Patho Block 4 Quiz
A 40 year old patient comes to the ER because he claims he is not clotting and has been continually bleeding form an area where he was pocked with a small needle. Patient denied that he is diabetic and said he does not even have a family history of diabetes. During physical assessment, patient hand was cold and clammy, he lost a lot of weight, shortness of breath, muscular weakness, pain or tenderness n bones or joints and spleen enlargement. Lab test of the blood sample revealed abnormally high number of WBC. Patient diagnosis was Leukemia? Which of the following is best form of therapy for this patient A. Maintenance therapy B. Consolidation therapy C. Induction therapy D. Only A and B E. All of the above
All of the above The goal of treatment for leukemia is to destroy the leukemia cells and allow normal cells to form in your bone marrow. Treatment decisions are based on the kind of leukemia you have, its stage, and your age and general health. Most treatment plans for acute lymphoblastic leukemia (ALL) have 3 steps. These are induction, consolidation, and maintenance.
3-year-old boy is brought by his parents to the emergency department because they are concerned that he has had a high fever for several days. On examination, the boy has conjunctival and oral erythema. He has palpable cervical lymphadenopathy and ery thema of his palms and soles. What is a potential life-threatening complication of this disorder? (A) Aneurysm of the abdominal aorta (B) Aneurysm of the coronary arteries (C) Dissection of the thoracic aorta (D) Ischemia and gangrene of the extremities (E) Rupture of a berry aneurysm
Aneurysm of the coronary arteries The patient presents with mucocutaneous lymph node syndrome, or Kawasaki disease. It is often a self-limiting condition, although as many as 20% of patients develop an aneurysm of the coronary vessels. Dissection of the thoracic aorta is associated with hypertension. Rupture of a berry aneurysm can result in a subarachnoid hemorrhage. Aneurysm of the abdominal aorta is associated with atherosclerosis. Ischemia and gangrene can be a result of Buerger disease.
A 72 year old female with no significant past medical history passes out while exercising. He has intermittent exertional chest pains and dyspnea on exertion as well. Her physical examination reveals a III/VI late-peaking creshendo-decreshendo murmur at the right upper sternal border and a III/VI holosystolic murmur at the apex. Her S2 heart sound is very soft and her carotid upstroke is weak and delayed. Which of the following is most likely causing her symptoms? A) Aortic valve regurgitation B) Aortic valve stenosis C) Mitral valve regurgitation D) Mitral valve stenosis E) Mitral valve prolapse
Aortic valve stenosis Aortic valve stenosis (AS) presents with one of the classic triad: syncope (passing out), exertional angina, or exertional dyspnea (from heart failure). Over the age of 70 the most likely cause is degenerative calcific aortic stenosis while under the age of 70 a bicuspid aortic valve is the likely culprit. Rheumatic heart disease is the third leading cause. Physical examination reveals a crescendo-decrescendo murmur at the aortic listening post (right upper sternal border) which radiates to the carotids. The more severe the aortic stenosis the later the peak of the murmur in systole and the softer the A2 component of the S2 heart sound.
A 30-year-old man presents with fever and sore throat of 2 days' duration. He reports several months of increasing fatigue and exertional dyspnea, as well as easy bruising. Examination reveals tachycardia, evidence of tonsillopharyngitis, and scattered ecchymoses. Which of the following is the correct diagnosis? A. Aplastic anemia B. Vitamin B12 Deficiency C. Folate Deficiency D. HIV Infection
Aplastic anemia AA typically presents with symptoms and signs referable to peripheral cytopenias. There are no pathognomonic presenting features. The finding of other abnormalities on examination, in particular splenomegaly, should suggest an alternative diagnosis. It is important to look for features that may suggest an inherited marrow failure syndrome, such as younger age or short stature. Pigmentation abnormalities (most classically cafe au lait spots), hearing defects, macrocytosis, urogenital abnormalities, or solid tumors occurring at an unusually young age suggest Fanconi anemia. Nail malformations, reticular rash, oral leukoplakia, epiphora, pulmonary fibrosis, cirrhosis, osteoporosis, premature hair loss or graying, esophageal strictures, or extensive dental caries or loss may suggest dyskeratosis congenita. Exocrine pancreatic insufficiency, liver abnormalities, and skeletal dysplasia occur in Shwachman-Diamond syndrome. Finally, a family history of the above abnormalities may also suggest an inherited marrow failure syndrome.
A 48 year old man presents to his primary care physician with complaints of lower leg pain that occurs after he walks a few city blocks and is relieved with rest. He has no other complaints. His blood pressure is 165/ 86 mmHg, his pulse is 83/ min, and his respiratory rate is 18/min. After further questioning he admits to smoking two packs of cigarettes per day. Which of the following types of vessels is most likely involved in the pathologic process surrounding this patient's symptoms? A. Arteries B. Arterioles C. Capillaries D. Veins E. Venules
Arteries The patient is presenting with intermittent claudication. Combined with his history of smoking, this points to peripheral arterial disease, which is often the presenting sign of atherosclerosis. Peripheral atherosclerosis targets areas of high turbulence typically found at branching arterial sites; most common sites are the abdominal aorta and iliac arteries, femoral and popliteal arteries (the cause of the calf pain) and tibial and peroneal arteries. Medical therapy with antiplatelet drugs such as aspirin has moderate success, with surgical revascularization reserved for severe cases. Risk factors for atherosclerosis include smoking, hypertension, diabetes mellitus, hyperlipidemia, and positive family history.
