Term
A 51 year old male has noted right hip pain for months. A radiograph reveals a 10 x 13 cm mass involving the ischium of the pelvis. The mass has irregular borders and there are extensive areas of bony destruction along with some scattered calcifications. The lesion is resected, and grossly the mass has a bluish-white cut surface. The most appropriate statement regarding this lesion is that it is:
A The most frequent primary tumor of bone
B Usually seen in distal skeletal bones
C More common in females
D Sometimes seen to arise in benign cartilagenous tumors
E Associated with Paget's disease of bone |
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Definition
| Chondrosarcomas can occur in association with osteochondromatosis.d |
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Term
An 18 year old female presents with pain and swelling of her right distal thigh associated with activity. She notices tenderness after performing physical exercise. She has no history of any trauma. Radiographs show an expansile, eccentric, lytic lesion located in the metaphysis of the distal femur that is surrounded by a rim of reactive new bone as a host response. What is this lesion most likely to be:
A Fibrous dysplasia
B Ewing's sarcoma
C Chondrosarcoma
D Osteosarcoma
E Aneurysmal bone cyst |
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Definition
The major differential diagnosis is giant cell tumor of bone.e?
V. Aneurysmal bone cyst (ABC).
A. Deforms the bone in aneurysmal fashion; metaphyseal location.
B. Produces pain and/or swelling clinically. Can fracture.
C. May occur on top of another bone tumor.
D. Histologically consists of hemorrhagic cysts with giant cells & cellular fibroblastic tissue with surrounding “eggshell” of new woven bone.
E. Most frequent in first two decades.
ddx also would include giant cell ca. |
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Term
A 23 year old male notes some minor discomfort over the lateral aspect of his right knee after a day working in a cramped cubicle at work. There is a palpable "bump" in this region. A radiograph of the knee reveals a lateral projection from the metaphysis of the lower femur. The lesion is excised and is composed of a 3 cm stalk of bony cortex capped by cartilage. This lesion is probably a(an):
A Osteochondroma
B Giant cell tumor
C Aneurysmal bone cyst
D Osteoid osteoma
E Enchondroma |
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Definition
They are benign and slow-growing. They may cause problems because they project outward from the bone.a
II. Osteochondroma (exostosis) – most common benign bone mass.
A. Most common in young males. Not a true neoplasm, but a projection of the cartilaginous growth plate.
B. Most are solitary, on long bones (primarily femur) and composed of a bony stalk capped by cartilage. Most are less than 4 cm thick.
C. If multiple, may be hereditary termed osteochondromatosis.
D. Rarely may develop chondrosarcoma in a persistent cartilaginous cap. |
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Term
A 75 year old male has complained of lower back pain for years. As part of a workup for a persistently elevated lymphocytosis, he has a right posterior iliac crest biopsy performed. Hematopoiesis is normal, but the bone spicules are thickened with irregular cement lines forming a mosaic appearance with both increased osteoblastic and osteoclastic activity. Which of the following conditions is most likely to occur in conjunction with this disease process:
A Dementia
B Myelofibrosis
C Hepatic cirrhosis
D Chronic renal failure
E Congestive heart failure |
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Definition
| He has Paget's disease of bone, and this is the classic phase of mixed osteolysis and osteogenesis. The increased blood flow into the involved areas of bone can lead to high output congestive heart failure in older persons with minimal cardiac reserve. Paget's disease of bone is a disease of older Caucasians.e |
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Term
A 21-year-old male college student has noted increasing pain and soreness in his left shoulder after a summer working on the ranch and bucking bales of hay while at a ranch up in the Cache Valley. He can hardly raise his left arm. Clear fluid is aspirated from the subacromial region. He probably has:
A Tenosynovitis
B Costochondritis
C Bursitis
D A ganglion cyst
E Pseudogout |
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Definition
| Synovial-lined bursal sacs become inflamed and distended with watery fluid, more often in males engaging in excessive manual labor or exercise.c |
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Term
Over the past few days, a 15 year old male has developed marked pain and swelling in his left hip that makes movement difficult. A joint aspirate from the left hip reveals cloudy fluid that demonstrates numerous neutrophils but no crystals. This condition is LEAST likely to:
A Complicate gonorrheal infection
B Complicate septicemia
C Involve large joints
D Lead to ankylosis with chronicity
E Occur with Lyme disease |
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Definition
| The arthritis of Lyme disease is often seen in stages II or III and is not typically purulent.e |
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Term
A young boy has complained of pain in the region of the right upper thigh for several weeks. This pain is worse at night and is relieved by aspirin. A radiograph reveals a small lucent area in the proximal femoral cortex surrounded by sclerotic bone. The most likely diagnosis is:
A Enchondroma
B Multiple myeloma
C Eosinophilic granuloma
D Osteoid osteoma
E Osteochondroma |
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Definition
There is a central nidus with surrounding sclerotic bone. They are very painful from release of prostaglandins.d
I. Osteoid osteoma--”oma” (tumor) of the “osteo” (bone) producing “osteoid” (bone-like matrix).
A. Seen in first two decades, male predominance (3:1); in diaphysis or metaphysis.
B. Painful, waking from sleep at night, responsive to aspirin.
C. Pathologic appearance is that of a central nidus of irregular well vascularized osteoid surrounded by a shell of sclerotic bone; no more than 1.5 cm size.
D. When located near a joint may produce arthritic symptoms.
E. It is important to extirpate the nidus to stop the pain.
F. Osteoblastoma is a similar looking lesion pathologically, but is much larger and more aggressive in radiographic appearance, resembling osteosarcoma. |
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Term
An adult patient with a 10 kg weight loss in the past two months now has extensive bone pain. A nuclear medicine scan reveals areas of focally increased uptake ("hot spots") in the pelvis, vertebrae, and ribs. All of the following are likely primary sites to be considered EXCEPT:
A Adenocarcinoma of colon
B Oat cell carcinoma of lung
C Infiltrating ductal carcinoma of breast
D Renal cell carcinoma
E Adenocarcinoma of prostate |
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Definition
| The other primary tumors listed metastasize to bone far more frequently.a |
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Term
A 49-year-old female has noted that she cannot completely extend the middle finger of her left hand. In the palm of her hand at the base of this finger there is an ill-defined mass that is very firm to palpation. What histologic appearance will this mass most likely demonstrate:
A Fibromatosis
B Rhabdomyosarcoma
C Granulomatous inflammation
D Dystrophic calcification
E Giant cell tumor of tendon sheath |
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Definition
| This is a so-called Dupuytren's contracture due to a palmar fibromatosis. The etiology is obscure.a |
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Term
A 55-year-old patient with a history of poorly controlled diabetes mellitus has a serum urea nitrogen of 65 mg/dL and serum creatinine of 7.0 mg/dL. He develops increasing bone pain in his extremities over several months. He may have all of the following findings EXCEPT:
A Enlarged parathyroid glands
B Hyperostosis
C Aluminum toxicity
D Hyperphosphatemia
E Hypocalcemia |
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Definition
| Hyperostosis does not occur with renal osteodystrophy. |
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Term
A 10 year old girl complains of joint pain, only involving her knees and ankles, that occurs with and without movement. The arthralgias are accompanied by a fever. Physical examination reveals no joint deformities. She has generalized lymphadenopathy. These findings are most likely to be associated with:
A Gonococcal arthritis
B Ankylosing spondylitis
C Juvenile rheumatoid arthritis
D Tuberculous arthritis
E Gout |
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Definition
| Also known as Still's disease, JRA is not frequent. About 5% of rheumatoid arthritis cases occur in persons <16 years of age.c |
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Term
| A 30 year old male has pain about his left knee that he has noted worsening "for weeks". A radiograph reveals a 7 cm lytic lesion involving the epiphyseal region of the distal femur with a "soap bubble" appearance. |
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Definition
| A giant cell tumor typically arises in the epiphysis. |
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Term
A 38 year old male has increasing dyspnea with peripheral edema. A chest radiograph reveals that the heart appears to fill the chest. A chest CT scan demonstrates a 10 cm mass involving the right ventricle that appears to have hemorrhage and necrosis. Which of the following neoplasms is most probable, given these findings:
A Rhabdomyosarcoma
B Mesothelioma
C Myxoma
D Angiosarcoma
E Papillary fibroelastoma |
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Definition
This is a rare neoplasm (all cardiac neoplasms are rare) but the size alone suggests a malignancy.d
Angiosarcoma - highly malignant, bulky tumor. Morphology varies widely. |
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Term
A 25 year old previously healthy woman has worsening fatigue with dyspnea, palpitations, and fever over the past week. Her vital signs on admission to the hospital show T 38.9 R 30 P 105 and BP 95/65. Her heart rate is slightly irregular. An ECG shows diffuse ST-T segment changes. A chest x-ray shows mild cardiomegaly. An echocardiogram shows slight mitral and tricuspid regurgitation but no valvular vegetations. Her troponin I is 12 ng/mL. She recovers over the next two weeks with no apparent sequelae. Which of the following laboratory test findings best explains the underlying etiology for these events:
A Anti-streptolysin O titer of 1:512
B Total serum cholesterol of 537 mg/dL
C Coxsackie B serologic titer of 1:160
D Blood culture positive for Streptococcus, viridans group
E ANCA titer of 1:80 |
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Definition
| She has findings that suggest myocarditis, which can have features of cardiomyopathy. One of the most likely organisms is Coxsackie B virus. |
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Term
A 44 year old male has had no major medical problems throughout his life, except for arthritis pain involving all extremities for the past couple of years. He has had worsening orthopnea and pedal edema in the past six months. He is afebrile. There is no chest pain. A chest radiograph shows cardiomegaly with both enlarged left and right heart borders, along with pulmonary edema. Laboratory test findings include sodium 139 mmol/L, potassium 4.3 mmol/L, chloride 99 mmol/L, CO2 25 mmol/L, urea nitrogen 18 mg/dL, creatinine 1.3 mg/dL, and glucose 167 mg/dL. Which of the following additional laboratory test findings is he most likely to have:
A Spherocytes on his peripheral blood smear
B Hemoglobin of 10.7 g/dL with MCV of 72 fL
C Erythrocyte sedimentation rate of 79 mm/Hr
D Anti-centromere antibody titer of 1:320
E Serum ferritin of 8700 ng/mL |
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Definition
E) CORRECT. He has findings of a cardiomyopathy with right and left heart failure. Hereditary hemochromatosis can produce iron deposition in visceral organs, including the heart. The serum ferritin is a good indicator of body iron stores. Hemochromatosis affects the pancreas as well, leading to diabetes mellitus. Deposition of iron in joints leads to arthritis. The onset of disease is typically in the 40's in males and 60's in females.
