg (134 pounds), had tall and narrow head, elongated arms and legs, long, slender, and curved fingers. On auscultation, a diastolic murmur over the aortic area was heard. His blood pressure was 150/90 mm Hg. ECG displayed sinus rhythm, heart rate of 80 beats per minute, without any abnormalities. A chest X-ray revealed a heart size that encompassed almost entire left hemi-thorax. Transthoracic echocardiography showed enormous diameter of the aortic root, measuring 80 mm at the level of aortic cusps (normal: 29-45 mm) associated with aortic regurgitation. Mitral valve prol

Aortic dissection

A 44-year-old woman presented with new palpitations. Cardiac auscultation revealed a distinctive murmur. The representative gross appearance of her heart is shown. Which of the following most likely contributed to the development of this lesion? A) carcinoid heart disease B) aortic regurgitation C) cor pulmonale D) mitral stenosis E) Tricuspid regurgitation

Aortic regurgitation

A 61-year-old woman was admitted with complaints of shortness of breath on exertion. The representative gross appearance of her heart is shown. Which of the following most likely contributed to the development of this lesion? A) arterial hypertension B) aortic regurgitation C) cor pulmonale D) mitral stenosis E) tricuspid regurgitation

Arterial hypertension

A 3-year-old girl presented with failure to thrive, progressive exertional dyspnea, and easy fatigability of six months duration. She also has a history of frequent respiratory tract infections. A grade III/VI ejection systolic murmur at the left upper sternal border was audible. Chest X-ray showed cardiomegaly and increased pulmonary blood flow. Transthoracic echocardiography showed a large ostium primum atrial septal defect, a single atrioventricular valve annulus, a common atrioventricular valve with five leaflets, and a ventricular septal defect. Which of the following would most likely explain this patient's clinical findings?

Atrioventricular canal

A 49-year-old woman with insignificant past medical history presented to the emergency department with a chief complaint of progressively worsening shortness of breath of approximately four weeks evolution. Physical examination was remarkable for marked jugular venous distension, basal crackles in both lungs, as well as bilateral +2 lower leg edema. The patient's laboratory studies were remarkable for anemia and markedly elevated natriuretic peptide. Chest X-ray showed a markedly enlarged cardiac silhouette. Transthoracic echocardiogram confirmed the presence of a significantly dilated left and right ventricles with no valvular structural abnormalities. In addition, a compromised left ventricular ejection fraction of 30%-35% was shown. Endomyocardial biopsy showed nonspecific focal interstitial fibrosis and marked variation in cardiomyocyte size. The family history was significant for his father who suffered from hea

B

A study was designed to determine the spectrum of risk factors influencing essential hypertension. A total of 165 cases of essential hypertension and 330 control individuals were selected by systematic random sampling from two hospitals. The clinical, racial, metabolic and dietary profile was evaluated, including body weight and lifestyle factors. Which of the following risk factors would be least strongly associated with essential hypertension in this study?

BMI of 23 kg/m^2

A photomicrograph of coronary artery with atherosclerotic narrowing is shown in the attached figure. The object indicated by the arrow in this photomicrograph is: A) central lipid core B) thrombus occluding the lumen C) calcium deposit in the plaque D) fibrous cap E) uninvolved tunica media

Calcium deposit in the plaque

A photomicrograph of coronary artery with atherosclerotic narrowing is shown in the attached figure. The object indicated by the rectangle in this photomicrograph is: A) central lipid core B) thrombus occluding the lumen C) calcium deposit in the plaque D) fibrous cap E) uninvolved tunica media

Central lipid core

Mr. Martin is a 61-year-old man who presented with exercise-induced chest pain and shortness of breath, associated with numbness in the left shoulder and elbow. His chest pain is provoked by ten blocks of exercise with no change in exercise tolerance. The pain subsides when he slows down, and he seldom had discomfort at a slow pace. He denied pain at rest or nocturnal pain. The patient had a history of mild systemic hypertension and hypercholesterolemia. His resting ECG was normal, but the exercise ECG showed ST-segment depression at a moderate level of bicycle exercise. Coronary angiography revealed an 85% stenosis at the end of the proximal third of the left anterior descending (LAD) coronary artery and a 50% stenosis of the right coronary artery. A myocardial single photon emission thallium scintigram confirmed exercise-induced myocardial ischemia, predominantly in the anterior wall and anterior two thirds of the

Chronic stable angina

A 57-year-old woman presented with a 2-day history of symmetrically distributed rash over lower extremities, and migratory polyarthralgia five days after being treated with amoxicillin and clavulanic acid for pneumonitis. This prescription was discontinued and an intravenous bolus of prednisolone as well as anthistamines and topical glucocorticoids were administered. After two days, her purpuric rash progressed to purpuric, palpable, non-blanching, pruritic and painful palpable plaques on her lower extremities. Which of the following pathophysiologic mechanisms is most likely to underlie this patient's clinical findings?

Circulating immune complexes that secondarily deposit on normal vessel walls

Referring to the previous question, on the eighth day, Mr. Walker had an abrupt cardiac arrest and cardiopulmonary resuscitation with intubation for one hour could not save him. At autopsy, a transmural myocardial infarct was noted with left ventricular free wall rupture at the infarct site. A large blood clot of approximate volume of 300 mL was found inside the pericardial cavity. Which of the following histologic descriptions best characterizes the infarcted myocardium of this patient at the time of death? A) dense collagenous scar B) coagulation necrosis with loss of nuclei C) coagulation necrosis with macrophages at infarct border D) coagulation necrosis with neutrophilic infiltrate E) only edema and wavy fibers

Coagulation necrosis with macrophages at infarct border

A 68-year-old woman presented with severe backache. CT scan of the thoracolumbar spine documented a T12 osteoporotic compression fracture. She was otherwise asymptomatic. Her medical history was remarkable for arterial hypertension, which was effectively controlled with amlodipine 5 mg/day. On physical examination, weak bilateral femoral and pedal pulses were noted. Her blood pressure was 140/90 mm Hg in the upper extremities and 90/60 mm Hg in the lower extremities. A systolic murmur was heard in the interscapular area. On chest X-ray, there was a figure of 3-sign at the aortic knuckle and notching of the inferior ribs. Which of the following most correctly explains this patient's cardiovascular findings?

Coarctation of aorta

A gross photograph of the carotid bifurcation from a deceased individual opened lengthwise with the luminal surface visible is shown in the attached figure. Which of the following types of atherosclerotic lesions is present? A) fatty streak B) mature atherosclerotic plaque C) complicated plaque

Complicated plaque

A 42-year-old woman presents with complaints of headache. She has a 7-year history of hypertension first diagnosed during her last pregnancy. Her family history is positive for hypertension, with her mother dying at 56 years of age from hypertension-related cardiovascular disease. She reports that she has been taking many medications for her hypertension in the past but stopped taking them because of the side effects. Currently she is taking 100 mg/day atenolol and 12.5 mg/day hydrochlorothiazide. Despite this antihypertensive regimen, her blood pressure remains elevated, ranging from 150/110 mm Hg to 165/115 mm Hg. Her physical assessment is unremarkable except for the presence of moderate obesity and minimal retinopathy. An electrocardiogram was significant for left ventricular hypertrophy. Her echocardiogram revealed increased left ventricular wall thickness and normal left ventricular cavity size. Her left ventri

Concentric left ventricular hypertrophy

A 50-year-old woman presented to her physician with dyspnea on exertion that had progressed over the previous several months, particularly with activities such as climbing stairs. Cardiac examination revealed a regular heart rate and rhythm with no rubs or heaves. A 2/6 late-peaking, systolic murmur at the right upper sternal border was transmitted to the carotid arteries and encroached on the second heart sound. The carotid pulse was delayed with low amplitude and upstroke. The point of maximal impulse was nondisplaced but enlarged. Jugular venous pressure was normal. Transthoracic echocardiography revealed an aortic valve with moderate stenosis. The patient moved to the cardiac surgery department, where an aortic valve replacement was performed. The gross appearance of her aortic valve at surgery is shown in the attached figure. Which of the following is the most likely cause of aortic valve stenosis in this patien