A 68-year-old woman visits her primary care physician with a complaint of unilateral left calf pain and cramping after moderate exercise. These symptoms began several weeks ago. The woman has a history of type II diabetes mellitus. She has never smoked and drinks only rarely and never to excess. Medications are metformin and divided dose regular and long-acting insulin. Her vital signs are normal. Physical examination is significant for dependent rubor and decreased anterior tibial and dorsalis pedis pulses in the left lower extremety. A pulsatile mass is appreciated in the left subsartorial region. What is the most probable underling pathophysiologic basis for this patient's condition? A. Atherosclerosis B. Embolic phenomena C. Focal necrosis of ectopic bursal cysts D. Intimal fibrosis
Atherosclerosis Taking into consideration pulsatilla mass( aneurism which is common in atherosclerosis ) and involvement popliteal artery atherosclerosis fits better. Arteriosclerosis which involve smaller arterioles will lead to Diabetic/HTNsive nephropathy and lacunae infarcts.
A 56-year-old has not received any medical care nor seen a physician for years. He reports reduced exercise tolerance over the past 5 years. On occasion in the past year he has noted chest pain after ascending a flight of stairs. He smokes 2 packs of cigarettes per day. He is found to have a blood pressure of 155/95 mm Hg. His body mass index is 30. Laboratory findings include a total serum cholesterol of 245 mg/dL with an HDL cholesterol that is 22 mg/dL. Which of the following vascular abnormalities is most likely to be his most serious health risk? A Lymphedema B Atherosclerosis C Deep venous thrombosis D Plexiform arteriopathy
Atherosclerosis He has multiple risk factors for atherosclerosis, including his weight, smoking, hypertension, and high total cholesterol with low 'good' HDL cholesterol. His findings suggest coronary artery disease.
A 4-year-old man presents with increasing fatigue, easy bruising and the diffuse formation of petechiae. His peripheral leukocyte count is increased, and multiple immature cells are present. A bone marrow biopsy is markedly hypercellular as a result of a diffuse proliferation of immature cells. The presence of which of the following abnormalities in these immature cells favors the diagnosis of AML rather than ALL? A. Auer rods B. Howell-Jolly bodies C. Pappenheimer bodies D. Reinke crystals E. Russell bodies
Auer rods The two basic types of acute leukemias are AML and ALL. It is important to differentiate between these two malignancies because their therapies are different. AML is characterized by the proliferation of myeloblasts, whereas ALL is characterized by the proliferation of lymphoblasts. Myeloblasts may be larger than lymphoblasts, but the key distinguishing cytologic feature is the presence in myeloblasts of Auer rods, which are distinctive red-staining rodlike structures within the cytoplasm. Auer rods are a feature of AML but not of ALL.
A 67 year old female with a history of breast cancer and tobacco use complains of dizziness and dyspnea on exertion. Her heart sounds are distant and her systolic blood pressure is noted to markedly decrease with inspiration. Which of the following is the likely diagnosis? A) Restrictive cardiomyopathy B) Mitral valve regurgitation C) Congestive heart failure D) Pulmonary embolus E) Cardiac tamponade
Cardiac tamponade Cancer is the most common cause of pericardial effusion and when enough fluid accumulates in the pericardial space, cardiac tamponade occurs. "Pulses paradoxus" is when there is a decrease in systolic blood pressure during inspiration due to failure of the right ventricle to accept the normal increased venous return that occurs with inspiration. This also results in a "Kussmal's sign" or elevated jugular venous distension during inspiration (normally the opposite occurs). Treatment is with emergent pericardiocentesis.
A 42 year old man is seen because of a long history of slowly developing congestive heart failure. His blood pressure is normal. Coronary artery angiography reveals no vascular disease. No heart murmurs are heard. The white blood cell count, differential, and erythrocyte rate are normal. The most likely diagnosis is? A. Carcinoid heart disease B. Cardiomyopathy C. Coarctation of the aorta D. Constrictive pericarditis E. Myocardial Infarction
Cardiomyopathy Cardiomyopathies are noninflammatory myocardial disorders that are not associated with coronary artery obstruction, hypertension, valvular disease, congenital heart disease, or infectious disease. They are most often characterized by otherwise unexplained ventricular dysfunction, such as cardiac failure, ventricular enlargement, or ventricular arrhythmias.
A 61 year old man had a myocardial infarction 1 year ago, which was the first major illness in his life. He now wants to prevent another myocardial infarction and is advised to begin a program of exercise and to change his diet. A reduction in the level of which of the following serum laboratory findings 1 year later would best indicate the success of this diet and exercise regimen? (A) Cholesterol (B) Glucose (C) Potassium (D) Renin (E) Calcium
Cholesterol Reducing cholesterol, particularly LDL cholesterol, with the same or increased HDL cholesterol level, indicates a reduced risk of atherosclerotic complications
A 53 year old woman presents with dyspnea on exertion orthopnea, paroxysmal nocturnal dyspnea, edema in the legs and feet, and fatigue. She has no history of angina, other sings of coronary artery disease, hypertension, or valvular disease. Echocardiography reveals cardiomegaly, with four chamber hypertrophy and dilation. Which of the following is the most likely diagnosis? A. Congestive or dilated cardiomyopathy B. Hypertrophic cardiomyopathy C. Myocarditis D. Restrictive Cardiomyopathy
Congestive or dilated cardiomyopathy Congestive or dilated cardiomyopathy is the most common form of cardiomyopathy. It is characterized by four chamber hypertrophy and dilation as well as right and left sided severe heart failure.