(D) Incorrect. Anti-centromere antibody can be detected with sclerodema, which can produce cardiac findings resembling a cardiomyopathy, but hyperglycemia and joint problems are not part of scleroderma |
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Term
A 69 year old female with a 20 kg weight loss over the past 6 months now has developed painless jaundice. She is found to have a large mass involving the head of the pancreas, along with widespread nodules in the liver, by abdominal CT scan. Nodules are seen in the lungs by chest radiograph. The most likely cardiac complication to be present in her is:
A Dilated cardiomyopathy
B Non-bacterial thrombotic endocarditis
C Acute fibrinous pericarditis
D Endocardial fibrosis
E Acute myocardial infarction |
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Definition
CORRECT. Such cancers can be associated with a hypercoagulable state (Trousseau's syndrome) with formation of marantic cardiac valvular vegetations.B
(C) Incorrect. Fibrinous pericarditis is most often seen with renal failure and uremia. It can also occur with myocardial infarction and with acute rheumatic fever. |
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Term
A 51 year old woman has had several syncopal episodes over the past year. Each episode is characterized by sudden but brief loss of consciousness. A brain MRI scan shows a 1.5 cm cystic area in the left parietal cortex. Vital signs show T 36.8 R 20 P 80 and BP 110/75. A chest radiograph shows no cardiac enlargement, and her lung fields are normal. She has no chest pain. She has no pedal edema. Her serum total cholesterol is 165 mg/dL. Which of the following cardiac lesions is she most likely to have:
A Cardiac amyloidosis
B Left atrial myxoma
C Tuberculous pericarditis
D Mitral valve prolapse
E Ischemic cardiomyopathy |
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Definition
| CORRECT. Atrial myxomas are more often on the left. Though benign, they can occlude the mitral valve and produce sudden loss of cardiac output. They may embolize small portions of themselves or thrombus forned over their surface.B |
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Term
| A 25-year-old male dies suddenly and unexpectedly while at a Beverly Hills nightclub late one evening. Autopsy reveals no evidence for trauma. THere are no gross pathologic findings of internal organs. Postmortem toxicologic findings are significant for high blood levels of cocaine and its metabolite benzoylecgonine. The most likely histopathologic finding in heart will be: |
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Definition
| Contraction band necrosis. This finding has been associated with sudden death and cocaine use. It is thought that it may be mediated by high norepinephrine levels. |
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Term
If more people were eating oat bran cereal instead of bacon for breakfast the incidence of which of the following problems would decrease the most:
A Mitral annulus calcification
B Ventricular aneurysm
C Left atrial dilation
D Thoracic aortic aneurysm
E Aortic valve stenosis |
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Definition
CORRECT. This is a complication of myocardial infarction. The incidence of MI is increased with an atherogenic diet.B
(D) Incorrect. Most thoracic aortic aneurysms are a consequence of cardiovascular syphilis. |
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Term
A 35-year-old female dies suddenly. At autopsy, one of her cardiac valves demonstrates attenuation of the chordae tendineae, with rupture of one, while the leaflets show myxomatous change. The most likely cause of death is:
A Carcinoid heart syndrome
B Mitral valve prolapse
C Rheumatic heart disease
D Infective endocarditis
E Acute myocardial infarction |
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Definition
| The valve leaflet can balloon upward. This condition can be associated with Marfan's syndrome.B |
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Term
Cardiovascular syphilis is most likely to be manifested by all of the following findings EXCEPT:
A Complications of syphilitic aortitis occur weeks following initial infection.
B The media of the thoracic aorta is mainly affected.
C Patients may have signs and symptoms of aortic regurgitation.
D Aortic aneurysms may develop and contain mural thrombus.
E The causative infectious agent is Treponema pallidum |
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Definition
| Cardiovascular syphilis manifests decades after the original infection.A |
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Term
A 66 year old male has chronic renal failure with a serum urea nitrogen of 100 mg/dl and creatinine of 9.8 mg/dl. Auscultation of the chest reveals a friction rub. He is most likley to have a pericarditis that is termed:
A Fibrinous
B Hemorrhagic
C Purulent
D Serous
E Constrictive |
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Definition
The uremia leads to exudation of fibrin onto the epicardial and pericardial surfaces.A
(B) Incorrect. This is more typical of tuberculosis or metastatic tumor.
(C) Incorrect. This is not common but can occur with spread of infection from lung or mediastinum.
(D) Incorrect. This is more typical for collagen vascular diseases.
(E) Incorrect. This is a late complication of tuberculous pericarditis. It may also follow cardiac surgery or radiation to the chest. |
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Term
A 56 year old male is found to have a blood pressure of 175/110 mm Hg. A month later his blood pressure is 170/105 mm Hg. He elects to do nothing about this, because he feels fine. He is at greatest risk for development of:
A Polyarteritis nodosa
B Pulmonary passive congestion
C Hyaline arteriolosclerosis
D Tricuspid insufficiency
E Giant cell myocarditis |
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Definition
CORRECT. The pressure load on the left ventricle leads to left venricular hypertrophy, but eventually the heart can no longer compensate and there is left heart failure, which leads to pulmonary congestion.B
(C) Incorrect. Hyaline arteriolosclerosis can be seen in some cases of benign hypertension. |
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Term
| Atrophy of caudate nucleus and gliosis |
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Definition
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Term
During radical prostatectomy, the anesthesiologist reports a drop in blood pressure in a 73-year-old male. The hypotension persists for 30 minutes despite intervention with pressor agents. The most likely consequence of this prolonged hypotensive episode is development of:
A Hydranencephaly
B Linear parasaggital infarction
C Lacunar infarcts of basal ganglia
D Parietal lobe hemorrhagic infarct
E Anterior pituitary necrosis |
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Definition
CORRECT. The most sensitive areas of the brain to ischemia will be the 'watershed' areas between anterior and middle cerebral arterial circulations.B
C) Incorrect. Lacunar infarcts are typically a consequence of hypertensive cerebrovascular disease. |
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Term
A 28 year old male undergoes induction chemotherapy for acute myelogenous leukemia. He becomes severely pancytopenic, with a WBC count of 1320/microliter, Hgb 7.9 g/dL, and platelet count of 72,000/microliter. He becomes comatose. A CT scan of the head reveals a right parietal hemorrhage. He dies a day later. At autopsy, he is found to have a right middle cerebral arterial thrombosis with right parietal hemorrhagic infarction. Which of the following infectious agents is most likely to produce these findings:
A Herpes simplex virus
B Toxoplasma gondii
C Human immunodeficiency virus
D Cytomegalovirus
E Aspergillus niger |
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Definition
| Aspergillus loves to invade blood vessels and produce thrombosis. Neutropenia is a risk for aspergillosis.E |
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Term
Following an episode of severe head trauma suffered in a motorcycle accident, an 18 year old female is noted to have decerebrate posturing. Funduscopic examination reveals marked bilateral papilledema. A CT scan reveals changes of marked diffuse cerebral edema. This increase in brain volume because of an increase in sodium and water content is most likely to be severest in which of the following components:
A Neuronal cell bodies
B Meninges
C White matter
D Dura
E Ependyma |
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Definition
The greatest amount of salt and water increase with cerebral edema occurs within white matter.C
(A) Incorrect. Cerebral edema is most marked in white matter. Enlargement of neuronal cell bodies is most marked with storage diseases. |
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Term
| Foci of multnucleated cells and macrophages in grey and white matter |
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Definition
|
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Term
| Periventricular lymphoid aggregates with cells marking with CD19 |
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Definition
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Term
A 23 year old primagravida has a fetal ultrasound performed at 17 weeks gestation. There is no family history of inherited diseases. The pregnancy has been uncomplicated. Which of the following congenital conditions is the most likely to be seen in this setting:
A Anencephaly
B Encephalocele
C Arnold-Chiari malformation
D Lissencephaly
E Holoprosencephaly |
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Definition
| This occurs in about 1 birth per 1000. It is usually not associated with chromosomal abnormalities. It may be associated with maternal folate deficiency.A |
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Term
A 28 year old G3 P2 woman has had an uncomplicated pregnancy. An ultrasound is performed at 16 weeks gestation, and the findings prompt performance of an amniocentesis. The amniotic fluid is found to have an elevated alpha fetoprotein. Which of the following findings seen on ultrasound examination of the fetus is most likely to have been present:
A Holoprosencephaly
B Hydrocephalus
C Encephalocele
D Hydrops fetalis
E Spina bifida |
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Definition
This is a form of neural tube defect in which the occiput is not formed and herniation of brain occurs. Alpha fetoprotein is increased in amniotic fluid or maternal serum.C
(E) Incorrect. Though there is a vertebral defect with spina bifida, there is no open defect to increase the AFP. |
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Term
A 50-year-old African-American male had a blood pressure of 182/108 mm Hg at the last visit to his physician several months ago. He refuses to take any medications. He is admitted to the hospital after suddenly losing consciousness. When he is aroused, he cannot speak and he cannot move his right arm or his right leg. He probably has:
A An embolus to the middle cerebral artery
B A subfrontal meningioma
C Cerebral venous thrombosis
D Alzheimer's disease
E Hemorrhage into the putamen |
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Definition
| The basal ganglia region is the typical location for hypertensive hemorrhages.E |
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Term
A 52 year old male has a history of chronic alcoholism. He is admitted after an episode of trauma in which he fell and hit the back of his head. A head CT scan shows no intracranial hemorrhage. He continues to exhibit decreased mentation and a brain MRI scan is performed that shows anterior vermian atrophy of the cerebellum. Of the following physical examination findings, which would he most likely have:
A Choreiform movements
B Nystagmus
C Truncal and lower limb ataxia
D Tremor at rest that diminishes or disappears with movement
E Short-term memory loss |
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Definition
| Cerebellar disease often manifests with ataxia.C |
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Term
Enlargement of the cerebral ventricles from obstruction to the flow of cerebrospinal fluid is LEAST likely to occur from which of the following conditions:
A Previous meningitis from Pneumococcus
B Forking of the aqueduct of Sylvius
C Epidural hematoma
D Ependymoma
E Intraventricular hemorrhage |
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Definition