Congenital bicuspid aortic valve

A 39-year-old woman was referred to the outpatient lipid clinic by her family doctor due to increased plasma lipid levels. The patient denied any symptoms indicative of cardiovascular disorders, but her father had a heart attack at the age of 50, whereas her mother had been diagnosed with peripheral artery disease at the age of 40. Physical examination revealed no pathologic findings. Lipid profile analysis revealed a total cholesterol of 325 mg/dL (normal: <200 mg/dL), triglycerides of 481 mg/dL (normal: 30-150 mg/dL), LDL cholesterol of 94 mg/dL (normal: <100 mg/dL), and HDL cholesterol of 47 mg/dL (normal: 40-59 mg/dL). Secondary causes of dyslipidemia were excluded by evaluation of thyroid and renal function, urinary protein excretion, serum protein electrophoresis, erythrocyte sedimentation rate and autoantibodies. Genetic testing would most likely reveal abnormalities in which of the following genes? A) ABCG5 B

APOE

A 78-year-old Caucasian male presented with subacute low back pain. The patient reported an intermittent history of low back pain over the past one and half months. The pain was described as a dull achy sensation in the lumbosacral region that radiated into the posterior left leg and was rated on a pain scale as 5 of 10. There was no bowel or bladder dysfunction. His past medical history was remarkable for hypertension and coronary bypass surgery 15 years ago. His vital signs at presentation included a temperature of 37°C (98°F), a blood pressure of 155/100 mm Hg, and a pulse rate of 90 beats per minute. On physical examination, all low back movements were painful. Strong pulsations were found with light palpation over the middle of the abdomen. Aortic bruits were detected by auscultation. His abdominal CT scan is shown in the attached figure. Which of the following best explains this patient's clinical findings?

Abdominal aortic aneurysm

A 36-year-old man with a past medical history of hypertension arrived to emergency department (ED) a few hours after he developed sudden, severe retrosternal pain. The pain was stabbing and was associated with nausea and profuse sweating. Shortly after arrival to the ED, he complained that his chest pain spread to the back. On physical examination his blood pressure was 150/80 mm Hg and pulse 68 beats per minute. The chest, lung, and cardiac exams were reported as normal; the remainder of the physical examination was unremarkable. Laboratory results were all within normal limits. ECG showed sinus tachycardia but no acute ischemic changes. His chest CT scan revealed an aortic dissection extending from the aortic root to the level of the descending thoracic aorta. The patient was taken to the operating room emergently. He underwent prosthetic Dacron graft replacement of the aorta. Histopathological evaluation of his ex

Cystic medial necrosis

An 85-year-old man presented to the emergency department with a 2-hour history of a typical anginal chest discomfort associated with shortness of breath, diaphoresis, nausea, and vomiting. His past medical history was remarkable for chronic stable angina. The initial ECG demonstrated ST-segment elevation in the anterior leads. Emergent coronary angiography showed complete obstruction of the left anterior descending coronary artery. Echocardiography revealed severe akinesis of the anterior region of the left ventricle, apex and the anterior two thirds of the ventricular septum. Laboratory studies revealed a troponin-T level of 17.3 ng/mL (normal: 0.2 ng/mL). One hour later his blood pressure dropped to 75/44 mm Hg, and arterial oxygen saturation dropped to 85%. The patient became, tachypneic, and diffuse bilateral rales were noted at chest auscultation. Ten minutes later he suffered cardiac arrest and could not be res

D

A 66-year-old man was referred to a cardiologist with a history of a heart murmur and three episodes of non-exertional dizziness. His wife reported that he had decreased his physical activity over the past two years because he was "getting old." On physical examination, his blood pressure was 120/70 mm Hg, is pulse was 80 beats per minute, his respiration rate was 13 breaths per minute, and his body temperature was 37.2oC (99.0oF). Cardiovascular examination revealed normal central venous pressure. His carotid upstrokes were reduced in volume and delayed in upstroke. A forceful sustained apical impulse in its normal position was noted. There was a 3/6 mid-systolic ejection murmur, heard best over the right second intercostal space, with radiation into the right neck. The first heart sound (S1) is normal and S2 was soft. The rest of the physical examination was unremarkable. ECG indicated left ventricular hypertrophy.

Degenerative, age related stenosis of a normal trileaflet valve

A 50-year-old man was admitted with severe loin pain as well as macroscopic hematuria. His blood pressure was 180/110 mm Hg. Laboratory studies revealed blood urea nitrogen of 35 mg/dL (normal: 10-20 mg/dL) and creatinine of 2.2 mg/dL (normal: 0.7-1.4 mg/dL). Laboratory studies revealed mild anemia, neutrophilic leukocytosis, and elevated erythrocyte sedimentation rate and C-reactive protein. A percutaneous renal biopsy was performed showing glomeruli and tubules that were unremarkable except at one site where a medium-size artery showed transmural fibrinoid necrosis with moderate neutrophilic infiltration of the adventitial and medial layers. A renal angiography is shown in the attached figure. Which of the following is the most likely underlying mechanism of this patient's condition?

Deposition of antibody-antigen immune complexes

A 71-year-old nursing home male resident was brought in by emergency medical services due to abdominal pain. The nurse had found him diaphoretic with a systolic blood pressure in the 90s. The patient complained of acute abdominal pain but denied chest pain or shortness of breath. The patient had hypertension, insulin-dependent diabetes mellitus, coronary bypass surgery 15 years ago, and peripheral vascular disease resulting in a right above-knee amputation and a left below-knee amputation. He had been smoking 15 cigars per day for nearly 50 years and consumed alcohol occasionally. Physical examination revealed an ill-appearing man who seemed in no acute distress on presentation. His blood pressure was 140/85 mm Hg (controlled). Radiographic examination of the lumbar spine revealed extensive calcification throughout the aorta and an aneurysm about its bifurcation. Abdominal CT scan showed a 4 cm infrarenal aneurysm an

Diabetes The other choices are all risk factors: smoking history, age, coronary artery disease, hypertension

A 20-year-old man with no past medical history collapsed while swimming in a river. His girlfriend along with a bystander began basic life support measures until an ambulance arrived. He was intubated by the paramedical crew and was discovered to be in ventricular fibrillation. Following asynchronous cardioversion, asystole ensued, and he was transferred to hospital receiving endotracheal ventilation and cardiac massage. Further resuscitation in hospital was unsuccessful. An autopsy was performed by the medical examiner. The gross appearance of the heart is shown in the attached figure. Which of the following pathophysiological types of cardiac dysfunction is most likely underlying this patient's heart condition? A) high-output HF B) decreased LV EF C) systolic dysfunction D) diastolic dysfunction E) cor pulmonale

Diastolic dysfunction

A 56-year-old man presented to the emergency department with dyspnea on exertion and fatigue. The patient had one-year-history of shortness of breath and bilateral lower extremity edema. His vital signs were normal. On physical examination, bilateral pitting edema of the foot and ankle was noted. On chest X-ray, the patient had cardiomegaly with perihilar congestion. His ECG demonstrated normal sinus rhythm with low voltage in limb leads. The results of the laboratory studies were within the reference range. Transthoracic echocardiography showed dilated left ventricle, with an ejection fraction of 15%, and moderate functional mitral regurgitation. No coronary disease was detected on cardiac catheterization. All serologies for myocarditis were negative. The representative gross appearance of the heart is shown in the attached figure. Which of the following is the most likely diagnosis? A) hypertrophic cardiomyopathy B

Dilated cardiomyopathy

A 75-year-old man was admitted with severe back pain. The next day, the patient complained of pain irradiating to the abdomen and right lower extremity. On physical examination, decreasing bowel sounds and pulse deficit in the right femoral artery were noted. Chest CT scan revealed a Stanford type B acute aortic dissection. Which of the following descriptions correspond to this type of aortic dissection?

Dissection originated in distal thoracic aorta and propagated to the common iliac arteries

A 56-year-old man was admitted because of retrosternal pain associated with profuse sweating and shortness of breath. The chest pain lasted for 3 hours. The ECG showed signs of acute lateral myocardial infarction. Mild congestion of the lungs was seen on chest X-ray. Laboratory studies revealed serum troponin I of 6.5 ng/dL (normal: < 0.03 ng/mL). Coronary angioplasty and stenting were performed successfully, and the patient was discharged without further complications and was put on anti-ischemic regimen and anticoagulation. Nineteen days after the onset of acute myocardial infarction, the patient was readmitted in ICU for chest pain. The pain was worse when lying flat and relieved by leaning forward and was accentuated by any movement and by deep inspiration. A pericardial friction sound was easily audible at the lower left sternal border. His ECG showed ST segment elevation in all leads. Echocardiography revealed

Dressler syndrome

A 30-year old man with a history of alcohol use disorder presented to the emergency department seeking alcohol detoxification. He reported a two-year history of heavy alcohol intake that averaged approximately ten standard drinks per day. He had developed tremors, anxiety, and diaphoresis during previous cessation attempts at home and therefore presented to the emergency department seeking a medically supervised detoxification. The patient was intoxicated upon presentation, with an elevated blood alcohol concentration as measured in serum. Physical exam was notable only for a slight tremor in outstretched hands. Serum lipid profile is most likely to show which of the following?