A 55-yr-old unmarried, African American man presents to the doctor's office complaining of chest pain with radiation to his left arm. He has high cholesterol and hypertension. He is a cigarette smoker and is considered obese based on his height-to-weight ratio. His daily activities include reading the newspaper while rocking in his chair and eating hamburgers while smoking throughout the day. He also watches T.V a lot and dreads having to leave the house, but when he does his favorite event is getting something from McDonald's. This patient may be experiencing pain due to which of the following condition? A). Nicotine Addiction B). Coronary Artery Disease C). Type 1 Diabetes D). Pulmonary Embolism E). Giant Cell Arteritis
Coronary Artery Disease The risk factors for Coronary Artery Disease (CAD) include age over 45 years in men and over 55 years in women. Family history of early heart disease proves to be a significant factor. High blood cholesterol levels (specifically, low-density lipoprotein cholesterol), high blood pressure, lack of physical activity, obesity, and cigarette smoking are also risk factors. Typical angina symptoms such as substernal pressure-like chest pain with/without radiation to the jaw or left arm may be present in CAD. Less typical symptoms include dyspnea, diaphoresis, back pain, neck pain, nausea, fatigue and palpitations.
A 27-year-old HIV+ man presents with fever, waxing and waning mental status, and hematuria. Blood analysis shows thrombocytopenia and hemolytic anemia, but a normal prothrombin time and partial thromboplastin time. Which of the following is the most likely basis for the pathogenesis of these findings? A. Decreased activity of plasma metalloproteinase B. Abnormal sequestration of platelets in the spleen C. Immune-mediated platelet destruction D. Drug-induced antibody binding of platelets E. A lack of von Willebrand factor protein
Decreased activity of plasma metalloproteinase The findings describe the presentation of thrombotic thrombocytopenic purpura (TTP). The pathogenesis of TTP is related to a deficiency of, or antibodies to, a plasma metalloproteinase, called ADAMTS13, which cleaves the ultrahigh molecular weight multimers of von Willebrand factor (vWF) produced by endothelial cells into smaller multimers. The ultrahigh molecular weight multimers of vWF initiate platelet aggregation and thrombosis.
A 12-year-old white girl is brought to the emergency room by her parents due to 12 hours of rapidly worsening nausea, vomiting, abdominal pain, and lethargy. Over the last week she has felt excessively thirsty and has been urinating a lot. Physical examination reveals a lean, dehydrated girl with deep rapid respirations, tachycardia, and no response to verbal commands. Her blood test revealed abnormal insulin levels. A. Diabetes Mellitus B. Atherosclerosis C. Embolic phenomena D. Arteriosclerosis
Diabetes Mellitus The rate of beta-cell destruction varies in diabetes. In some patients, there may be a slow destruction leading to gradual onset of symptoms that is clinically indistinguishable from type 2 diabetes. This patient's are in accordance with type 1 diabetes.
A 65-year-old man who has a long history of hypertension presents to the emergency department with tearing chest pain that radiates to the back. An electrocardiogram is normal, as are cardiac enzymes. A "stat" chest radiograph demonstrates widening of the mediastinum. Which of the following is the most likely? (A) Arteriovenous fistula (B) Atherosclerotic aneurysm (C) Berry aneurysm (D) Dissecting aneurysm (E) Syphilitic aneurysm
Dissecting aneurysm The clinical presentation of a dissecting aortic aneurysm mimics that of a myocardial infarction; however, electrocardiographic changes and increased concentration of cardiac enzymes are notably absent. The mediastinum is often widened by radiographic examination. Although there is an association with hypertension and disorders of connective tissue (e.g., Marfan syndrome and Ehlers-Danlos syndrome) , there is no association with atherosclerosis. The presentation exemplified by this scenario is a true surgical emergency!
A 4-year-old boy presents to the urgent care center with 3 days of fever, sore throat and malaise. The mother tells you he is usually a healthy child and he is up to date on all of his immunizations. His temperature is 39.5˚C (103.1˚F). On physical exam, he is noted to have mild conjunctivitis, a peeling rash on his extremities, one 2cm lymph node on the right anterior cervical chain, a confluent truncal rash and swollen hands and feet. His tongue is noted to be bright red. Labs are remarkable for an absolute neutrophilic leukocytosis, left-shift, and an elevated ESR. Platelets are normal. Appropriate management is taken. The most long term management of this child is A. Antibiotic prophylaxis to prevent rheumatic fever B. Echocardiograms to look for coronary artery aneurysms C. Excisional biopsy and surveillance of lymph nodes for malignancy D. Nothing, as this is a case of scarlet fever and he will completely recover.
Echocardiograms to look for coronary artery aneurysms Kawasaki disease is a childhood disease that occurs in young children < 5 years old. It is often characterized by at least 4 of the following: Acute phase: 1-2 weeks of the following symptoms high fever (> 5 days above 104˚F) conjunctivitis (bilateral, nonexudative, painless) polymorphous, truncal rash cervical adenopathy (unilateral) strawberry tongue with cracked lips erythema/edema of the palms/soles desquamation of the hands and feet Subacute phase follows resolution of fever, usually lasts 2-3 weeks and includes: elevated ESR thrombocytosis coronary artery aneurysms (up to 40%, if untreated) Chronic phase begins when all clinical symptoms are resolved; persist until ESR returns to normal. Treatment of choice is Aspirin and Immunoglobulin. KD is the leading cause of heart disease in children. Key test is an echogram to detect coronary artery aneurysms.