| Postmeningitic states more typically lead to communicating hydrocephalus from deficient reabsorption of CSF.A |
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Term
A 48 year old woman has developed chronic renal failure, and a renal scan shows bilaterally enlarged kidneys with multiple cysts. She has the sudden onset of a severe headache. A cerebral angiogram demonstrates marked narrowing of cerebral artery branches near the base of the brain, consistent with vasospasm, but no intraparenchymal hemorrhage is present. Which of the following conditions most likely produced these findings:
A Bacterial meningitis
B Severe atherosclerosis
C Malignant hypertension
D Cerebral edema
E Subarachnoid hemorrhage |
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Definition
| The blood irritates the arteries. This effect is often delayed by several days following the initial hemorrhagic event. Berry aneurysms are located in the circle of Willis at the base of the brain, and rupture leads to extravasation of blood into the subarachnoid space. Her renal scan suggests a diagnosis of dominant polycystic kidney disease (DPKD). About 10% of persons with DPKD develop berry aneurysms. |
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Term
A 41 year old woman had a worsening headache for the past week, along with a fever and increasing obtundation,. A head CT scan reveals a solitary 3 cm diameter lesion with ring enhancement located in the right parietal lobe. A stereotactic biopsy is performed and a frozen section shows granulation tissue with adjacent collagenization, gliosis, and edema. The probable cause for these findings is:
A Chronic brain abscess
B Aspergillosis
C Progressive multifocal leukocencephalopathy
D Toxoplasmosis
E Rabies |
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Definition
Granulation tissue with fibrosis is a typical reaction to a cerebral abscess. Collagen deposition around a ring enhancing lesion is typical for an abscess that organizes. A common source for such a brain abscess is a lung infection.A
(B) Incorrect. Aspergillus loves to invade blood vessels, leading to hemorrhage and infarction.
D) Incorrect. Toxoplasmosis can produce ring-enhancing lesions, but they do not have granulation tissue. |
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Term
| This storage disease results from lack of sphingomelinase and can lead to severe neurologic impairment in children. There is a mild adult form of the disease. |
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Definition
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Term
| This rare disease has scattered hamartomatous masses scattered in cortex. It is manifested with mental retardation, skin lesions and seizures. |
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Definition
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Term
A 72 year old female has a five year history of worsening mental functioning with trouble remembering things. She has no problems with movement. She is noted on an MRI scan of the brain to have symmetrically increased size of the lateral ventricles along with cerebral cortical atrophy in a mainly frontal and parietal distribution. A lumbar puncture reveals a normal opening pressure, and analysis of the clear, colorless cerebrospinal fluid reveals a glucose and protein which are in normal ranges. Cell count on the CSF shows 3 WBCs (all lymphocytes) and 1 RBC. A funduscopic examination is normal. Which of the following findings is most likely associated with her underlying disease process:
A Loss of gamma aminobutyric acid
B Presence of the e4 allele of apolipoprotein E
C Increased numbers of Lewy bodies
D Perivascular mononuclear inflammation
E Loss of Betz cells |
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Definition
She has findings characteristic for Alzheimer's disease. The neuritic plaques have amyloid cores, and patients may also have a congophilic angiopathy.
(A) Incorrect. She does not have Parkinson's disease.
(D) Incorrect. She does not have multiple sclerosis.
(E) Incorrect. She does not have amyotrophic lateral sclerosis, characterized by loss of muscular movement, but preservation of intellect. |
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Term
A stillbirth occurs at 28 weeks gestation to a 30 year old G2 P1 woman whose previous pregnancy resulted in a normal term birth. At autopsy, the cerebrum of the fetus demonstrates extensive diffuse periventricular areas of necrosis. Which of the following infections is most likely to have caused these findings:
A Taenia solium
B Cytomegalovirus
C Poliovirus
D Candida
E Syphilis |
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Definition
CORRECT. Remember TORCH for congenital infections. The 'T' for toxoplasmosis and the 'C' for cytomegalovirus are most likely to involve the CNS. The extent of necrosis and calcification with cytomegalovirus can be considerable.B
E) Incorrect. Interstitial keratitis of the cornea can occur, and meningo-vascular syphilis is uncommon. |
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Term
| A 73 year old male has exhibited problems remembering things for several months, and he is noted to confabulate. He dies as a consequence of a hepatocellular carcinoma. At autopsy, his brain demonstrates bilaterally small mammillary bodies that show brown discoloration. Microscopically, there is gliosis and vascular proliferation and hemosiderin deposition. These findings are most typical for: |
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Definition
| Wernicke's disease can also lead to hemorrhage and/or loss of periaqueductal grey matter. The Wernicke-Korsakoff syndrome is seen with chronic alcoholism. The mechanism may have to do with thiamine deficiency. (Micronodular cirrhosis with alcoholism is a risk for hepatocellular carcinoma.) |
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Term
| von Recklinghausen disease |
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Definition
| Another name for Nuerofibromatosis |
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Term
| Spinal Muscular atrophy Type I |
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Definition
| "Infantile" Werdnig-Hoffmann disease 0-6 months SMA type I, also known as severe infantile SMA or Werdnig Hoffmann disease, is the most severe, and manifests in the first year of life. This type generally onsets quickly and unexpectedly after birth; babies diagnosed with Type I SMA do not generally live past one year of age. Pneumonia is considered the ultimate cause of death due to deterioration of survival motor neurons; motor neuron death causes insufficient functioning of the major bodily organ systems, particularly respiratory (e.g. breathing, ridding of pooled secretions inside lungs). |
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Term
| Spinal Muscular atrophy Type II |
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Definition
| "Intermediate" or "infantile chronic" 7-18 months Type II SMA, or intermediate SMA, describes those children who are never able to stand and walk, but who are able to maintain a sitting position at least some time in their life. The onset of weakness is usually recognized some time between 6 and 18 months. It is known to vary, some patients gradually grow weaker over time, while others through careful maintenance avoid any progression. Major causes for concern include the Respiratory System, as once weakened it never fully recovers. |
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Term
| Spinal Muscular atrophy Type III |
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Definition
| Type 3: "Juvenile" or "mild childhood and adolescent" Kugelberg-Welander disease >18 months SMA type 3 describes those who are able to walk at some time. Many may later lose this ability, but the designation at type III remains based upon their history. |
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Term
A 45 year old man has had a severe headache for a week. Physical examination reveals papilledema on the right. A head CT scan reveals a marked right to left midline shift. An MRI scan demonstrates a 6 cm enhancing mass lesion in the right parietal region with marked surrounding edema. He develops a dilated pupil on the right and soon thereafter loses consciousness and dies. At autopsy, which of the following lesions is most likely to be found:
A Superior sagittal sinus thrombosis
B Right cerebellar hemispheric hemorrhage
C Pontine hemorrhages
D Thrombosis of the posterior cerebral artery
E Diffuse subarachnoid hemorrhage |
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Definition
| The brain swelling leads to herniation, which damages small perforating vessels and results in Duret hemorrhages in the pons and midbrain.C |
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Term
A 45 year old female noticed tinnitus in her left ear which progressed over some weeks to hearing loss in that ear. On physical examination she is found to have a marked decrease in hearing on the left, with Rinne test indicating air conduction better than bone conduction. The other cranial nerves I - VII and IX - XII are intact. A brain MRI scan revealed a solitary, fairly discreet, 3 cm mass located in the region of the left cerebellopontine angle. Which of the following statements is most appropriate to tell the patient regarding these findings:
A You are unlikely to survive for more than a year
B Remissions and exacerbations are likely to occur in coming years
C Other family members should undergo MR imaging of the brain
D The lesion can be resected with a good prognosis
E A test for HIV-1 is likely to be positive |
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Definition
| The findings are characteristic for schwannoma, called an acoustic neuroma when the 8th cranial nerve is involved. These are benign neoplasms. A solitary mass is unlikely to be part of neurofibromatosis.D |
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Term
A 65 year old male has general paresis with increasing loss of higher mental functions. A VDRL is positive on cerebrospinal fluid obtained by lumbar puncture. The CSF protein and glucose are normal, and there is 1 mononuclear cell present. Which of the following pathologic findings is UNLIKELY to be a feature of his disease:
A Cortical neuronal loss with atrophy
B Hemorrhagic encephalitis
C Chronic meningitis
D Endarteritis
E Atrophy of spinal cord dorsal columns |
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Definition
| This is not a feature of neurosyphilis. Hemorrhagic encephalitis would be more typical of herpes simplex virus infection.B |
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Term
A 50 year old man was noted by his wife to have personality changes over the last year. He was slovenly and didn't appear to take an interest in his work. He became more forgetful. On examination he had frontal release signs and memory loss. He appeared unconcerned about his illness. A 3 cm left frontal lobe mass was found by MR imaging. The mass had areas of calcification. Which of the following diagnoses is most likely to be made on microscopic examination of the mass:
A Thrombosed berry aneurysm
B Oligodendroglioma
C Meningioma
D Schwannoma
E Organizing abscess |
|
Definition
| The location and radiographic changes suggest a glial neoplasm, most likely an oligodendroglioma.B |
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Term
| Dandy-Walker malformation |
|
Definition
| characterized by an enlarged posterior cranial fossa with absent or rudimentary cerebellar vermis replaced by a midline cyst. |
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Term
An infant displays failure to reach developmental milestones. There is a prominent 2 cm lumbar meningomyelocele. An MRI scan of the brain demonstrates downward extension of the cerebellar vermis and displacement of the medulla from a small posterior fossa into the foramen magnum. There is tenting of the tectum of the midbrain. The cerebral ventricles are enlarged. The spinal cord has findings of hydromyelia. Which of the following conditions is most likely to account for these findings:
A Dandy-Walker malformation
B Viral meningoencephalitis
C Arnold-Chiari malformation
D Maternal folate deficiency
E Werdnig-Hoffman disease |
|
Definition
The findings are those of a Chiari type II malformation.C
Type II: Usually accompanied by a myelomeningocele[12] leading to partial or complete paralysis below the spinal defect. Abnormal development of the cerebellar vermis and medulla oblongata occur, and they both descend into the foramen magnum. Hydrocephalus is frequently present.