Elevated triglycerides

A 37-year-old man presented with one-year history of unstable angina. Past medical history included active smoking and grade 2 obesity. On physical examination, the patient was in good condition. Blood pressure was 160/80 mm Hg. Laboratory studies revealed a total cholesterol of 210 mg/dL (normal: <200 mg/dL) and triglycerides of 165 mg/dL (normal: 101-150 mg/dL). The treadmill test was positive for myocardial ischemia. A transthoracic echocardiogram showed dilatation of the ascending aorta and severe ostial stenosis of both the left and right coronary arteries. Simultaneously, the echocardiogram indicated slight aortic regurgitation. Chest CT scan confirmed high-grade ostial stenosis of both the left main coronary artery and the right coronary artery. No atherosclerotic lesions were observed in the distal coronary arteries. The CT clearly showed coronary ostial circumferential aortic wall thickening with partial cal

Endarteritis obliterans of vasa vasorum with fibrosis and destruction of the media

A 50-year-old man presented to his family physician for weight-loss counseling. He reported that his brother recently had a heart attack at 55 years of age. He does not smoke and takes no medications other than a daily multivitamin. Vital signs are normal except for a BMI of 35 kg/m2 (normal: 18.5-25 mg/m2). Fasting laboratory work revealed that his total cholesterol was 315 mg/dL (normal: <200 mg/dL), and his LDL is 189 mg/dL (normal: <100 mg/dL). Which of the following processes is most directly involved in formation of atherosclerosis in this patient?

Endothelial dysfunction

A 70-year-old woman, with a history of childhood asthma, was admitted because of an episode of hemoptysis, nonproductive cough, and right calf numbness that extended into the dorsolateral foot corresponding to the distribution of the sural nerve. Laboratory studies revealed a leukocyte count of 19,500 cells/mm3 (normal: 4,000-10,000 cells/mm3) with 25% eosinophils (normal: 0-6%) and erythrocyte sedimentation rate of 102 mm/hour (normal: 0-15 mm/hour). ANCA panel was positive for perinuclear antineutrophil cytoplasmic antibody (p-ANCA). Chest CT scan showed multiple pulmonary nodules in both lungs surrounded by a ground glass appearance. The CT-guided lung mass biopsies revealed granulomas with few multinucleated giant cells surrounding a central area of necrotic eosinophils. Sural nerve biopsy demonstrated eosinophilic infiltration, especially around the vessels and the perineurium. Which of the following is the most

Eosinophilic Granulomatosis with Polyangiitis

Referring to the previous question, which of the following histopathologic types of coronary lesions would most likely be responsible for Mrs. Bradshaw's chest discomfort? A) complete rupture of the fibrous cap with occlusive coronary thrombosis B) coronary microvascular dysfunction C) erosion of the fibrous cap with a non-occlusive thrombus D) large lipid-core and a thin fibrous cap E) small lipid-core and a thick fibrous cap

Erosion of the fibrous cap with a non-occlusive thrombus

The 33-year-old Mrs. Jordan presented with a skin eruption that had appeared three months earlier. Examination revealed crops of pink-yellowish papules over the elbows, buttocks, knees, flank, and palmar creases as shown in the attached figure. A 4 mm punch biopsy of one of the papules was performed. Histologic examination of H&E-stained sections demonstrated foamy macrophages scattered throughout multiple levels of the dermis. Based on the patient's history, physical examination and biopsy results, which one of the following is the most likely diagnosis? A) eruptive xanthomas B) tendinous xanthomas C) tuberous xanthomas D) xanthelasma

Eruptive xanthomas

A 22-year-old woman was noted to have yellowish deposit near the inner angle of the upper eyelids as shown in the attached figure. She was identified by an emergency room physician who attended to her for a minor wound over her leg due to a cycling accident. She had no complains of chest pain, shortness of breath and had good effort tolerance. Three of her siblings died at a very young age, which she claimed were due to "heart attacks". There was marital consanguinity in the family in that both parents are cousins. On examination, there was a whitish ring at the corneoscleral limbus. Other systems were unremarkable. Laboratory studies revealed a total cholesterol level of 620 mg/dL (normal: <200 mg/dL), LDL cholesterol of 450 mg/dL (normal: 80-140 mg/dL), HDL cholesterol of 58 mg/dL (normal: 50-59 mg/dL) and triglyceride of 90 mg/dL (normal: 50-100 mg/dL). She was found to have homozygous mutation in the LDLR gene. W

Familial hypercholesterolemia

A 40-year-old man, who came for a routine screening in a diagnostic laboratory, was diagnosed having hypertriglyceridemia. His laboratory studies revealed a plasma triglyceride of 372 mg/dL (normal: 30-150 mg/dL), fasting glucose of 89 mg/dL (normal: 70-100 mg/dL), hemoglobin A1c of 5.4% (normal: <5.7%), total cholesterol of 175 mg/dL (normal: <200 mg/dL), LDL-cholesterol of 95 mg/dL (normal: <100 mg/dL), and HDL cholesterol of 55 mg/dL (normal: 40-59 mg/dL). His blood urea nitrogen was 8 mg/dL (normal: 10-20 mg/dL), his serum creatinine was 0.7 mg/dL (normal: 0.7-1.4 mg/dL), and urine was within normal limits. His thyroid function, and serum protein electrophoresis were normal. His blood pressure was normal, his body-mass index was 23 kg/m2 (normal: 18.5-25 kg/m2), and he did not have xanthoma or hepatosplenomegaly. On interrogation, he was found to be a nonsmoker, non-alcoholic, reasonable diet with abundant fruits

Familial hypertriglyceridemia

A gross photograph of the aorta from a deceased individual opened lengthwise with the luminal surface visible is shown in the attached figure. Which of the following types of atherosclerotic lesions is present? A) fatty streak B) mature atherosclerotic plaque C) complicated plaque

Fatty streak

A 14-year-old girl presented with complaints of palpable purpura over lower extremities for last five days. The patient was receiving anticonvulsive therapy (phenytoin) for epilepsy since she was three years old. Two weeks ago, her neurologist added levetiracetam 500 mg daily to phenytoin 50 mg thrice daily, owing to worsening of epilepsy symptoms. Eight days after initiation of levetiracetam, the patient developed palpable purpura over lower extremities, associated with itching. Routine laboratory studies were normal. Complete hemogram ruled out the presence of eosinophilia and antineutrophil cytoplasmic antibodies. Microscopic examination of a skin biopsy specimen from one of her lesions would most likely demonstrate:

Fibrinoid necrosis with nuclear debris of necrotic neutrophils

A 35-year-old woman was diagnosed with hypertension refractory to three antihypertensive medications. CT scan demonstrated stenosis of the right renal artery. A catheter-directed angiography was performed, and the angiographic appearance of the right renal artery is shown in the attached figure. Which of the following conditions is most likely to underlie this patient's refractory hypertension?

Fibromuscular dysplasia

A 30-year-old man was incidentally found to have hypertension with a documented markedly elevated blood pressure. On physical examination, there was a vascular bruit over the right subclavian arterial area. His blood pressure was 180/90 mm Hg in the left arm but non-recordable in the right arm. Assessment with magnetic resonance angiography found severe stenosis of the right subclavian artery, stenosis of the left renal artery and stenoses of the infra-renal abdominal aorta. Laboratory studies revealed anemia, leukocytosis and elevated erythrocyte sedimentation rate. A bypass surgery was performed between the right common carotid artery and right axillary artery using a saphenous vein graft. Doppler ultrasound demonstrated adequate blood flow through the graft and the axillary artery. Which of the following microscopic lesions would most likely be present in the biopsied right subclavian artery?