An autopsy done on a 45 year old obese man reveals evidence of an atheroma formation. The gross appearances of the aortas are recorded and compared with microscopic findings of atheroma formation. Which of the following is most likely to be the first visible gross evidence for the formation of an atheroma? A Thrombus B Fatty streak C Calcification D Hemorrhage
Fatty streak This is the first sign. It is benign and reversible, but it may be the precursor to more severe plaques.
In the third match of a volleyball tournament, a 15yearold girl jumps up for a block and collapses. Despite cardiopulmonary resuscitation, she cannot be revived. She had been healthy all her life and complained only of limited episodes of chest pain in games during the current school year. Which of the following pathologic findings of the heart is the medical examiner most likely to find? (A) Haphazardly arranged hypertrophied septal myocytes (B) Extensive myocardial hemosiderin deposition (C) Tachyzoites within foci of myocardial necrosis and inflammation (D) Mitral valvular stenosis with left atrial enlargement (E) Large, friable vegetations with destruction of aortic valve cusps
Haphazardly arranged hypertrophied septal myocytes Hypertrophic cardiomyopathy is the most common cause of sudden unexplained death in young athletes. There is asymmetric septal hypertrophy that reduces the ejection fraction of the left ventricle, particularly during exercise. Histologically, haphazardly arranged hypertrophic myocardial fibers are seen
A mother brought her 2 years old child to the physician's office reporting a red lumpy growth on the side of his face, resembling a raspberry. The mother claimed the child had the growth from birth and it is getting bigger. Upon palpation the growth felt lumpy and firm, the child did not react to pain. What is the most likely cause of this? A. Kaposis Sarcoma B. Contact dermatitis C. Hemangiomas D. Cancer
Hemangiomas
A mother brings her 5-year old boy to the clinic because of a rash on his legs and buttocks that she noticed this morning. He has also been complaining that is "belly hurt," but has no change in appetite. He had upper respiratory tract infection and sore throat about a week ago. He has not had any fevers, recent weight loss or joint pain, and has not taken any medications. His temperature is 37°C (98.6°F). Physical examination shows mild periumbilical tenderness and multiple 3-6mm raised erythematous lesions on his lower extremities and buttocks. The lesions do not blanch with pressure. His leukocyte count, hemoglobin, platelet count and coagulation studies are normal. Urinalysis shows 3-5 RBCs per hpf. A rapid strep test is positive. The most likely diagnosis is A. Idiopathic thrombocytopenic purpura B. Henoch-Schonlein purpura C. Kawasaki disease D. Rock Mountain Spotted Fever
Henoch-Schonlein purpura HSP is a small vessel vasculitis (leukocytoclastic angiitis) associated with IgA and C3 immune complex deposition in the blood vessels of the skin, joints, GI, and kidneys. It presents in children (ages 3-10) with colicky abdominal pain, a typical dependent purpuric rash on the lower extremities, and polyarthralgia, often in the large joints of the lower extremities. It is often related to recent infection, especially upper respiratory infections. Diagnosis is based on clinical criteria from the American College of Rheumatology. If uncertainty exists, a skin biopsy demonstrating leukocystoclastic vasculitis with IgA deposits is conclusive. Treatment is supportive as the disease is usually self-limited though controversy about use of steroids exists. Glomerulonephritis and potentially ESRD are long-term complications in some adult patients.
The mother asked you as the physician I am worry that my child will be disfigured. How can I prevent that, and you reply: A. The child can be cured by surgery B. There is no cure C. It will most likely disappear by age 5 D. We can remove it and follow by chemotherapy
It will most likely disappear by age 5
A 40 year old patient comes to the ER because he claims he is not clotting and has been continually bleeding form an area where he was pocked with a small needle. Patient denied that he is diabetic and said he does not even have a family history of diabetes. During physical assessment, patient hand was cold and clammy, he lost a lot of weight, shortness of breath, muscular weakness, pain or tenderness n bones or joints and spleen enlargement. Lab test of the blood sample revealed abnormally high number of WBC. What could be a possible diagnosis? A. Non-Hodgkin Lymphoma B. Leukemia C. B-Cell Lymphoma D. Mantle Cell Lymphoma E. None of the above
Leukemia Leukemia or leukaemia is a group of cancers that usually begin in the bone marrow and result in high numbers of abnormal white blood cells. These white blood cells are not fully developed and are called blasts or leukemia cells. Symptoms may include bleeding and bruising problems, feeling tired, fever, and an increased risk of infections. These symptoms occur due to a lack of normal blood cells. Diagnosis is typically made by blood tests or bone marrow biopsy.
A 52-year-old man with end-stage renal disease presents to his renal clinic with a hemoglobin concentration of 8 g/dL and an absolute reticulocyte count of <25,000 cells/mm3. His WBC count is 11,000/mm3 and the differential is within normal limits. A peripheral blood smear shows hypochromic, microcytic RBCs. Iron staining of a bone marrow aspirate shows macrophages positive and RBCs negative for iron within the cytoplasm. Which of the following is the most likely cause of this patient's condition? (A) An overdose of tacrolimus (B) Anti-Rh D immunoglobulin (C) Iron deficiency (D) Low erythropoietin (E) Myelodysplastic syndrome (F) Tissue plasminogen activator
Low erythropoietin Erythropoietin is a hormone produced in the kidney, and hence low levels are seen in the setting of kidney failure. Low levels of erythropoietin could cause anemia, which this patient is experiencing. Erythropoietin acts by increasing RBC proliferation and differentiation in the bone marrow. Lack of erythropoietin causes a microcytic anemia with iron sequestration in the bone marrow, as shown on the slide. Human recombinant erythropoietin (epoetin) is used in patients with severe anemias (hemoglobin <11 g/dL), which often occur in the setting of renal failure.