Type I: A congenital malformation. Is generally asymptomatic during childhood, but often manifests with headaches and cerebellar symptoms. Herniation of cerebellar tonsils. |
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Term
46-year-old female has a severe headache, worsening over the past two months. She now has difficulty moving her left arm. Papilledema is noted, worse on the right. These findings are most likely to be a consequence of:
A Blockage of lymphatics by metastatic tumor
B Overproduction of cerebrospinal fluid
C A neuronal storage disease
D Hyperosmolar coma with diabetes mellitus
E Neovascularization around a glioma |
|
Definition
| The new vessels around a tumor (or abscess) lack an effective blood-brain barrier, leading to cerebral edema.E |
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Term
A 58 year old male has never had headaches before in his life, but over the past 6 weeks he has had increasingly severe, almost constant, generalized headaches. An MRI scan of the brain shows an irregular enhancing mass 8 cm in size centered in the right frontal-parietal region and extending across the corpus callosum, with areas of necrosis. A lumbar puncture is performed, and the CSF reveals a protein of 190 mg/dL, glucose of 68 mg/dL (serum glucose is 100 mg/dL), and a cell count showing 5 WBCs (4 mononuclears and 1 PMN) and 6 RBCs. These findings are most indicative of:
A Glioblastoma multiforme
B Tuberculous meningitis
C Wernicke's disease
D Herpes simplex encephalitis
E Intraparenchymal brain hemorrhage |
|
Definition
One of the causes that must be considered with a high CSF protein is a tumor.A
(B) Incorrect. Tuberculosis is usually associated with increases in WBC's, usually a lymphocytic pleocytosis, though the total count rarely exceeds 500 per microliter. |
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Term
Excisional biopsy of a solitary 1 cm diameter pigmented lesion from the upper back on your 38 year old female patient shows a malignant melanoma that is 2 mm in thickness, with margins clear. These findings most strongly suggests that:
A She will not have to worry further about this lesion
B The lesion has occurred in a non sun-exposed area
C There is a genetic basis for the occurrence of this lesion
D Human papillomavirus infection was present
E There may be metastases within 5 years |
|
Definition
| This is very thick and suggests that there is a much poorer prognosis than a melanoma only 0.75 mm thick (which has a 98% 5 year disease free interval.E |
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Term
A 26-year-old male has pain when he sits down for any length of time. Examination reveals some small pitted areas in the skin at the tailbone above the anal cleft, beneath which is a 1 cm tender, soft mass. This is most probably a(an):
A Pilonidal sinus and abscess
B Carbuncle
C Decubitus ulcer
D Adnexal skin tumor
E Bone spur |
|
Definition
| This is a classic location and description. A sinus tract is lined by squamous epithelium and hair; this becomes secondarily infected to produce an abscess.A |
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Term
A 29 year old man is bothered by a "cold sore" on his upper lip. After a week, he notices the appearance of several rounded, slightly erythematous lesions on the palms of his hands averaging 0.4 to 0.8 cm in size. Each of the lesions has a targetoid appearance with a central dark red area. After a course of acyclovir, the lesions on his hands disappear. Which of the following conditions did he most likely have:
A Erythema multiforme
B Molluscum contagiosum
C Keratoacanthoma
D Impetigo
E Pemphigus vulgaris |
|
Definition
Erythema multiforme is a condition that typically follows an infection, often herpes simplex virus infection. The mechanism by which keratinocyte apoptosis is induced is unclear.A
(B) Incorrect. Molluscum contagiosum is due to infection with a pox virus and is characterized by a firm nodule on the trunk or anogenital region that generally subsides in a month or two. |
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Term
Which of the following statements regarding a nevus or nevi found on the skin of a 32-year-old male is INCORRECT:
A Nevus cells are a morphologic variant of melanocytes.
B Some nevi may not be pigmented.
C Most melanomas arise from nevi.
D Nevi evolve from junctional to compound to intradermal lesions.
E Most Caucasians have at least one nevus. |
|
Definition
| Melanomas arise de novo. Melanomas may arise in patients with dysplastic nevi, but benign nevi are not thought to give rise to melanomas.C |
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Term
A dome-shaped, flesh-colored nodule is present on the cheek of a 55-year-old male. While waiting several months for a referral in his managed care health plan, the lesion disappears. It is most likely a (an):
A Skin tag
B Keratoacanthoma
C Dermatofibroma
D Epidermal inclusion cyst
E Hemangioma |
|
Definition
Keratoacanthomas can enlarge rapidly and mimic a squamous cell carcinoma. Most patients with them are caucasians over 50 years of age.B
(C) Incorrect. These are tan to brown, firm papules that may be slightly tender. They may decrease somewhat in size over time. They dimple inward upon lateral compression.
(D) Incorrect. EIC's are subcutaneous, well-circumscribed, firm, and often movable cystic nodules. They may become painful upon traumatic rupture. |
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Term
| A 39 year old male with a previous diagnosis of cytomegalovirus (CMV) retinitis has noted the appearance of several reddish-purple plaques on his face and right hand. Biopsy reveals that the lesions are composed of a dermal proliferation of atypical spindle cells lining slit-like vascular spaces with extravasated red blood cells. These lesions most likely represent: |
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Definition
| The history suggests possible HIV infection, and the lesions are typical for Kaposi's sarcoma, which is now ascribed to infection with human herpesvirus 8. |
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Term
A 40 year old female has enlargement of the anterior neck region. Fine needle aspiration of the thyroid yields cells that are consistent with a neoplasm. A chest radiograph is normal. She is euthyroid, but her serum ionized calcium is elevated, and she has a blood pressure of 155/105 mm/Hg. She is taken to surgery, and a thyroidectomy is performed, after frozen sections of several thyroid masses show a malignant neoplasm composed of polygonal cells in nests. Immunoperoxidase staining for calcitonin of the permanent sections is positive, and the neoplasm has an amyloid stroma with Congo red staining. She is most likely to have:
A Thyroglossal duct cysts
B Multiple endocrine neoplasia
C Papillary thyroid carcinoma
D Metastatic renal cell carcinoma
E Parathyroid carcinoma |
|
Definition
| She has MEN IIa, with medullary thyroid carcinomas (often multiple when familial), parathyroid hyperplasia, and pheochromocytoma.B |
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Term
A 38 year old male sees his physician because of abdominal pain, nausea, and constipation. He has no palpable abdominal masses and bowel sounds are present. His lungs are clear to auscultation. He has a heart rate of 80 with an irregular rhythm. An electrocardiogram demonstrates a shortened QT interval and a prolonged PR interval. He has a stool positive for occult blood. Upper endoscopy reveals multiple 1 cm diameter shallow ulcerations of the gastric antrum. Which of the following serum laboratory test findings is most likely to be present:
A Thyroid peroxidase antibody of 4 IU/mL
B Calcium of 12.4 mg/dL
C Glucose of 225 mg/dL
D Total thyroxine of 21 ng/mL
E Cortisol of 45 microgm/dL at 8 am |
|
Definition
| He most likely has a parathyroid adenoma secreting excessive parathormone to increase serum calcium and decrease serum phosphorus. The hypercalcemia leads to increased gastrin production and peptic ulcer disease. Hypercalcemia produces cardiac arrhtymias (or asystole).B |
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Term
A 58 year old man with a history of diabetes mellitus has noted the presence of bone pain, especially of his hands, for the past 6 months. There is no swelling or redness. His range of motion is slightly decreased, but there is no joint deformity. A serum chemistry panel shows sodium 139 mmol/L, potassium 4.0 mmol/L, chloride 98 mmol/L, C02 22 mmol/L, glucose 153 mg/dL, creatinine 7.8 mg/dL, calcium 7.8 mg/dL, phosphorus 5.7 mg/dL, total protein 6.2 g/dL, and albumin 4.0 g/dL. Which of the following conditions best accounts for these findings:
A Adrenal adenoma
B Medullary carcinoma
C Extra-adrenal pheochromocytoma
D Parathyroid hyperplasia
E Pituitary adenoma |
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Definition
| He has secondary hyperparathyroidism from chronic renal failure. Retention of phosphorus drives the calcium down and parathormone secretion up, leading to osteitis fibrosa cystica.D |
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Term
Which of the following pathologic processes is LEAST likely to be associated with laboratory evidence for hyperthyroidism as measured by an increased serum thyroxine:
A Subacute granulomatous thyroiditis
B Multinodular goiter
C Medullary carcinoma of thyroid
D Hashimoto's thyroiditis
E Follicular adenoma of thyroid |
|
Definition
Medullary carcinomas, derived from interstitial 'C' cells, do not secrete thyroid hormone.C
(D) Incorrect. There may be transient hyperthyroidism in association with Hashimoto's thyroiditis early on, but eventually the patient becomes hypothyroid. |
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Term
A 47 year old woman feels a "lump" in her neck that she didn't notice several months before. Her physician palpates a firm nodule about 2 cm in size to the left of midline in the region of the thyroid gland. By scintigraphic scanning with Tc99, this nodule appears "cold" with normal activity in the surrounded gland. Based upon these findings, she is most likely to have a:
A Papillary carcinoma
B Follicular adenoma
C Medullary carcinoma
D Toxic nodular goiter
E Granulomatous thyroiditis |
|
Definition
| The majority of 'cold' nodules are benign and many are adenomas.B |
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Term
A 40 year old female has painless swelling of her anterior neck in the region of the thyroid. She has an increased titer of anti-microsomal and anti-thyroglobulin antibodies. Within a month, the swelling has diminished. All of the following statements regarding her condition are true EXCEPT:
A There is an increased risk for lymphoma.