Granulomas with multinucleate giant cells

A 40-year-old man presented with chest pain, lethargy, and breathing difficulties of 6 hour duration. His past medical history was significant for tuberculosis diagnosed two years prior. He was being treated for tuberculosis at the time of presentation. At the hospital, he was noted to have ventricular tachycardia (120 beats per minute), mild hypotension (104/46 mm Hg), and fever (38.3°C, 101.1°F). His respiration was rapid, shallow, and grunting-like. His pupils were dilated but reactive. Laboratory studies revealed a leukocyte count of 13,100 cells/mm3 (normal: 4,000-10,000 cells/mm3) and cardiac troponin T of 9.6 ng/mL (normal < 0.2 ng/mL). A urine toxicology screen was negative. Chest X-ray revealed an enlarged heart. ECG revealed nonspecific ST abnormalities. Transthoracic echocardiography demonstrated a severely depressed ejection fraction. An endomyocardial biopsy showed an inflammatory infiltrate composed of

Granulomatous myocarditis

A 54-year-old man with no past medical history presented to the emergency room with two weeks history of bilateral lower extremity edema and dyspnea on exertion. Physical exam revealed a healed wound in the neck that had been present since he was stabbed in the neck by his girlfriend thirty years ago. A palpable thrill was also present over the right base of the neck with a loud continuous bruit. The patient had 2+ edema extending up to his knees bilaterally, jugular venous distension, and bibasilar pulmonary rales. Brain natriuretic peptide was 1,000 pg/mL (normal: <100 pg/mL), and hemoglobin was 13.3 g/dL (normal: 12-18 g/dL). A transthoracic echocardiogram was notable for moderate left ventricular dilation with left ventricular ejection fraction of 35%. Left heart cardiac catheterization revealed no significant coronary artery disease. Right heart catheterization showed pulmonary hypertension (45/22 mm Hg). Cardia

High-output heart failure

A 43-year-old man with a history of uncontrolled hypertension, 13-year history of hemodialysis-dependent renal failure and medical noncompliance was found unresponsive in his apartment. Resuscitation efforts failed, and the patient died. His last hemodialysis appointment was 5 days prior, as he had refused dialysis 2 days prior to his demise. On postmortem examination, an enlarged heart was identified. The left ventricle had a markedly thickened free wall but a normal-sized cavity. The coronary arteries had a mild degree of atherosclerosis. The kidneys were markedly shrunken with granular surfaces consistent with a history of chronic renal failure and hemodialysis. The renal cortices were thin. The remainder of the internal examination was unremarkable. The microscopic appearance of the renal afferent arteriole is shown in the attached figure. What is the appropriate histopathological diagnosis?

Hyaline arteriosclerosis

A study was designed to investigate renal outcomes in biopsy-proven benign hypertensive nephrosclerosis. A total of 194 patients were enrolled. All enrolled patients had either proteinuria (>0.4 g/24 h), or hematuria (urine sediment red cell count >100,000 cells/mL) and/or impaired renal function (plasma creatinine >1.5 mg/dL). The median duration of hypertension was 5 years, and the mean systolic and diastolic blood pressures were 155 mm Hg and 95 mm Hg, respectively. Renal biopsies were performed and the tissue for light microscopy was serially sectioned, using H&E and Masson trichrome stains. All biopsy slides were re-reviewed by two pathologists. Which of the following microscopic changes would be least likely to be found in these patient's renal biopsies?

Hyperplastic arteriosclerosis The other choices are more likely to be microscopic changes found in their renal biopsies: - hyaline arteriosclerosis of the afferent arteriole - completely hyaline glomeruli - interstitial fibrosis - tubular atrophy

A 33-year-old woman was referred to internal medicine department by her primary care provider after the recent diagnosis of severe hypertension. While the diagnosis of hypertension dated back to the day before, its onset was actually unknown, as the patient had no memory of having ever measured her blood pressure before, consistent with the low awareness that young adults have of their hypertension. During the visit she complained of fatigue. Otherwise, her medical and family histories were unremarkable. She did not take any prescription or over-the-counter medications. On admission, her blood pressure was 240/140 mmHg, but her other vital signs were normal and physical examination was unremarkable. Following 30 minutes, a repeat blood pressure was 218/120 mm Hg. Initial laboratory studies revealed a serum creatinine of 2.11 mg/dL (normal: 0.7-1.4 mg/dL), and a 24-hour proteinuria of 1.9 g. Echocardiography showed in

Hypertensive emergency

A 16-year-old boy complained of feeling unwell at the end of the school day. Shortly afterwards he collapsed while walking home. Onlookers promptly started cardiopulmonary resuscitation, but these measures were unsuccessful. According to his mother, the boy had been active, healthy, and in generally good physical condition until the day he died. He had not been involved in competitive athletics. A complete forensic necropsy was performed. Mass spectrophotometric analysis for over 300 drugs on blood and urine was negative. All non-cardiac organs were normal. The heart weighed 600 g. A concentric hypertrophy of the left ventricle (LV) was observed. The microscopic appearance of the myocardium is shown in the attached figure. Which of the following underlying cardiac disorders is most likely responsible for this patient's sudden death? A) cardiac amyloidosis B) hypertrophic cardiomyopathy C) restrictive cardiomyopathy D

Hypertrophic cardiomyopathy

An animal model of hypercholesterolemia was developed by knocking out LDLRAP1 gene expression in mice. Exon 4 of the LDLRAP1 gene was deleted via homologous recombination. No immunoreactive LDLRAP1 protein was detected in liver lysates of mice homozygous for the disruption. LDLRAP1-deficient mice were housed on a 12-h dark/12-h light cycle and given standard chow and water ad libitum. At various time points, blood samples were drawn, and cholesterol and triglyceride levels were measured. Lipid profile evaluation of the mice with homozygous deletion of LDLRAP1 gene revealed an average total cholesterol plasma level of 133 mg/dL. Lipid profile evaluation of the wild type mice revealed an average total cholesterol plasma level of 58 mg/dL. The reference range of total cholesterol levels in mice is 49-89 mg/dL). Which of the following mechanisms is most likely to explain elevated plasma total cholesterol in mice with hom

Inefficient LDL uptake in hepatocytes due to LDL receptor malfunction

The 68-year-old Mr. Connolly visits his physician. He was told a year earlier that his blood pressure was somewhat elevated and was advised to reduce salt intake and increase physical activity. Otherwise he has been in good health. On physical examination, his blood pressure is 178/72 mm Hg, with no clinically significant differences between arms or on standing. He has a body mass index of 28.4 kg/m2 (normal: 18-25 kg/m2). The examination is otherwise unremarkable. Urinalysis is normal. The nonfasting blood glucose level is 95 mg/dL (normal: 80-100 mg/dL), serum potassium is 4.2 mEq/L (normal: 3.5-5 mEq/L), and creatinine is 1.2 mg/dL (normal: 0.7-1.4 mg/dL). Which of the following high blood pressure-related conditions would he most likely have?

Isolated systolic hypertension Arterial stiffening with age causes a disproportionate rise in systolic pressure associated with a decrease in diastolic pressure

Mr. Walker is a 60-year-old man who was admitted for an acute typical anginal pain which occurred at rest and continued for several hours. The pain radiated to the shoulders, and was associated with diaphoresis, and lightheadedness. On physical examination he had no fever, his blood pressure was 120/70 mm Hg, his heart rate was 65 beats per minute and cardiopulmonary auscultation was normal. ECG on admission, seven hours after the onset of pain, showed ST segment elevation in the precordial leads (V1-V6) and leads I and aVL. His troponin I was 1.7 ng/mL (normal: < 0.03 ng/mL) and his CK-MB was 23.8 ng/mL (normal: <7 ng/mL). An ECG on the seventh day showed a Q wave in V1-V4. A representative cross section of the heart from a person with a similar condition is shown in the attached figure. A coronary angiography would most likely show a critical lesion in which of the coronary arteries? A) Left anterior descending B)

Left anterior descending

A 27-year-old woman presented with six-month history of exertional dyspnea. ECG showed right axis deviation and evidence of right ventricular hypertrophy. A chest x-ray revealed cardiomegaly. A cardiac MRI with gadolinium enhancement revealed the presence of the anomalous pulmonary vein arising from the upper lobe of the left lung and draining into the left brachiocephalic vein. What pathophysiologic pattern of circulatory alterations is this patient most likely to have?