A 41-year-old woman presents with increasing fatigue, lethargy, and muscle weakness. Her CBC reveals decreased numbers of erythrocytes, leukocytes, and platelets along with an increase in the MCV of the erythrocytes. The associated picture is from a smear of her peripheral blood. The abnormal neutrophil seen in this picture is most likely to be seen in an individual with what type of anemia? A. Fanconi anemia B. Leukoerythroblastic anemia C. Megaloblastic anemia D. Myelophthisic anemia E. Sideroblastic anemia
Megaloblastic anemia In contrast to a normal, mature neutrophil, which has from two to five nuclear lobes, the neutrophil shown has at least six lobes and is an illustration of neutrophilic hypersegmentation. Granulocytic hypersegmentation is significant and among the first hematologic findings in the peripheral blood of patients who have megaloblastic anemia in its developmental stages. Neutrophilic hypersegmentation is generally considered a sensitive indicator of megaloblastic anemia, which can be caused by a deficiency in vitamin B 12 , in folate, or in both. In contrast, Fanconi anemia is an autosomal recessive disorder characterized by bone marrow failure combined with certain birth defects, such as abnormalities of the radius. Leukoerythroblastic anemia refers to any space occupying lesion of the bone marrow (myelophthisic anemia) that causes immature red blood cells and immature white blood cells to appear in the peripheral blood. Finally sideroblastic anemia is characterized by the presence of numerous sideroblasts in the bone marrow and may be caused by a deficiency of pyridoxine or it may be a form of myelodysplasia.
One week ago, a 72-year-old woman had an episode in which she became disoriented, had difficulty speaking, and had weakness on the right side of the body. On physical examination, she is afebrile with pulse of 68/min, respirations of 15/min, and blood pressure of 130/85 mm Hg. On auscultation, the lungs are clear, the heart rate is irregular, and there is a midsystolic click. An echocardiogram shows nodular deposits with the density of calcium around the mitral valve. One leaflet of the mitral valve appears to balloon upward. The ejection fraction is estimated to be 55%. Laboratory findings show Na•, 141 mmoi/L, K•, 4.1 mmoi/L, Cl-, 98 mmoi/L, C02, 25 mmoi/L, glucose, 77 mg/dL, creatinine, 0.8 mg/dL, calcium, 8.1 mg/dL, and phosphorus, 3.5 mg/dl. Which of the following is the most likely diagnosis? (A) Carcinoid heart disease (B) Hyperparathyroidism (C) Infective endocarditis (D) Infiltrative cardiomyopathy (E) Mitral annular calcification (F) Rheumatic heart disease (G) Senile calcific stenosis
Mitral annular calcification Mitral annular calcification is often an incidental finding on chest radiograph, echocardiograph, or at autopsy. Larger accumulations of calcium in the mitral ring can impinge on the conduction system, however, causing arrhythmias or disrupting the endocardium to provide a focus for thrombus formation (which can embolize and cause a "stroke," as in this patient) or infective endocarditis. Some cases are associated with mitral valve prolapse. Carcinoid heart disease leads to endocardial and valvular collagenous thickening. Hyperparathyroidism can cause metastatic calcification, which usually does not involve the heart, and deposits would not be so focal, this patient does not have hypercalcemia. Infective endocarditis is a destructive process, and healing may lead to fibrosis, but not to nodular calcium deposition. The most common infiltrative cardiomyopathies are hemochromatosis and amyloidosis. Rheumatic heart disease can lead to scarring with some calcium deposition, but the valve leaflets undergo extensive scarring, with shortening and thickening of the chordae that preclude upward prolapse. Senile calcific stenosis involves the aortic valve; in this case, there is no evidence of stenosis.
A 29 year old male with a history of ectopia lentis presents for a routine physical examination. He is noted to have pectus excavatum, pes planus, a high arched palate, and a positive wrist and thumb sign. Which of the following cardiac disorders is associated with his condition? A) Aortic valve stenosis B) Coarctation of the aorta C) Mitral valve prolapse D) Ventricular septal defect E) Ebstein's anomaly
Mitral valve prolapse Marfan's syndrome is associated with mitral valve prolapse and aortic aneurysms. Specifically, the ascending aorta may dilated and predispose patient's to acute aortic dissection which can be fatal. Also, when the ascending aorta dilates, the aortic valve annulus stretches causing the valve leaflets to fail to coapt which results in aortic regurgitation. Aortic valve stenosis (A) is not associated with Marfan's syndrome and is caused be either senile calcific degeneration of the valve or from a congenital bicuspid aortic valve. Coartaction of the aorta (B) is associated with Turner's syndrome and presents with hypertension in the upper extremities and hypotension in the lower extremities. "Rib notching" is seen on the chest x-ray. Ventricular septal defects (D) and Ebstein's anomaly (E) are not associated with Marfan's.
A 76 year old female with a history of coronary artery disease and prior inferior wall myocardial infarction presents with gradual onset of increased shortness of breath on exertion and lower extremity swelling. Her physical examination reveals a IV/VI holosystolic murmur at the 5th intercostals space at the midclavicular line. Which of the following is likely the cause of her symptoms? A) Tricuspid valve regurgitation B) Mitral valve regurgitation C) Ventricular septal defect D) Mitral valve stenosis E) Left ventricular aneurysm
Mitral valve regurgitation Mitral regurgitation occurs as a complication of an inferior wall myocardial infarction due to papillary muscle dysfunction resulting in failure of the mitral valve leaflets to coapt normally. Recall the cardiac anatomy of the mitral valve, specifically that there are two papillary muscles, the anterolateral and posteromedial. The anterolateral papillary muscle is perfused by the left anterior descending AND the left circumflex coronary arteries, thus dysfunction of the anterolateral papillary muscle is uncommon (since it would require 2 major artery occlusions). The posteromedial papillary muscle receives its sole blood supply from the right coronary artery (which also supplies the inferior wall in 80% of people). Thus a right coronary artery occlusion resulting in inferior wall myocardial infarction frequently causes mitral regurgitation due to concomitant papillary muscle infarction. Rarely, rupture of a papillary muscle can cause acute mitral regurgitation and cardiogenic shock which requires emergent surgical correction.