B Hypothyroidism may eventually occur.
C Lymphoid follicles are often present histologically.
D There is a genetic deficiency in antigen-specific suppressor T lymphocytes.
E Amyloid is often present histologically. |
|
Definition
Amyloid stroma is a feature of medullary carcinomas of the thyroid, not Hashimoto's thyroiditis.E
(D) Incorrect. This is a theory for the pathogenesis of Hashimoto's thyroiditis. |
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Term
A 30-year-old female from Barcelona has noted enlargement of her neck over the past few months. On physical examination, she has a diffusely enlarged thyroid that is not painful to palpation. Her TSH level is 0.2 microU/ml. A subtotal thyroidectomy is performed and histologically the tissue shows follicles with papillary infoldings lined by tall columnar cells. These findings are most consistent with:
A Subacute granulomatous thyroiditis
B Papillary carcinoma
C Multinodular goiter
D Hashimoto's thyroiditis
E Grave's disease |
|
Definition
Diffuse hyperplasia is typical for Grave's disease. The TSH is low from negative feedback from increased thyroid hormone production.E
(B) Incorrect. The papillary infoldings of papillary carcinoma would be lined by neoplastic cells unlikely to be columnar, and the neoplasm would probably not be diffuse. |
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Term
A multinodular goiter in a 39 year old female whose thyroid function tests are normal is best characterized by which of the following statements or conditions:
A Possible development of thyrotoxicosis
B Predisposition to development of papillary carcinoma
C Presence of thyroid stimulating immunoglobulins
D Previous viral infection
E Painful enlargement |
|
Definition
| A toxic multinodular goiter occurs in less than half of patients with multinodular goiter.A |
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Term
A 50 year old male has had persistent nausea for many years with occcasional vomiting. An endoscopy performed 5 years before showed chronic gastritis with the presence of Helicobacter pylori. He now undergoes a second endoscopy, and a small area of gastric mucosa is noted in the fundus which has loss of rugal folds. A biopsy reveals well-differentiated adenocarcinoma confined to the mucosa. This lesion is best characterized by:
A Metastases limited to regional lymph nodes
B A 5-year survival following resection of >90%
C A signet ring cell pattern by light microscopy
D The gross appearance of linitis plastica
E A high incidence of occurrence in the United States |
|
Definition
The prognosis with early gastric carcinoma (EGC) is good, compared with other gastric cancers.B
(C) Incorrect. A signet ring cell pattern is more typical for a poorly differentiated, diffuse type of gastric carcinoma, not an early gastric carcinoma (EGC).
(D) Incorrect. Linitis plastica characterizes a diffuse cancer with a poor prognosis, not an early gastric carcinoma (EGC). |
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Term
A 34 year old female has the sudden onset of severe abdominal pain. Physical examination reveals marked diffuse tenderness in all quadrants, and bowel sounds are absent. No masses are palpable. The serum amylase is 410 U/L and lipase is 610 U/L. The LEAST likely etiology for these findings is:
A Hypertriglyceridemia
B Trauma
C Hypercalcemia
D Cystic fibrosis
E Viral infection |
|
Definition
| Pancreatitis is not a complication of cystic fibrosis. The acinar pancreas atrophies over time, with fatty replacement, but it does not typically become acutely inflamed.D |
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Term
A 25-year-old male gives a history of recent cramping abdominal pain for the past week associated with fever, low-volume diarrhea, and right lower quadrant abdominal pain. On physical examination, there is right lower quadrant tenderness. Bowel sounds are present. His stool is positive for occult blood. A colonoscopy reveals mucosal edema and ulceration in the ascending colon, but the transverse and descending portions of the colon are not affected. The most likely characteristic microscopic finding would be:
A Crypt abscesses
B Entameba histolytica organisms
C Adenocarcinoma
D Band-like mucosal fibrosis
E Granulomas |
|
Definition
CORRECT. This history is most typical for Crohn's disease, which tends to involve the bowel in a segmental pattern.E
(A) Incorrect. This is a more typical finding of ulcerative colitis, which would typically involve the rectum first and extend upward in a continuous fashion. |
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Term
A 72 year old female notes scleral icterus and some nausea for the past month. When she becomes increasingly icteric, she goes to a hospital where the following laboratory findings are noted: total protein 6.1 g/dL, albumin 3.3 g/dL, alkaline phosphatase 210 U/L, AST 89 U/L, ALT 60 U/L, total bilirubin 7.2 mg/dL, and direct bilirubin 6.3 mg/dL. Her serum lipase is 40 U/L. A physical examination reveals no abdominal pain. She has active bowel sounds. A stool tested for occult blood is negative. Which of the following conditions is most likely to account for these findings:
A Pancreatic adenocarcinoma
B Cystic fibrosis
C Chronic active hepatitis
D Primary biliary cirrhosis
E Chronic persistent hepatitis |
|
Definition
CORRECT. Adenocarcinoma of the head of pancreas produces extrahepatic biliary obstruction with an elevation predominantly of the direct bilirubin along with an elevation in alkaline phosphatase. This results in the classic finding of 'painless jaundice'.A
(D) Incorrect. The AMA would be positive in most cases of PBC. |
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Term
A solitary sharply demarcated 2-cm gastric antral ulcer found on upper endoscopy in a 40-year-old male will be most commonly associated with which of the following findings:
A Gastric achlorhydria
B Crohn's disease
C Hematemesis
D Lymph node metastases
E Islet adenoma of pancreas |
|
Definition
CORRECT. Most gastric ulcers of this size are benign. Bleeding, however, is a common complication seen in 25 to 33% of cases.C
(A) Incorrect. Both acid and pepsin must be present (though not increased in amount) for peptic ulceration to occur. Achlorhydria is more typical for chronic atrophic gastritis. |
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Term
A 40 year old male has a long history of chronic alcoholism. His liver is firm on palpation. An abdominal CT scan reveals that the liver has changes consistent with cirrhosis. He joins a support group for alcoholics and stops drinking. Despite his continued abstinence from alcohol, he remains at risk for development of which of the following diseases:
A Hepatocellular adenoma
B Focal nodular hyperplasia
C Cholelithiasis
D Angiosarcoma
E Hepatocellular carcinoma |
|
Definition
CORRECT. Hepatocellular carcinoma occurs most often in the setting of cirrhosis (which is most often from chronic alcoholism in the U.S., but from viral hepatitis in the rest of the world).E
(A) Incorrect. Such adenomas, which are rare, are more often related to oral contraceptive use. |
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Term
| Difficulty swallowing has been noted for over 6 months by a 45-year-old female. A barium swallow reveals an area of stricture in the lower esophagus just above the gastroesophageal junction. She has an upper endoscopy performed and a biopsy of the lower esophagus is taken which shows no acute inflammation or ulceration, only submucosal atrophy along with fibrosis of smooth muscle. These findings most strongly suggest: |
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Definition
| Patients with progressive systemic sclerosis with either the CREST syndrome or diffuse scleroderma have fibrosis in the gastrointestinal tract, most often in the esophagus. |
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Term
A 43 year old female has noted increasing icterus for several weeks. A liver biopsy shows only intracanalicular cholestasis in the centrilobular regions, along with swollen liver cells and portal tract edema. There is no necrosis and no fibrosis. There is no increase in stainable iron. The most probable cause for these findings, among the following disease processes is:
A Chronic passive congestion
B Hepatitis B viral infection
C Extrahepatic biliary atresia
D Veno-occlusive disease
E Choledocholithiasis |