Left-to-right shunt

A 29-year-old woman was admitted with complaints of malaise and progressive breathing difficulties. Clinical examination revealed hepatomegaly, raised jugular venous pressure, bilateral pleural effusion and bilateral pitting pedal edema. Murmurs of tricuspid and mitral regurgitation were noted. Laboratory studies showed a leukocyte count of 16,000 cells/mm3 (normal: 4,000-10,000 cells/mm3) with 30% eosinophils (normal: 1-6%). Her symptoms began 12 months after she returned to the United States from a 10-day safari in northern Uganda. Chest X-ray revealed cardiomegaly. Transthoracic echocardiography showed grossly dilated right and left atria, and tricuspid and mitral regurgitation. Doppler studies showed left and right ventricles with preserved wall thickness and systolic function but with reduced ventricular cavities at the longitudinal axes, due to the filling of their apices by thrombi and restrictive flow pattern

Loeffler endocarditis

A 22-year-old woman presented at the emergency department with severe, substernal, crushing chest pain of 45 minutes' duration and shortness of breath. Within the past week, she had experienced shortness of breath, diaphoresis, nausea, and vomiting. She reported having a gastrointestinal illness involving emesis and diarrhea the week before she developed the chest discomfort. Her vital signs at presentation included a temperature of 37.5°C (97.5°F), blood pressure of 106/64 mm Hg, pulse rate of 66 beats/min, and respiratory rate of 24 breaths/min. On physical examination she had jugular venous distention and ankle edema. Serum levels of cardiac troponin I and C reactive protein were elevated. Her ECG showed ST segment elevations in all leads. A chest X-ray revealed globular cardiomegaly. A transthoracic echocardiogram demonstrated biventricular dilatation, global hypokinesis, with ejection fraction of 20% (normal: 50

Lymphocytic inflammatory infiltration and cardiomyocyte necrosis

A 6-month-old girl was admitted with difficulty in breathing since birth, fever and cough for the past two months. When she was two months old, she was diagnosed with Down syndrome and an echocardiogram revealed an 8-mm wide ventricular septal defect. The patient had tachycardia and tachypnea at admission. On examination, the child was hypotonic and had upslanting palpebral fissures, flat nasal bridge, low set ears, a protruding tongue and sandal toes (dysmorphic features suggestive of Down syndrome). She lower chest indrawing, nasal flaring and diffuse rales on auscultation, but was not cyanosed and did not have finger clubbing. Her cardiac examination revealed a pan systolic murmur best heard over the lower left sternal border on auscultation. Her chest x ray revealed patchy opacification and an enlarged heart. Transthoracic echocardiogram showed a large ventricular septal defect (1 cm in diameter). The representat

Membranous

A 65-year-old woman was admitted with chief complaint of peripheral neuropathy. About 12 weeks prior she had burning pain, tingling sensation and numbness in her left foot ascending up to the knee. Twenty days later she developed similar tingling sensation and numbness in her right upper limb extending from elbow to hand. One months later she developed pinpricking sensation and numbness in her left foot extending up to the knee. Physical examination was remarkable for symmetrical palpable purpura of her lower extremities. Laboratory studies revealed mild anemia, neutrophilic leukocytosis, and elevated C-reactive protein. Her blood urea nitrogen and serum creatinine were 66 mg/dL (normal: 10-20 mg/dL) and 5.29 mg/dL (normal: 0.7-1.4 mg/dL), respectively. The myeloperoxidase (MPO)-ANCA titer was positive at 234 U/mL (normal < 3.5 IU/mL). Skin biopsy of the lower extremity purpura revealed leukocytoclastic vasculitis. P

Microscopic Polyangiitis

A 51-year-old woman was hospitalized admitted because of chest pain, occurring both during effort and at rest. Episodes of rest angina lasted 20 minutes and were unresponsive to nitrates. An exercise stress test induced ST-segment depression and long-lasting chest pain. Coronary angiography demonstrated no evidence of obstructive coronary disease. Intracoronary administration of ergonovine did not provoke epicardial coronary artery spasm. Intracoronary administration of acetylcholine induced chest pain and ST-segment elevation. Which of the following is the most likely diagnosis? A) chronic stable angina B) microvascular angina C) prinzmetal angina D) ST-segment elevation MI E) unstable angina

Microvascular angina

A study was initiated to determine the spectrum of cardiac valvular abnormalities in patients with acute rheumatic heart disease by use of two-dimensional and color Doppler echocardiography. One hundred eight consecutive patients with acute rheumatic heart disease formed the study population. The diagnosis of acute rheumatic fever was based on the revised Jones criteria. The age of the patients ranged from 5 to 23 years. The structure and function of all cardiac valves were carefully evaluated. Anomalies identified with two-dimensional and Doppler echocardiography included valvular thickening, restriction of leaflet mobility, valvular stenosis, valvular regurgitation and presence of focal valvular nodules. The study would most likely demonstrate that the most commonly affected cardiac valve in patients with rheumatic fever is:

Mitral

A 55-year-old man was referred to the cardiovascular department with shortness of breath and paroxysmal atrial fibrillation. He had a long-time history of hypertension, type 2 diabetes mellitus, and hyperlipidemia. He had a cerebrovascular accident past year with full recovery. His symptoms were mainly shortness of breath and palpitation upon mild exertion with no other cardiac symptoms. A grade 3 mid-diastolic rumbling murmur of was noted. The murmur features were low pitch, rumbling in character, and best heard at the apex with the patient in the left lateral position. Laboratory tests of renal function, liver function, calcium, phosphate, and parathyroid hormone were normal. His chest x-ray is shown in the attached figure. Which of the following is the most likely explanation of shortness of breath in this patient?

Mitral annular calcification

An autopsy was performed on a deceased 67-year-old man who died during an acute asthmatic attack. The pathologist noted that the pancreaticoduodenal, tibial and radial arteries feel hard, cord-like and nodular on palpation. Multiple samples were taken from these vessels and histologic examination showed dystrophic calcification of the tunica media. The intima and adventitia were unaffected. There was no associated inflammatory reaction. The diagnosis is:

Monckeberg arteriosclerosis

A 62-year-old man with type 2 diabetes mellitus presented with chest pain at rest over the past 6 days. Physical examination at admission was normal. Laboratory studies revealed elevated levels of troponin T suggestive of recent myocardial infarction. Coronary angiography was attempted through the radial artery but failed because of extensive calcification of radial artery extending up to the brachial artery, revealed at fluoroscopy. Coronary angiography was subsequently performed via the femoral approach and revealed significant stenoses in any of the three major epicardial coronary arteries. Plain x-rays of hands and forearms performed after unsuccessful radial puncture, showed extensive calcification of radial, ulnar and digital arteries bilaterally. The representative microscopic appearance of his radial artery cross section is shown in the attached figure. This patient's radial artery calcifications are most lik

Monckeberg's medial calcific sclerosis

A 16-year-old girl presented with chief complaint of palpitations. She reported experiencing daily symptoms of "rapid heart beating out of her chest". Her blood pressure was 117/71 mm Hg, and her heart rate was 127 beats per minute. ECG revealed minimal ST segment abnormality, minimal non-specific T-wave flattening, and nonparoxysmal junctional tachycardia. Cardiac auscultation was remarkable for a midsystolic click followed by a late systolic murmur heard at the apex. Cardiac isoenzymes were normal. The gross appearance of the representative mitral valve with the same condition is shown in the attached figure. Which of the following pathologic processes is most likely to explain this patient's clinical findings?

Myxomatous degeneration

A 24-year-old man reported to the radiology department for a chest x-ray, as part of his pre-employment medical examination. He was asymptomatic, his past medical history was not significant. His chest x-ray is shown in the attached figure. A cardiac anomaly is noted. What pathophysiologic pattern of circulatory alterations is this patient most likely to have?

No shunt, No obstruction!

A 23-year-old male college student was seen with a 1-week history of numbness and the sensation of coldness of her left toes. The patient had a three-year history of smoking 20 cigarettes a day. Neither the left popliteal, posterior tibial, nor dorsalis pedis arteries had palpable pulses, and Doppler ultrasound scans showed no flow at the left ankle. Arteriograms demonstrated that the left popliteal artery was occluded segmentally at the popliteal fossa, and that the anterior and posterior tibial arteries were occluded at their origins. Which of the following microscopic findings would most likely be found in this patient's occluded vascular segments?