A 26-year-old man has had a fever with nonproductive cough for the past 10 weeks. On examination, his temperature is 37.4° C. A chest radiograph shows a 4-cm left upper lobe nodule. CBC shows hemoglobin, 13.3 g/dL; hematocrit, 40.5%; platelet count, 281,000/mm3; and WBC count, 13,760/mm3 with 38% segmented neutrophils, 2% bands, 45% lymphocytes, and 15% monocytes. What is the most likely diagnosis? A) Acute lymphoblastic leukemia/lymphoma B) Hodgkin lymphoma, lymphocyte rich type C) Mycobacterium tuberculosis granuloma D) Myelodysplastic syndrome E) Staphylococcus aureus abscess
Mycobacterium tuberculosis granuloma This granulomatous infection has led to leukocytosis with lymphocytosis and monocytosis. Blood monocytes become tissue macrophages that evolve into epithelioid cells and giant cells of granulomatous inflammation. The most consistent form of leukocytosis from infection is neutrophilia with acute bacterial infections, such as Staphylococcus aureus infection. Viral, mycobacterial, and fungal infections produce less consistent peripheral blood findings. An acute lymphoblastic leukemia is likely to be accompanied by a higher WBC count with circulating lymphoblasts. Hodgkin lymphomas have no consistent peripheral blood findings and are not likely to produce solitary lung nodules. Myelodysplastic syndromes are marked by the presence of immature myeloid cells and cytopenias in the peripheral blood.
A 14-year-old boy is brought by his family to your clinic in Utah with a long history of recurrent epistaxis. His father also reports such a history. Of the following, which is the most likely condition consistent with these findings? (A) Cavernous hemangioma (B) Glomangioma (C) Hemangioendothelioma (D) Osler-Weber-Rendu syndrome (E) Varicose veins
Osler-Weber-Rendu syndrome The hemorrhagic phenomena in this patient likely represent recurrent rupture of the convolutions of venules and capillaries in the mucous membranes of the nose, as occurs in the Osler-Weber-Rendu syndrome (hereditary hemorrhagic telangiec tasia) . This disorder is inherited as an autosomal dominant condition and also affects the vessels of the gastrointestinal tract. Characteristic lesions can often be seen on the lips, conjunctivae, and mucous membranes of the nose or mouth. Cavernous hemangioma is a common lesion. On occasion, it can be seen as a component of the von Hippel-Lindau syndrome. A glomangioma is a benign tumor of blood vessels on the fingers or toes. A hemangioendothelioma is a rare malignant tumor of blood vessels. Varicose veins are a manifestation of incompetency of the valves in the superficial veins of the legs of older patients.
A 60-year-old male with a history of hypertension and hepatitis B presents to his primary care physician complaining of several months of fever, weakness, myalgias, and arthralgias. Home medications include lisinopril. Figure A shows biopsy findings from the mesenteric vasculature. No evidence of involvement of microscopic vessels, pulmonary vessels, or glomerulonephritis is found. Which of the following is the most likely diagnosis? A. Microscopic Polyangiitis B. Polyarteritis Nodosa C. Churg-Strauss Syndrome D. Granulomatosis with Polyangiitis (also know as Wegener's Granulomatosis) E. Giant cell arteritis
Polyarteritis Nodosa The patient's symptoms, medical history, and biopsy findings are consistent with polyarteritis nodosa (PAN). PAN is a vasculitis of medium sized-vessels involving the kidney, nervous system and GI tract. On the USMLE it is often paired with a history of hepatitis B or HIV. Diagnosis is made by biopsy of involved tissue or via mesenteric angiography. Hypertension, mononeuritis multiplex, and livedo reticularis are common in affected patients. PAN affects medium or small arteries. Notably, it does not cause glomerulonephritis or vasculitis in arterioles, capillaries, or venules. Figure A shows the circumferential infiltrate of polymorphonuclear leukocytes and resulting intimal proliferation typical of PAN. Answer 1: Microscopic polyangiitis is distinguished from PAN by the presence of microscopic vessel involvement, especially skin, lung, and glomerular vessels. 70% of patients are ANCA positive Answer 3: Churg-Strauss syndrome is notable for eosinophilic and granulomatous inflammation often involving the respiratory tract. Answer 4: Granulomatosis with polyanginitis is distinguished from PAN by pulmonary involvement. 90% of patients with active disease are ANCA positive. Answer 5: Giant cell (temporal) arteritis is a large-vessel vasculitis of arteries above the clavicles.