|
Definition
CORRECT. The bile stasis results from blockage of the biliary tract drainage.E
(C) Incorrect. Extrahepatic biliary atresia is a cause for biliary tract obstruction in a neonate. |
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Term
| Veno-occlusive disease (VOD) |
|
Definition
| can be due to ingestion of plant alkaloids that may be found in herbal teas. VOD can be caused by radiation therapy, some chemotherapy agents (particularly those used prior to bone marrow transplantation), and alcoholic liver disease. The morphologic changes resemble Budd-Chiari syndrome, but in the early stages, central and sublobular veins show intimal thickening and progressive narrowing of the lumen. |
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Term
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Definition
| (thrombosis primarily of hepatic veins) is usually idiopathic, although some cases are associated with oral contraceptives, myeloproliferative disorders, or neoplastic involvement of veins. The histologic changes resemble those of severe congestive heart failure with marked sinusoidal dilation and congestion. |
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Term
70 year old previously healthy male notes blood streaked stool and sees his physician, who confirms that the stool guaiac is positive, though a lesion cannot be detected by digital rectal examination. Physical examination of the abdomen reveals no palpable masses or organomegaly. There is no tenderness and bowel sounds are active. An appendectomy scar is present in the right lower quadrant. A colonoscopy is performed, and there is an area of obstruction from an encircling mass with superficial ulceration that is located at 20 cm. Which of the following risk factors was most likely to have been present for development of this lesion:
A Human papillomavirus infection
B Crohn's disease
C High fat diet
D Diverticulosis
E Prior abdominal surgery |
|
Definition
| CORRECT. This is a colonic adenocarcinoma. At his age, this is likely a sporadic type of colon cancer, not an inherited condition nor a complication of inflammatory bowel disease, particularly in view of no past history of bowel disease.C |
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Term
A 42 year old man has been an intravenous drug user of heroin for 20 years. He has recently noted blood-streaked stool on defacation. He is most likely to have:
A Hemorrhoids
B Megacolon
C Angiodysplasia
D Perirectal fistulae
E Adenocarcinoma |
|
Definition
| CORRECT. Hemorrhoids form in the setting of chronic constipation. (Actually, this is probably the worst direct consequence of opiate usage; serious health problems arise from nonsterile injection).A |
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Term
A 42 year old male has had fevers for several weeks. His CBC shows a Hgb of 12.2 g/dL, Hct 37.1%, MCV 92 fL, platelet count 243,000/uL, and WBC count 75,000/uL. The WBC differential count shows 82 segs, 8 bands, 3 metamyelocytes, 1 myelocyte, 4 lymphocytes, and 1 monocyte. The leukocyte alkaline phosphatase (LAP) score is high at 130. Which of the following laboratory test findings is most likely to be present:
A Bone marrow karyotype of 46, XY, t(9;22)
B Serologic titer of 1:1024 for anti-double stranded DNA
C Serum vitamin B12 level of 100 pg/mL
D 4+ ketonuria and 4+ proteinuria
E Blood culture positive for Streptococcus viridans |
|
Definition
CORRECT. The high WBC count with left shift (but no blasts) and the high LAP score are consistent with the leukemoid reaction. The term 'leukemoid' is used because there is an increased WBC count with immature forms similar to leukemia.E
(A) Incorrect. This translocation is the 'Philadelphia chromosome' of chronic myelogenous leukemia. With CML, the LAP score is low. |
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Term
The spleen does all of the following EXCEPT:
A Serve as a reserve and storage site for platelets
B Trap red blood cell inclusions (Howell-Jolly bodies, etc.)
C Contribute to the function of the humoral immune system
D Become a site for extramedullary hematopoiesis (EMH)
E Become enlarged in adults with sickle cell anemia |
|
Definition
| CORRECT. This statement is false. The spleen undergoes multiple infarctions with sickle cell anemia, leading to a small shrunken spleen in surviving adults.E |
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Term
| A 30 year old male noticed a progressively worsening cough for one month. On physical examination, a few small lymph nodes were palpable in the axillae, and the tip of the spleen was palpable. A CBC showed: Hgb 10.2 g/dl, Hct 31.1%, MCV 90 fL, WBC count 67,000/microliter, and platelet count 36,000/microliter. If many blasts with Auer rods are seen in the peripheral blood smear, then the most likely diagnosis is: |
|
Definition
| CORRECT. The high WBC count with the blasts and Auer rods are very characteristic for an acute myelogenous leukemia. |
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Term
A 64-year-old male has noted the presence of several lumps on the right side of his neck for several months. These are firm, non-tender, movable lymph nodes palpable in right posterior cervical region. He does not have splenomegaly or hepatomegaly. He has a mild normocytic anemia, and his WBC count is normal. A lymph node biopsy shows numerous crowded follicles of small, monomorphic lymphocytes. This is most typical for:
A Chronic lymphocytic leukemia
B Poorly differentiated lymphocytic lymphoma
C Infectious mononucleosis
D Hodgkin's disease, lymphocyte predominance type
E Reactive hyperplasia |
|
Definition
CORRECT. Lymphadenopathy with malignant lymphoma is typically nontender, as contrasted with the lymphadenopathy of infections.
(D) Incorrect. Although there are numerous lymphocytes, occasional Reed-Sternberg cells or variants can be found. The lymphoid population of Hodgkin's disease is reactive.
(E) Incorrect. Reactive hyperplasia is polyclonal, not monoclonal. |
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Term
A 45 year old male has had increasing abdominal discomfort with enlargement for the past two years. On physical examination, the spleen can be felt below the left costal margin. An abdominal CT scan reveals massive (3000 gm size) splenomegaly. Of the following conditions, the one most likely to cause this finding is:
A Myelofibrosis
B Sickle cell anemia
C Portal hypertension
D Infectious mononucleosis
E Hemochromatosis |
|
Definition
CORRECT. Myeloproliferative disorders, and myelofibrosis in particular, are known to cause massive splenomegaly.A
(C) Incorrect. A spleen will rarely be larger than 800 gm in a patient (such as a chronic alcoholic with micronodular cirrhosis of liver) who has portal hypertension. |
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Term
A 72-year-old Caucasian male has a Hgb of 9.1 gm/dL, Hct 27.9%, MCV 96 fL, WBC count 3700/microliter, and platelet count 125,000/microliter. The peripheral blood smear shows nucleated RBC's, but he has a negative direct and indirect Coombs test. The most probable cause is:
A Metastatic carcinoma to bone marrow
B Chronic alcoholism
C Malabsorption
D Hemoglobinopathy
E Chronic blood loss |
|
Definition
CORRECT. Metastatic tumor or marrow fibrosis leads to a peripheral 'leukoerythroblastic' picture with immature RBC's and WBC's in the peripheral blood smear.A
(E) Incorrect. Chronic blood loss leads to iron deficiency, and this decreases marrow RBC production. Nucleated RBC's are not released in that circumstance. |
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Term
A 40 year old female presented with a week long history of fever and mental confusion. Physical examination revealed widespread petechiae of skin and mucosal surfaces. Her serum BUN and creatinine were elevated. She had marked thrombocytopenia, but deteriorated rapidly following platelet transfusion. At autopsy, pink hyaline thrombi were found in small myocardial arteries. The best diagnosis is:
A Disseminated intravascular coagulopathy
B Idiopathic thrombocytopenic purpura
C Thrombotic thrombocytopenic purpura
D Trousseau's syndrome
E Warm autoimmune hemolytic anemia |
|
Definition
CORRECT. The hyaline thrombi are typical for TTP. Platelet transfusion is contraindicated.C
(B) Incorrect. ITP can lead to thrombocytopenia, but an antibody to platelets is involved, and hyaline thrombi are not featured.