Occlusive inflammatory thrombus containing a microabscess

A 10-year-old boy was admitted with two-week history of pain, redness and swelling affecting multiple joints. His right knee and left ankles were affected first. The pain, redness and swelling then gradually spread to his left knee and right ankle and impeded his ability to walk. On the day of admission, the patient also complained of new onset of some left metacarpophalangeal and distal interphalangeal joint tenderness. He reported a sore throat six weeks prior, which was treated with amoxicillin for seven days. On physical exam, the patient had a temperature of 38.8°C (100.6°F), redness and swelling in both knees, right ankle and fifth distal interphalangeal joint. There was no evidence of a rash or cardiac murmur. Laboratory studies revealed neutrophilic leukocytosis and elevated erythrocyte sedimentation rate. Knee arthrocentesis yielded cloudy yellow fluid with elevated leukocyte count and negative culture. Join

One major manifestation and two minor manifestations

A heart murmur is noted during the preschool physical examination of a 4-year-old boy. He was an active child but tired easily with prolonged exertion. On physical examination, a slight prominence of the precordium was observed, and upon palpation, a hyperactivity of the heart was detected along the lower left sternal border. The lungs were clear and there was no cyanosis. Transthoracic echocardiography revealed an atrial septal defect with left-to-right shunt, with dilated right atrium and right ventricle. He was admitted to the hospital for corrective surgery. At operation a septal defect was present resulting in a roughly elliptical opening which was 3 cm long by 2 cm wide and located in the lower portion of the atrial septum immediately above the atrioventricular valves. Which of the following types of atrial septal defect is this patient most likely to have?

Ostium primum defect

A study was designed to examine the morphometric characteristics of diet-induced atherosclerotic lesions in rabbits maintained on varying levels of dietary cholesterol. Rabbits were meal-fed a daily ration of a chow diet supplemented with 2% cholesterol for a total of 9 weeks. All the rabbits demonstrated sharply elevated serum cholesterol levels which averaged 1,100 mg/dL after four weeks of dietary treatment with relatively little elevation thereafter. The rabbits were sacrificed after 9 weeks. The aorta, and its major aortic branches were collected, fixed in 10% formalin, immersed in Sudan solution and were grossly evaluated for the location of Sudanophilic atherosclerotic lesions. Sudanophilic arterial lesions were present in the gross in all animals and developed preferentially in:

Outer wall of bifurcations opposite the flow divider

A 33-year-old woman was admitted because of a sudden onset of right-sided facial droop, right-sided weakness and slurred speech associated with headache. Her medical history was unremarkable. Her vital signs at admission are within the norm al range. Cardiovascular examination was unremarkable. Neurological examination was notable for expressive dysphasia, right facial nerve palsy sparing the forehead, and grade 2/5 right upper and lower limb weakness. Lipid panel was normal. The complete thrombophilia screen was negative. Brain MRI showed an acute left basal ganglia infarction. ECG showed sinus rhythm. A transthoracic echocardiography showed no cardiac thrombi. Duplex ultrasonography revealed an underlying deep-vein thrombosis of the left popliteal vein. Pulmonary perfusion scintigraphy excluded pulmonary embolism and lung diseases. Which of the following conditions would most likely explain this patient's clinical

Patent foramen ovale

A 14-year-old boy felt very fatigued after a high school football match. He was taken to a local emergency department where an ECG revealed frequent premature ventricular complexes. His family history was significant for several paternal male relatives who died prematurely in their early fifties. A 24-hour Holter monitor revealed significant incidence of premature ventricular complexes (20%). Echocardiography showed severe dilation of the right ventricle (RV), with depressed RV systolic function and moderate tricuspid valve regurgitation. Left ventricular size and function were normal. Coronary arteriograms were normal. Cardiac MRI revealed severe dyskinesia of the anterior wall of the right ventricle, and moderate RV dysfunction with an ejection fraction of 32% (normal: 50-65%). His family history was significant for a brother who died suddenly at the age of 26 years. Genetic analysis would most likely reveal mutati

Plakophilin-2

An 18-year-old man was referred because of lateral facial birthmark. On the clinical examination a unilateral, extensive, flat-thick, red-purple cutaneous lesion was noted on his right face, not crossing the midline and not associated with increased skin temperature. The lesion extended from about two cm below the hairline superiorly to the angle of the mouth inferiorly. There was no family history of similar lesions. He was otherwise healthy. Brain MRI showed absence of abnormalities. This lesion most likely represents a/an:

Port wine stain

A 38-year-old man presented with six-week history of persistent swelling and multiple red raised lesions on left lower limb. There was no history of trauma or fever with chills prior to onset of lesions. On physical examination, non-pitting edema of the left lower limb was noted associated with multiple pink to red compressible papules coalescing to form plaques. There was no lymphadenopathy. The patient underwent an ELISA test for human immunodeficiency virus (HIV) which turned out to be positive. Laboratory studies revealed an absolute CD4 count of 115 cells/mm3 (normal: 337-1513 cells/mm3) and CD8 count of 1336 cells/mm3 (normal: 174-1240 cells/mm3). A biopsy of the cutaneous plaque showed numerous slit-like spaces formed by vascular channels dissecting into the collagen of upper and mid-dermis. There was extravasation of red blood cells admixed with sparse perivascular lymphocytic infiltrate. Which of the followi

Positive PCR test for a virus that belongs to Herpesviridae family

A 19-year-old woman was admitted to emergency department with chief complaint of increasing dyspnea on exertion of 2- or 3-days duration. She had had an upper respiratory tract infection three weeks previously. Physical examination revealed normal first and second heart sounds without any audible murmurs, rubs or gallops. Laboratory studies revealed markedly elevated serum levels of cardiac troponin I. ECG revealed diffuse ST-segment elevation throughout the precordial leads, with 1.0-mm PR-segment depression in leads I and II. Chest x-ray showed mild cardiac enlargement, and subsequent transthoracic echocardiography revealed a small circumferential pericardial effusion and normal left ventricular function. The patient immediately underwent coronary angiography, which showed normal epicardial coronary arteries. Endomyocardial biopsy procedure obtained five specimens were from the right ventricle side of the intervent

Primary myocarditis

A 45-year-old woman presented to the emergency room (ER) with retrosternal 'squeezing' chest pain. Admission ECG demonstrated ST-segment elevation in the inferior leads. She reported several episodes of similar chest pain that occurred during the night and wake her up from sleep. These episodes of chest pain were approximately of 10 minutes' duration. Additionally, she reported a 20-pack year smoking history with continued use. Her past medical history was unremarkable. In the ER symptoms spontaneously resolved after some minutes and ST-segment elevation disappeared in a repeated ECG. On examination, her vitals were stable. Electrolytes, full blood count, cardiac troponin I, and lipids were normal. Initial coronary angiography showed a tapering 90% stenosis of the mid left anterior descending (LAD) coronary artery. After administration of intravenous nitroglycerin impressive vasodilation of the LAD was observed. Whic

Prinzmetal angina

A 70-year-old woman presented with sudden resting angina. A 12-lead ECG showed typical ST-T changes of acute myocardial infarction. Echocardiography revealed akinesis of the anterior region of the left ventricle including apex. Urgent coronary angiography did not show an obstructed coronary artery. Administrations of intravenous ergonovine during coronary angiography triggered a spasm of the proximal segment of the left anterior descending coronary artery associated with chest pain and transient ST-segment elevation on ECG. After injection of intracoronary nitroglycerin, the spasm was completely resolved, and her ST-segment changes were normalized. The results of laboratory studies including cardiac troponin and creatine kinase-MB were within normal levels during the entire time. The patient was discharged and did well after that. One-week follow-up echocardiography showed persistent anterior left ventricular akines

Prinzmetal angina with myocardial stunning

A 66-year-old man was admitted with fever, cough, and 10 kg (22 pounds) weight loss within the last two months. He had a 30 pack/year history of smoking. Six months before admission, he developed chronic obstructive sinusitis with a right nasal mass. CT of the sinuses revealed an inflammatory mass lesion in the upper right nasal cavity and anterior ethmoid sinuses with associated destruction of the anterior body septum and nasal bone. His temperature at admission was 38ºС (100.4ºF). A chest x-ray showed right upper lobe consolidation. Laboratory studies revealed an erythrocyte sedimentation rate of 123 mm/hr (normal: 0-20 mm/hour), and serum creatinine of 3.11 mg/dL (normal: 0.7-1.4 mg/dL). Serum PR3 antibody was 149 AU/mL (normal: <19 AU/mL). Chest CT scan revealed 8 x 6 cm cavitating right upper lobe lesion and bilateral hilar and mediastinal lymphadenopathy. Transbronchial needle biopsy was positive for necrotizin