A 29-year-old African woman, living in Canada for 3 years, presented with 3 weeks of dysphagia, cervical lymphadenopathy, and a productive cough that failed to respond to 2 courses of antibiotics. She complained of progressive chest heaviness, dyspnea, orthopnea, fever, chills, night sweats, generalized weakness, nausea, and vomiting. She denied arthralgia, myalgia, rashes, or joint pain. Her past history included remote tuberculosis, successfully treated; multiple hereditary osteochondromatosis; and eosinophilic pneumonia. Previous investigations did not find a satisfactory etiology for the eosinophilia. She had no history of atopy or asthma. What is diagnosis? A). Primary Hypereosinophilic B). Parasitic infection C). Malignant disease D). Secondary Hypereosinophilic
Primary Hypereosinophilic The hypereosinophilic syndrome was clearly described in 1968 by Hardy and Anderson. The incidence of hypereosinophilic syndrome (HES) is estimated at 1 to 2 cases per 200 000 people per year. Whites are affected more than blacks, and men more often than women (9:1). The condition typically affects individuals aged 20 to 50 years. Hypereosinophilic syndrome may affect any organ system. Eosinophils release toxic substances, including eosinophil-derived neurotoxin, cationic protein, major basic protein, reactive oxygen species, and arachidonic acid derivatives. These may cause endothelial and myocyte damage, resulting in thrombosis, fibrosis, and infarction.
A 28-year-old man is brought to the emergency department with shock that developed over the past 12 hours. On physical examination, his temperature is 38.6° C, pulse is 101/min, respirations are 26/min, and blood pressure is 80/40 mm Hg. Needle tracks are noted in the left antecubital fossa. Crackles are heard over the lower lung fields. CBC shows hemoglobin, 14.1 g/dL; hematocrit, 42.6%; MCV, 93 μm3; platelet count, 127,500/mm3; and WBC count, 12,150/mm3 with 71% segmented neutrophils, 8% bands, 14% lymphocytes, and 7% monocytes. The neutrophils show cytoplasmic toxic granulations and Döhle bodies. Which of the following is the most likely diagnosis? A) Acute myelogenous leukemia B) Chronic myelogenous leukemia C) Infectious mononucleosis D) Pneumocystis jiroveci pneumonia E) Pseudomonas aeruginosa septicemia F) Pulmonary Mycobacterium tuberculosis
Pseudomonas aeruginosa septicemia Toxic granulations, which are coarse and dark primary granules, and Döhle bodies, which are patches of dilated endoplasmic reticulum, represent reactive changes of neutrophils that are most indicative of overwhelming inflammatory conditions, such as bacterial sepsis. The route of infection in this case is injection drug use. Infectious mononucleosis is accompanied by an increase in "atypical" lymphocytes. Leukemia, granulomatous infections, or viral infections do not cause toxic changes in neutrophils.
A 65-year-old Caucasian male presents to the emergency room with chest pain. Coronary angiography reveals significant stenosis of the left anterior descending (LAD) artery. Which of the following characteristics of the atheroma is the most important physiologically plausible predictor of myocardial necrosis in this patient? A. Cholesterol crystal presence B. Rate of formation C. Calcium content D. Presence of cytokines E. Amount of foam cells
Rate of formation A slow-forming atheroma allows for formation of collateral circulation, thereby lowering the chance of myocardial infarction (MI) and necrosis. The most important factor as to whether or not a coronary artery plaque will cause an ischemic MI, is the rate in which the artery is occluded. If occluded slowly, there is time for the development of new arterial collaterals which are protective from myocardial ischemia. These collateral vessels provide blood flow to hypoperfused area of cardiac tissue distal to the point of occlusion. Conversely, with rapid coronary occlusion, there is not enough time for collateral development and thus myocardial ischemia is inevitable.
A 32-year-old woman returns to her physician for follow-up of hypertension that has been poorly controlled in spite of numerous antihypertensive medications. It is decided to evaluate the patient for possible "secondary" hypertension. Which of the following is a well-known cause of secondary hypertension? (A) Ethnicity (B) Obesity (C) Renal artery stenosis (D) Smoking (E) Stress
Renal artery stenosis The great majority of cases of hypertension are classified as essential hypertension. Essential hypertension results from the interactions of predisposing determinants and exogenous factors, including family history, ethnicity, stress, obesity, sodium intake, smoking, and physical activity. A small minority of cases of hypertension are due to secondary causes. Unilateral renal artery stenosis is a secondary cause that is typically correctable by surgery.
A 6-month-old boy with no previous medical problems presents with fever and painful swelling of the hands and feet. His parents are concerned because he has been inconsolable for 6 hours. The infant has been refusing bottles and has needed fewer diaper changes over the last 2 days. What is the most likey diagnosis? A. B12 deficiency B. Sickle Cell Anemia C. Avascular Necrosis D. Perthes Disease
Sickle Cell Anemia Most cases are diagnosed through neonatal screening programs. Very young children may present with jaundice, hemolysis, or splenic sequestration crisis. For children older than 4 months, presentation may include swelling of the joints, especially dactylitis, leukocytosis in the absence of infection, protuberant abdomen (often with umbilical hernia), cardiac systolic flow murmur, and maxillary hypertrophy with overbite. For all patients, acute painful episodes and hemolysis are characteristic of the disease. Patients with hemoglobin SC disease may have fewer painful episodes but are at higher risk of retinal hemorrhage. In patients born before widespread newborn screening was available, this can be the first presentation of the disease.