(A) Incorrect. DIC may produce peripheral hyaline thrombi, but typically follows as a complication of a serious disease process, not de novo. |
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Term
Immature white blood cells are readily seen in the peripheral blood in all of the following conditions EXCEPT:
A Leukemoid reaction
B Chronic myelogenous leukemia
C Acute myelogenous leukemia
D Acute lymphocytic leukemia
E Chronic lymphocytic leukemia |
|
Definition
| CORRECT. Most of the circulating cells are small, mature lymphocytes.E |
|
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Term
A 16-year-old male has a Hgb of 8.8 g/dL, Hct 24.1%, MCV 65 fL, WBC count 7400/microliter, and platelet count 187,000/microliter. His serum ferritin is 3740 ng/mL. He has a palpable spleen tip. A bone marrow biopsy reveals a myeloid:erythroid ratio of 1:4, and there is 4+ stainable iron. He is most likely to have:
A G6PD deficiency
B Beta-thalassemia
C Sickle cell anemia
D Hereditary spherocytosis
E Malaria |
|
Definition
| CORRECT. Reduced beta-globin chain synthesis leads to RBC hypo-chromia, ineffective erythropoiesis, and excessive iron absorption. |
|
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Term
|
Definition
| formed of the cytoplasmic granules of the myeloid blasts of AML and are a typical finding with AML. |
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Term
A 42 year old female notices that even minor bumps produce major bruises over her body. The protime is 12.9 seconds and the partial thromboplastin time is 26.2 seconds. Her CBC shows a Hgb of 11.1 g/dL, Hct 33.1%, MCV 84 fL, platelet count 790,000/uL, and WBC count of 45,400/uL. A bone marrow biopsy reveals a hypercellular marrow with increased numbers of myeloid precursors and megakaryocytes. These findings most strongly suggest a diagnosis of:
A Epstein-Barr virus infection
B Myeloproliferative disorder
C Drug reaction to recent antibiotic therapy
D Wiskott-Aldrich syndrome
E Megaloblastic anemia |
|
Definition
| CORRECT. There is an increase in more than one cell line. Paradoxically, there is bleeding because the platelets are functionally abnormal, even though there are lots of them.B |
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Term
Which one of the following findings is LEAST likely to be associated with drug use:
A G6PD deficiency with anemia
B Autoimmune hemolytic anemia
C Macrocytic anemia
D Aplastic anemia
E Microcytic anemia |
|
Definition
| CORRECT. This is usually an iron deficient state not associated with drug usage.E |
|
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Term
An abdominal ultrasound is performed on a 35 year old male. In which of the following conditions is the spleen most likely to appear normal in size:
A Hemolytic anemia
B Macronodular cirrhosis
C Myelofibrosis
D Idiopathic thrombocytopenic purpura
E Sickle cell anemia |
|
Definition
| CORRECT. Though with ITP there are circulating antibodies to platelets that lead to platelet destruction, the spleen itself is usually not enlarged. |
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Term
A 45 year old male has a low serum albumin and increased prothrombin time. His serum ferritin is 5800 ng/mL. These findings may occur as a complication of all of the following conditions EXCEPT:
A Beta-Thalassemia
B Autoimmune hemolytic anemia
C Anemia of chronic disease
D Polycythemia vera
E Pernicious anemia |
|
Definition
| CORRECT. There is an increased RBC mass with primary polycythemia, but iron stores are not increased.D |
|
|
Term
| Acute lymphoblastic leukemia (ALL) |
|
Definition
Mostly occurs in children and young adults (peak incidence at age 4); uncommon in adults
Most (80%) are of B or preB-cell origin (and CD19 positive); most common type is an early pre-B cell subtype that is CALLA antigen positive (CD10) and has a good prognosis
The TdT (terminal deoxyribonucleotidyl transferase) is almost always positive
Lymphadenopathy and splenomegaly (not massive) can be present |
|
|
Term
| Acute myeloblastic leukemia (AML) |
|
Definition
Mostly occurs in the age range of 15 to 39 years, but can occur into 80’s
There is a wide range of subtypes, based upon morphology
Myeloperoxidase granules are present in the cytoplasm of leukemic cells
Auer rods (red rod-like structures) in the cytoplasm are distinctive
WBC count is often high
Prognosis is fair with chemotherapy; better with allogeneic bone marrow transplantation |
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Term
| Chronic myelogenous leukemia (CML) |
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Definition
Mostly occurs in the age range of 30 to 60 years
Course is one of slow progression, but half of patients eventually develop an accelerated phase with “blast crisis”
Often a very high WBC count (neutrophilia with few blasts, but basophilia, eosinophilia, and many immature WBC forms), low leukocyte alkaline phosphatase score helps to distinguish CML from a leukemoid reaction and polycythemia vera; hepatosplenomegaly (can be massive) often present
Diagnostic feature is presence of Philadelphia (Ph1) chromosome, t(9:22)
Prognosis is poor |
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Term
| Chronic lymphocytic leukemia (CLL) |
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Definition
Seen in older adults (over 50 years), more often males
Small mature lymphocytes (B-cells) collect in marrow (from lack of apoptosis) and appear increased in peripheral blood; do not contain TdT
Indolent course; some patients develop autoimmune hemolytic anemia; hypogammaglobulinemia from lack of proper B-cell function with resultant bacterial infections often occurs |
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Term
A 45-year-old male has a Hgb 14.2, MCV 92, platelet count 225,000, and WBC count 44,500 with 59 segs, 20 bands, 8 meta, 4 myelo, 2 promyelo, 5 lymphs, 2 monos. A bone marrow biopsy shows myeloid hyperplasia with <5% blasts. Which of the following laboratory test findings is probably present:
A Elevated leukocyte alkaline phosphatase
B Karyotype with t(9;22)
C Monoclonal gammopathy
D Elevated D-dimer
E Positive TdT |
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Definition
CORRECT. The findings point to a leukemoid reaction.A
(B) Incorrect. The findings are less likely to be chronic myelogenous leukemia than a leukemoid reaction. The Hgb and platelet counts are normal, and there is no eosinophilia or basophilia.
(E) Incorrect. He does not have acute lymphocytic leukemia. |
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Term
A 40 year old male is found to have posterior cervical lymphadenopathy on physical examination. This can be a feature of all of the following conditions EXCEPT:
A Human immunodeficiency virus infection
B Toxoplasmosis
C Infectious mononucleosis
D Non-Hodgkin's lymphoma
E Multiple myeloma |
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Definition
| CORRECT. Myeloma primarily involves the bone marrow.E |
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Term
A 45-year-old male has noted a change in the appearance of his face. The skin is thickened and reddened. A skin biopsy reveals infiltration by neoplastic T lymphocytes. His condition is known as:
A Hodgkin's disease
B Mycosis fungoides
C Burkitt's lymphoma
D Acute lymphocytic leukemia
E Hairy cell leukemia |
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Definition
| CORRECT. If the neoplastic cells circulate, it is known as Sezary's syndrome. |
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Term
A 49-year-old male has a Hgb of 10.1 g/dL, Hct 30.3%, MCV 90 fL, WBC count 1600/microliter, and platelet count 48,000/microliter. On physical examination he has massive splenomegaly but no lymphadenopathy. On his peripheral blood smear are increased numbers of peripheral blood lymphocytes containing tartrate-resistant acid phosphatase. The most probable diagnosis is:
A Chronic lymphocytic leukemia
B HTLV-1 infection with leukemia
C Hairy cell leukemia
D Gaucher's disease
E Myelodysplasia |
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Definition
| CORRECT. The proliferation of B-lymphocytes involves peripheral blood, bone marrow, and spleen. Hepatomegaly is not common, and lymphadenopathy is rare.C |
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Term
Each of the following statements regarding a monoclonal gammopathy is correct EXCEPT:
A May occur in the absence of a demonstrable plasmacytoma
B May not be associated with Bence-Jones proteinuria
C May be absent in serum or urine in a few multiple myeloma patients
D May be composed only of heavy chains
E May result from chronic inflammation |
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Definition
| CORRECT. Inflammation leads to a polyclonal gammopathy.E |
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Term
Of the following findings, the one that will be most likely to occur with extravascular, rather than intravascular, hemolysis is:
A Splenomegaly
B Increased reticulocyte count
C Acute renal failure
D Decreased serum haptoglobin
E Jaundice |
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Definition
| CORRECT. The RBC's are being sequestered in the spleen with extravascular hemolysis.A |
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Term
Of the following statements regarding a hemolytic anemia, the one that does NOT describe a usual feature is:
A The red cell distribution width (RDW) is increased with RBC fragmentation.
B The level of serum haptoglobin is increased.
C Reticulocytosis is suggested by the appearance of polychromasia.
D The level of unconjugated bilirubin is increased.
E Hemoglobinuria may appear with intravascular hemolysis. |
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Definition
| CORRECT. Haptoglobin is decreased as a consequence of hemolysis, because haptoglobin scavenges free hemoglobin in the bloodstream. |
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Term
A 37 year old male known to be infected with the human immunodeficiency virus for the past 10 years is admitted with abdominal pain of three days' duration. Physical examination reveals abdominal distension with absent bowel sounds. An abdominal CT scan reveals a mass lesion of the ileum. He is taken to surgery, and an area of bowel obstruction in the ileum is removed. The specimen is examined in surgical pathology and there is a near-encircling firm white mass 10 cm long and 3 cm in greatest depth that infiltrates through the wall of the bowel. The best diagnosis is:
A Plasmacytoma
B Hodgkin's disease, lymphocyte predominant type
C Small non-cleaved lymphoma
D Metastatic adenocarcinoma
E Myeloproliferative disorder |
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Definition
| CORRECT. Such intermediate to high grade lymphomas are typical for, as well as diagnostic of, AIDS. |
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Term
Following splenectomy, which of the following peripheral blood findings would be LEAST likely to be present:
A Pappenheimer bodies in RBC's
B Increased white blood cell count
C Howell-Jolly bodies in RBC's
D Increased platelet count
E Polycythemia |
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Definition
| CORRECT. The red cell mass does not increase following splenectomy.E |
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Term
A 59-year-old male with a history of prostate cancer now has a WBC count of 8800/mcl with Hgb 9.4, MCV 89, platelet count 135,000, and differential count with 61 PMN's, 15 bands, 14 lymphs, 5 monos, 3 metas, and 2 myelos with 4 NRBC's/100 WBC's. These findings are most consistent with:
A Iron deficiency anemia
B Leukoerythroblastosis
C Acute myelogenous leukemia
D Disseminated intravascular coagulation
E Leukemoid reaction |
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Definition
| CORRECT. The immature WBC's and RBC's in the peripheral blood are typical for leukoerythroblastosis. He probably has widely metastatic prostate cancer to bone marrow.B |
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Term
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Definition
| rare form of glycogen storage disease in which there is decreased myophosphorylase to utilize glycogen stores for quick ATP production in skeletal muscle. |
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Term
A 9 year old girl gets tired easily but is still able to perform nomal daily activities. Through young adulthood, she continues to exhibit mild generalized muscle weakness. On physical examination, she has motor strength 2/5 in all extremities along with hypotonia. Reflexes are normal. She has no myalgias. A deltoid biopsy shows muscle fibers with numerous rod-shaped intracytoplasmic inclusions. She is most likely to have:
A An autoimmune disease
B Lower motor neuron disease
C Muscular dystrophy
D A congenital myopathy
E Myophosphorylase deficiency |
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Definition
| CORRECT. She has findings that suggest nemaline rod myopathy, one of several types of congenital myopathy, some of which can be non-progressive. There are a variety of inheritance patterns. |
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Term
A 35-year-old female had a diagnosis of polymyositis made with a muscle biopsy. Following a course of corticosteroid therapy, she continues to have muscular weakness, particular in proximal muscles. The serum creatine kinase (CK) is only 41 U/L, and an electromyogram demonstrates no myopathic changes. These findings most strongly suggest:
A Treatment failure
B Upper motor neuron disease
C Corticosteroid-induced myopathy
D McArdle's disease
E Duchenne muscular dystrophy |
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Definition
| CORRECT. Corticosteroid-induced myopathy typically leads to proximal muscular weakness. Type II fiber atrophy on biopsy is also typical, if a rebiopsy were done in this case.C |
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Term
Which one of the following histologic findings on muscle biopsy would be most indicative of an active polymyositis:
A Marked fiber hypertrophy
B Many fibers undergoing degeneration and regeneration
C Grouping of atrophic fibers
D Extensive endomysial and perimysial fibrosis
E Subsarcolemmal glycogen deposition |
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Definition
CORRECT. The patchy inflammatory condition is destroying fibers which are then regenerating in response.B
(A) Incorrect. This is more typical of the early stages of a muscular dystrophy in which muscular 'pseudohypertrophy' is seen.