Release of leukocyte cytoplasmic granule contents in close proximity to the vessel wall

A 35-year-old woman presented with one-year history of intractable hypertension. Physical examination revealed a blood pressure of 150/100 mm Hg (multiple readings taken from both arms on different occasions were similar). The patient was being treated with three antihypertensive medications including a beta-blocker, diuretic and a calcium channel blocker. Her cardiovascular, respiratory, and central nervous system examinations were unremarkable. There was no evidence of retinopathy on fundus examination. There was no carotid, abdominal or femoral arterial bruits. Transthoracic echocardiography showed a normal left ventricular function but with a concentric hypertrophied left ventricle. Her blood urea nitrogen (BUN) was 50 mg/dL (normal: 10-20 mg/dL) and serum creatinine was 2.2 mg/dL (normal: 0.7-1.4 mg/dL). Selective renal angiography revealed severe stenosis of the distal segment of the left renal artery. These fi

Renin

A 41-year-old woman was admitted with complaints of shortness of breath on exertion and at rest, easy fatigability, and swelling of her feet and legs. During the previous three weeks she had gained 8 kg. She had been hospitalized one month earlier with similar symptoms. She denied chest pain and fever. Past history included two episodes, at ages 9 and 16 years, of febrile illness characterized by arthritis involving many joints simultaneously and heart murmur. At physical examination, her blood pressure was 130/85 mm Hg, her pulse was 110 beats per minute and irregular, her respiration rate was 40 breaths per minute, and her temperature was 37°C (98.6°F). She had 4+ edema of both feet and legs, moist bubbling sounds were heard over both lungs and the liver was palpated 4 cm below the rib cage in the right costal region. Cardiovascular examination revealed a diastolic murmur best heard at the apex with the patient

Rheumatic valvulitis

A 57-year-old man with a history of chronic obstructive pulmonary disease (COPD) presented with chief complaints of increasing dyspnea, ankle edema, and ascites over the past year. The patient was a smoker (20 pack-years). His vital signs at admission were temperature of 36.8°C (98.2°F), blood pressure of 90/60 mm Hg, pulse regular at 78 beats per minute, respiratory frequency of 22 cycles per minute, and oxygen saturation while breathing ambient air of 74% (normal: 95-99%). His jugular venous pressure was markedly elevated. The liver was pulsatile and palpable 5 cm below the costal margin. There was evidence of ascites, and there was 3+ symmetric lower extremity edema. The lungs were clear to percussion and auscultation. ECG revealed right axis deviation, with right atrial enlargement and right bundle branch block. A transthoracic echocardiography revealed severe right ventricular hypertrophy and dilation of the rig

Right-sided heart failure

A two-month old boy born at term presented with a large cutaneous pink-red macula on his median forehead and glabella. The size of the patch was approximately 4.0 × 3.0 cm. The boy's general health was good. This lesion regressed within the next few years. What is the most likely diagnosis of this lesion? A) port wine stain B) salmon patch C) lymphangioma D) sturge-Weber syndrome E) infantile hemangioma

Salmon patch

A 63-year-old man was hospitalized with symptoms of cardiogenic shock and frequent episodes of ventricular tachycardia. Laboratory studies revealed a cardiac troponin I of 12 ng/mL (normal <0.1 ng/mL) and C-reactive protein (CRP) of 76 mg/dL (normal <3 mg/dL). His ECG showed sinus rhythm with incomplete right bundle branch block and symmetric negative T waves in V1-V6. Transthoracic echocardiography revealed nondilated chambers, severe left ventricular dysfunction with ejection fraction of 25% (normal: 50-75%) with hypokinesia of multiple segments, and no pericardial effusion. Cardiac catheterization showed no angiographically significant coronary lesions. Six specimens were obtained from the right ventricle via endomyocardial biopsy and the microscopic appearance of one of them is shown in the attached figure. Serum polymerase chain reaction showed positivity for Coxsackie viruses and further analysis confirmed IgM

Secondary myocarditis

A two weeks old male infant, born preterm at the 35th week, presented with systolic murmur at the lower left sternal border. Transthoracic echocardiography demonstrated a moderate ventricular septal defect, 1 cm in diameter. Infant's parents refused the surgical treatment. About 20 years later the same patient was admitted with shortness of breath, generalized cyanosis, clubbed fingers, and severe edema of the lower extremities. Chest X-ray showed cardiomegaly. Transthoracic echocardiography documented an enlarged right atrium and right ventricle. Cardiac catheterization showed a right ventricular systolic pressure of 42 mm Hg (normal: <30 mm Hg) and a pulmonary to systemic cardiac output Qp/Qs of 2 (normal: 1). Her oxygen saturation in the left ventricle was 73% (normal: 95-99%). Which of the following would most likely explain this patient's clinical and laboratory findings?

Shunt reversal

Referring to the previous question, which of the following histopathologic types of coronary lesions would most likely be responsible for Mr. Martin's chest discomfort? A) complete rupture of the fibrous cap with occlusive coronary thrombosis B) coronary microvascular dysfunction C) erosion of the fibrous cap with a non-occlusive thrombus D) large lipid-core and a thin fibrous cap E) small lipid-core and a thick fibrous cap

Small lipid-core and a thick fibrous cap

A 44-year-old man presented with chief complaint of an ulcer in the tip of the left great toe. The patient reported a six-month history of left calf pain that occurred after walking about two blocks on flat ground. The pain was triggered at a shorter distance when walking at a faster pace or uphill and is relieved after resting for a few minutes. Physical examination revealed a cold and pale left foot that was without palpable pulse. Angiograms showed segmental occlusions in the anterior and posterior tibial and peroneal arteries, with corkscrew collaterals surrounding the obstruction. The aorta, iliac, femoral and popliteal arteries did not demonstrate any abnormalities. Which of the following risk factors is most likely related to this patient's condition?

Smoking

A 62-year-old man was referred to the hepatology clinic for evaluation of elevated levels of liver enzymes. His serum aspartate aminotransferase (AST) level was 385 U/L (normal: 0-35 U/L) and alanine aminotransferase (ALT) level was 356 U/L (normal: 4-36 U/L). The patient reported no symptoms except for a cutaneous reddish lesion on his forehead, about 3 cm in diameter, shown in the attached figure. He had been previously told that the lesion was fungal in origin (tinea corporis), and a course of local antifungal treatment had been administered, with no improvement. Laboratory studies revealed infection with hepatitis C virus and a viral load of 4,920,000 IU per milliliter. Cirrhosis was diagnosed on liver biopsy. This patient's cutaneous lesion most likely represents:

Spider angioma

A three-month-old boy was brought by his mother because of a large area of discoloration on left side of his neck. On physical examination, a 7 × 10 cm reddish-brown tumor was noted at the posterior triangle of the left side of the neck. The results of the laboratory studies and chest x-ray were with the normal range. His developmental milestones were up to date and there were no signs of systemic involvement. Biopsy of the tumor revealed masses of plump, rapidly dividing endothelial cells with and without lumens. The pathological diagnosis was infantile hemangioma. Which of the following would most likely be the natural history of this patient's untreated vascular tumor?

Spontaneous resolution

A 40-year-old man is referred to the hypertensive unit with a 6-year history of mild essential hypertension. Physical examination reveals a well-appearing individual who does not look acutely sick. The first measurement of his blood pressure is 164/100 mm Hg, and the second after 15 minutes is 160/97 mm Hg. Physical examination of systems is unremarkable. Results of laboratory studies are within normal limits: serum creatinine is 0.9 mg/dL (normal: 0.7-1.4 mg/dL), hemoglobin is 14.2 g/dL (normal: 12-16 g/dL), glucose is 92 mg/dL (normal: 70-100 mg/dL) and urinalysis is negative for proteinuria and hematuria. Based on recommendations of American College of Cardiology/American Heart Association (ACC/AHA) guidelines, this individual has which of the following?