A 10-year-old boy with jaundice, splenomegaly, and chronic normocytic, normochromic anemia with an abnormal osmotic fragility test and increased mean corpuscular hemoglobin concentration presents to the clinic for a consult. He has had these symptoms for most of his life, and his parents are requesting a definitive cure. Which of the following confers a definitive cure for this patient's disease? (A) Blood transfusions (B) Bone marrow transplant (C) Folate therapy (D) Iron therapy (E) Splenectomy
Splenectomy The patient is exhibiting symptoms of a hemolytic anemia because he is anemic and has jaundice, which results from the breakdown of RBCs leading to the formation of excess bilirubin. Moreover, his anemia is normochromic and normocytic with an abnormal osmotic fragility test and increased mean corpuscular hemoglobin concentration (MCHC). These are all characteristics of hereditary spherocytosis, an intrinsic, extravascular hemolysis due to spectrin or ankyrin defect. RBCs are small and round with no central pallor. The osmotic fragility test detects hemolysis by measuring the fraction of hemoglobin released from RBCs at progressively more dilute salt concentrations. It detects hemolysis in spherocytes at salt concentrations that do not affect normal RBCs. The MCHC measures the concentration of hemoglobin in a given volume of packed RBCs and is elevated only in hereditary spherocytosis. The definitive treatment of hereditary spherocytosis is splenectomy because the spleen is responsible for the breakdown of the defective RBCs in this disease. Once eliminated, the spleen no longer hemolyze the RBCs, and thus, the anemia improves.
A 60-yr-old man appears to the emergency room confused and having trouble speaking. He has a headache and is experiencing dizziness. Evaluation reveals numbness of the right side of his face and of his right arm. The patient is overweight, has high blood pressure and is known to drink heavily. The attending physician suspects that this man may have experienced which of the following? A). Conversion Disorder B). Systemic Infection C). Vertigo D). Stroke E). Seizure
Stroke Stroke is the 4th leading cause of death in the US, with one person dying every 4 minutes as a result. Strokes occur due to problems with the blood supply to the brain; either the blood supply is blocked or a blood vessel within the brain ruptures. A stroke is a medical emergency, and treatment must be sought as quickly as possible. Stroke is also more likely to affect people if they are overweight, aged 55 or older, have a personal or family history of stroke, do not exercise much, drink heavily or use illicit drugs. Strokes occur quickly, and as such their symptoms often appear suddenly without warning. The main symptoms of stroke are as follows: confusion, including trouble with speaking and understanding; headache, possibly with altered consciousness or vomiting; numbness of the face, arm or leg, particularly on one side of the body; trouble with seeing, in one or both eyes; and trouble with walking, including dizziness and lack of coordination.
A 72 year old woman has a 1 month history of left-sided jaw pain when chewing food, headache, fever, and fatigue. Laboratory studies reveal elevated erythrocyte sedimentation rate. Which of the following arteries is most likely involved? A. External carotid artery B. Facial artery C. Ophthalmic artery D. Post auricular artery E. Superficial temporal artery
Superficial temporal artery Individual is likely suffering from giant cell (temporal) arteritis (CCA), the most common systemic vasculitis. CCA, which affects large to small arteries, typically presents in people >50 years old and in more common in women. Patients commonly present with constitutional symptoms (anorexia, fatigue, weight loss) unilateral temporal or occipital headache with overlying scalp tenderness, jaw claudication, and impaired vision. The superficial temporal artery is the most common affected artery in CCA. On biopsy, affected arteries are characterized by nodular thickening that reduces the size of the lumen, granulomatous inflammation with mononuclear and giant cells, and fragmentation of internal elastic membrane. CCA is treated with high dose corticosteroids to reduce inflammation rapidly and prevent blindness.
A 41-year-old male has had several bouts of pneumonia in the past year. He now complains of vague abdominal pain and a dragging sensation. Physical examination reveals marked splenomegaly. A complete blood count shows a hemoglobin concentration of 8.2 g/dl, hematocrit of 24.6%, MCV of 90 fL, WBC count of 2400/uL, and platelet count of 63,000/uL. On the periphal blood smear are many small leukocytes with reniform nuclei and pale blue cytoplasm with threadlike extensions. Immunohistochemical staining shows that they mark with CD20. Which of the following laboratory test findings is most characteristic of this disease? A. Tartrate-resistant acid phosphatase in leukocytes B. Presence of Auer rods in leukocytes C. Presence of Ph1 chromosome D. Presence of toxic granulations in neutrophils E. Monoclonal IgM in serum
Tartrate-resistant acid phosphatase in leukocytes This patient has hairy cell leukemia, an uncommon neoplastic disorder of B cells (CD20+). These cells infiltrate the spleen and marrow. Pancytopenia results from poor production of hematopoietic cells in the marrow and sequestration of the mature cells in the spleen. There are two characteristic features of this disease: the presence of hairy projections from neoplastic leukocytes in the peripheral blood smear and tartrate-resistant acid phosphatase in the neoplastic cells.
A 50-year-old man has sudden onset of severe substernal chest pain that radiates to the neck. On physical examination, he is afebrile, but has tachycardia, hyperventilation, and hypotension. No cardiac murmurs are heard on auscultation. Emergent coronary angiography shows a thrombotic occlusion of the left circurnfex artery and areas of 50% to 70% narrowing in the proximal circurnfex and anterior descending arteries. Which of the following complications of this disease is most likely to occur within 1 hour of these events? (A) Ventricular fibrillation (B) Pericarditis (C) Myocardial rupture (D) Ventricular aneurysm (E) Thromboembolism
Ventricular fibrillation In the period immediately after coronary thrombosis, arrhythmias are the most important complication and can lead to sudden cardiac death. It is believed that, even before ischemic injury manifests in the heart, there is greatly increased electrical irritability. Pericarditis and rupture occur several days later. An aneurysm is a late complication of healing of a large transmural infarction; a mural thrombus may fill an aneurysm and become a source of emboli. If portions of the coronary thrombus break off and embolize, they enter smaller arterial branches in the distribution already affected by ischemia.