Stage 2 Hypertension

A 17-year-old mentally disabled boy presented with a three-month history of tonic-clonic seizures and hemiparesis of the right side of the body. On physical examination, a purple skin discoloration was present on his right face since birth. The birthmark extended from the middle of forehead and involved the eye, half of his nose, cheek, philtrum, and left side of his upper lip till the angle of mouth from where it extended to the left ear. The lower lip and lower jaw were not involved. Brain MRI revealed leptomeningeal angiomatosis along the surface of the right parietal and occipital lobes. The right lateral ventricle was wider compared to the left lateral ventricle. Which of the following is most likely to explain this patient's clinical and imaging

Sturge-Weber syndrome

A 3-year-old boy presented with bluish discoloration, and growth retardation. The child was detected to have a cardiac disease on the third month of age when cyanosis became apparent. Patient's cyanosis increased on crying and did not improve on 100% oxygen inhalation. His apex beat was within midclavicular line at the fifth intercostal space. A systolic thrill was present along the left sternal border at the upper left intercostal space. A systolic ejection murmur was heard along the left sternal border. Laboratory studies revealed hemoglobin of 18 mg/dL and hematocrit of 69% (normal: 32-42%). The leukocyte and platelet counts were within the normal range. A chest x-ray revealed a normal-sized heart with upturned cardiac apex due to right ventricular hypertrophy, concave pulmonary arterial segment, and decreased pulmonary vascularity. Which of the following is the most likely diagnosis?

Tetralogy of Fallot

Mrs. Miller was diagnosed with carcinoid syndrome in 2009, when she was 48 years old. At that time, the diagnosis was confirmed by laparoscopy and liver biopsy, which showed the presence of a primary neuroendocrine tumor of intestine and left hepatic lobe metastases. A small bowel resection was performed combined with left hepatic lobe resection. In 2010, in addition to symptoms of carcinoid syndrome (facial flushing and severe diarrhea), she presented with dyspnea on mild exertion. At physical examination, a 3/6 systolic ejection murmur was observed at the left lower sternal border. Laboratory studies revealed a serum level of 5-hydroxytryptamine of > 1000 ng/mL (normal: <230). Transthoracic echocardiography revealed moderate tricuspid regurgitation. Her tricuspid valve was replaced with a bioprosthetic valve. Which of the following gross and histopathologic appearances of resected cardiac valves would most closely

Thick plaques of smooth muscle cells lining the valve leaflets' surface

A 66-year-old obese man with type 2 diabetes mellitus presented with 1 week of fever, cough, chest pain, and difficulty walking. On physical examination, his left lower extremity was edematous and very painful to palpation from the mid-posterior thigh to the toes. Peripheral pulses were weak in the affected limb. A chest CT scan demonstrated bilateral pulmonary nodules consistent with septic pulmonary emboli. Blood cultures grew methicillin-resistant Staphylococcus aureus and remained positive for 12 days. The patient was treated with vancomycin. Despite treatment with appropriate antimicrobials, he remained intermittently febrile for 3 weeks. On hospital day 25, CT scan of the lower extremities revealed an enhancing fluid collection extending the full length of the left quadriceps muscle, consistent with pyomyositis. Surgical drainage revealed Gram positive cocci in clusters on Gram stain. The patient defervesced af

Thrombophlebitis

An 85-year-old woman was admitted presenting with a two-day history of progressively increasing right upper quadrant abdominal pain, associated with nausea. Laboratory studies revealed a leukocyte count of 17,000 cells/mm3 (normal: 4,000-10,000 cells/mm3) and C-reactive protein of 11 mg/dL (normal: <1 mg/dL). Abdominal CT scan findings were consistent with acute cholecystitis, a thickened gallbladder wall and a single, large gallstone. The patient underwent cholecystectomy. On post-operation day 3, she suddenly developed a high fever and her right upper extremity was found to be more edematous than her left. At this time, her leukocyte count was elevated to 20,800 cells/mm3 and her C-reactive protein was 48 mg/dL. The central venous catheter was immediately removed, and two sets of blood culture were obtained. Both blood cultures were positive for Staphylococcus aureus. She was treated with intravenous dicloxacillin.

Thrombophlebitis

A 40-year-old man presented to emergency department with a swollen, tender left leg. An ultrasound scan revealed deep vein thrombosis of the femoral vein with extension up to the iliac veins. Antithrombotic therapy was started with intravenous heparin and warfarin. His signs and symptoms gradually resolved over the next few days. Three days after being discharged from the hospital he presented with swelling of the right arm and violaceous discoloration of fingers. An ultrasound scan revealed thrombosis of the axillary vein. Warfarin therapy was continued at an increased dosage. The patient was admitted to a hospital for further investigations. A chest X-ray showed an ill-defined rounded density in the left mid-lung field. Chest CT scan revealed a left upper lobe nodule with left hilar adenopathy, and a small left pleural effusion. CT-guided fine needle biopsy of the pulmonary lesion showed features consistent with a

Trousseau syndrome

A 59-year-old Mrs. Bradshaw presented to emergency room with ongoing chest discomfort for 45 minutes that has been unrelieved by three sublingual nitroglycerin tablets. She has been suffering from increasing chest discomfort for the past two years. At first she only noticed the discomfort when she experienced extreme exertion, such as running for the bus, and these symptoms always promptly resolved when she stopped or slowed down. About 9 months ago, she noted that the chest pain episodes had become more frequent, occurring perhaps once or twice per week and precipitated by stress, either physical (walking up and down stairs at home with the laundry) or mental (especially after a day's work at her job in a government office), but again always resolved with rest. When the chest discomfort episodes began to occur more than once per week, she was persuaded to visit her primary care physician. Although she smoked for alm

Unstable angina

An 11-year-old girl presented to dyspnea on exertion. Cardiac auscultation documented a grade II/IV systolic murmur at the cardiac apex. Her blood pressure was 110/70 mm Hg. Transthoracic echocardiography revealed a congenital heart defect. The representative gross appearance of the child's heart is shown. The diagnosis is: A) aortic stenosis B) mitral stenosis C) VSD D) ASD E) aortic coarctation

Ventricular septal defect

A 10-year-old girl was hospitalized with chief complaint of worsening dyspnea on exertion. Her vital signs at admission included a blood pressure of 130/68 mm Hg and heart rate of 90 beats/min. A grade III/VI diastolic murmur was audible at the 4th left sternal border without jugular venous distention or peripheral edema. Chest x-ray showed an enlarged cardiothoracic ratio of 60% (normal: 42-50%). An echocardiogram showed severe aortic insufficiency. Acute rheumatic heart disease was diagnosed, and the patient underwent emergent aortic valve replacement surgery. Which of the following intraoperative appearances will most closely characterize rheumatic valvular involvement?

Verrucae near the free edge of aortic cusps

A 5-year-old boy was hospitalized because of fever (38°C, 100.4°F) and systemic skin maculopapular eruption. On day 2 of illness, conjunctival hyperemia, redness of the lips, and cervical lymphadenopathy emerged. Laboratory studies revealed leukocytosis accompanied by a left shift, and elevated platelet count and erythrocyte sedimentation rate. He was given intravenous immunoglobulin. After that, his fever subsided, and the skin rash started to disappear. Serial echocardiograms revealed a mild dilation of the left anterior descending artery with good biventricular function. Coronary angiography demonstrated an aneurysm in the mid segment of left anterior descending artery about 3.7 cm long and about 10 mm in diameter. Which of the following is the most likely underlying mechanism of this patient's condition?

Viral infection

A 75-year-old woman presented with headache of six weeks' duration. Pain was predominantly over the right side of the head, with the maximum being over the right temple. Pain was excruciating in intensity. She also experienced pain in her muscles of mastication on chewing and was unable to finish a meal where meat required chewing. About two weeks after the onset of headache, he developed blurred vision in the right eye. Laboratory studies revealed an erythrocyte sedimentation rate of 72 mm/hour (normal: 0-15 mm/hour) and C-reactive protein of 4.6 mg/dL (normal: <0.3 mg/dL). Which of the following is the most likely diagnosis?

giant cell arteritis

A 75-year-old man collapsed suddenly in the street. Emergency services attended and started cardiopulmonary resuscitation on site, eventually achieving return of spontaneous circulation. The patient experienced another cardiac arrest in the emergency room at the hospital and was given thrombolytic treatment, but he died shortly thereafter. According to the patient's relatives, he had a past medical history of ischemic heart disease. The autopsy revealed a 20-mm-long thrombus lodged in the right coronary artery associated with severe atherosclerosis in the same segment. The posterior wall of the left ventricle showed a sharply demarcated infarcted area with a largest diameter of 8 cm. The microscopic appearance of this area is shown in the attached figure. Based on the histologic findings, what is the approximate age of the posterior myocardial infarction in this patient? A) <6 hours B) 6 to 24 hours C) 2 to 3 days

2 to 3 days