(D) Incorrect. Fibrosis is more typical of a chronic myopathic muscular disease. |
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Term
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Definition
| Cherry red macula. GM2 gangliosidosis or Hexosaminidase A deficiency) is an autosomal recessive genetic disorder. In its most common variant known as infantile Tay-Sachs disease, it presents a relentless deterioration of mental and physical abilities which commences at six months of age and usually results in death by the age of four |
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Term
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Definition
| Inflammatory lesion of eyelids - Chalazion – a chronic inflammatory infiltrate of the meibomian or Zeis glands. If a presumed chalazion recurs, biopsy it to be sure that it is not a neoplasm such as a sebaceous carcinoma. |
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Term
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Definition
| Inflammatory lesion of eyelids - Chalazion – a chronic inflammatory infiltrate of the meibomian or Zeis glands. If a presumed chalazion recurs, biopsy it to be sure that it is not a neoplasm such as a sebaceous carcinoma. |
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Term
A 3-year-old child, who had been born prematurely at 30 weeks gestation and then developed hyaline membrane disease at birth, is found to be visually impaired. Funduscopic examination reveals retinal detachment on the left. The most likely pathogenesis of this condition is:
A Retinal damage by oxygen species of free radicals
B Hereditary hexosaminidase A deficiency
C Germinal matrix hemorrhage at birth
D Alobar holoprosencephaly
E Absence of tumor suppressor gene on chromosome 13 |
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Definition
| CORRECT. The child has retrolental fibroplasia, a complication of high dose oxygen therapy following birth, typically administered because of hyaline membrane disease.A |
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Term
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Definition
| a raised, yellowish-white, vascularized lesion (see arrow, below left) near the limbus (the scleral-corneal junction). It is seen in the elderly with extensive sun exposure. |
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Term
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Definition
| this is basically a pinguecula extending onto the cornea, interfering with vision |
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Term
| Fuchs’ endothelial dystrophy |
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Definition
| – The endothelium degenerates and becomes leaky, allowing fluid to seep into the cornea from the anterior chamber. It causes blurred vision, worst in the morning, in middle aged to elderly patients |
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Term
A 4 year old boy has lost vision in the right eye. Other children in his family have had a similar problem, with many losing vision in both eyes. A presumptive diagnosis of an intraocular tumor is made. Which of the following statements is LEAST appropriate for this condition:
A Leukocoria is often noted on physical examination
B The patient may have inherited a faulty tumor suppressor gene
C The tumor is composed of small blue cells that form rosettes
D Dystrophic calcification is often seen histologically
E The tumor often spreads via scleral vascular channels |
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Definition
| CORRECT. Retinoblatoma, though rare, is the most common childhood intraocular tumor. A retinoblastoma will invade into the optic nerve, whereas melanomas in adults tend to spread via scleral vascular channels.E |
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Term
A firm 0.4 cm nodule is noted on examination of the upper eyelid of a 41 year old male. Biopsy of this lesion demonstrates inflammation consisting of lymphocytes admixed with epithelioid cells and giant cells. The diagnosis is most likely to be a(an):
A Pinguecula
B Hordeolum (stye)
C Pterygium
D Chalazion
E Actinic keratosis |
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Definition
CORRECT. This lesion is due to chronic inflammation of the meibomian glands.D
(B) Incorrect. A stye is seen on the eyelid, but is due to an infection of the eccrine sweat glands (glands of Moll). |
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Term
| diabetes mellitus with a diabetic background retinopathy can be characterized by |
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Definition
| dot and blot hemorrhages, capillary microaneurysms, flame-shaped hemorrhages, hard exudates, and cotton wool spots (soft exudates). |
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Term
| an infection with acute inflammation of the eccrine sweat glands of the eyelid. |
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Definition
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Term
A 9 year old girl has recently complained of difficulty breathing for over a week. Her vital signs include T 37.9 C, P 80, R 25, and BP 110/60 mm Hg. On physical examination, her lung fields are clear to auscultation. Her heart rate is regular and no murmurs or gallops are heard. A chest radiograph shows prominent hilar lymphadenopathy along with a 1 cm peripheral right lung nodule in the middle lobe. No infiltrates or masses are present. A sputum gram stain shows normal flora and routine bacterial culture reveals no pathogens. Which of the following conditions is most likely to be present:
A Hypersensitivity pneumonitis
B Mycobacterium tuberculosis infection
C Bronchial carcinoid tumor
D Infective endocarditis
E Goodpasture syndrome |
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Definition
| CORRECT. This is a description of the typical 'Ghon complex' of an initial, or primary, TB infection. The peripheral granuloma does not produce symptoms, but the enlarged lymph nodes may impinge upon airways. Most persons with a primary infection, however, are asymptomatic and the infection goes unnoticed.B |
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Term
A 55 year old male with a long history of smoking has recently experienced an episode of hemoptysis along with his usual cough. He has a sputum cytology examination performed that demonstrates the presence of atypical cells with hyperchromatic nuclei and orange-pink cytoplasm. A chest radiograph is most likely to reveal a(an):
A Large hilar mass
B Pneumonia-like consolidation
C Peripheral nodule
D Carinal mass
E Left pleural thickening |
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Definition
CORRECT. Both squamous cell and oat cell carcinomas tend to be central in location.A
C) Incorrect. Such a peripheral mass would more likely be a granuloma, an adenocarcinoma or a hamartoma. |
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Term
A 41 year old man with a 10 kg weight loss over the past 3 months now has had worsening fever, non-productive cough, and dyspnea for several days. A chest radiograph shows some patchy infiltrates in both lungs. He has a CBC that shows a WBC count of 3250/microliter with differential of 78 segs, 3 bands, 5 lymphs, 11 monos, 2 eosinophils, and 1 basophil, Hgb 13.8 g/dL, Hct 41.4%, MCV 91 fL, and platelet count 317,000/microliter. His CD4 lymphocyte count is 79/microliter. Cryptosporidium organisms are found in a stool specimen. A bronchoalveolar lavage is performed, yielding fluid that microscopically demonstrates pink, foamy exudate with little inflammation. Which of the following additional findings on microscopic examination is he most likely to have:
A Acid fast bacilli
B Branching septate hyphae
C Multiple cysts with GMS stain
D Hemosiderin-laden macrophages
E Short gram positive rods |
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Definition
CORRECT. He is most likely to have Pneumocystis carinii pneumonia in association with the acquired immunodeficiency syndrome. PCP has an exudate composed of the Pneumocystis cysts and trophozoites with little accompanying inflammation. The clinical findings in this case are typical as well.C
(B) Incorrect. Aspergillus often produces a fungus ball, or a defined mass, rather than infiltrates. It does not produce an acellular exudate. |
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Term
A 20 year old man falls while jogging along a city street early one morning. He suffers a minor abrasion to his left hand. However, within minutes he is very dyspneic and has right-sided chest pain. He walks into a nearby store, and the manager calls for an ambulance. On arrival at the hospital, he has tachypnea and tachycardia. A chest radiograph reveals that the mediastinum is shifted to the left, and there are no fractures. A thoracentesis on the right yields a rush of air. Which of the following conditions is most likely to have given rise to these events:
A Bronchopleural fistula
B Paraseptal emphysema
C Foreign body aspiration
D Intrinsic asthma
E Pulmonary atherosclerosis |
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Definition
CORRECT. Paraseptal emphysema is not common, but does account for a significant number of cases of spontaneous pneumothorax in young persons. It is not related to smoking. There are subpleural bullae that can burst with minimal trauma.B
(A) Incorrect. A bronchopleural fistula typically occurs in the setting of a severe lung infection, not in a healthy person. |
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Term
Following a vehicular accident with blood loss leading to prolonged, severe hypotension, a 30 year old male is intubated and placed on a ventilator. He becomes progressively difficult to oxygenate despite increasing PEEP and FI02 of 100%. He remains afebrile. He dies several days later. At autopsy, the lungs show diffuse hyaline membranes in alveoli, thickened alveolar walls, and many alveolar macrophages but few neutrophils. These findings are most characteristic for:
A Bronchopneumonia
B Chronic bronchitis
C Bronchiectasis
D Viral pneumonia
E Diffuse alveolar damage |
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Definition
| CORRECT. Diffuse alveolar damage (or ARDS as it is known clinically) is the final event following lung injury from a variety of serious illnesses or accidents. In this case, it was probably initiated by the hypotension ('shock lung') and potentiated by the 100% oxygen.E |
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Term
| A 60-year-old male has a cough for about a week without production of much sputum. A chest radiograph reveal a pneumonia-like area of consolidation in the right lower lobe. He is given antibiotic therapy, but a month later the radiographic picture has not changed, and his cough continues. A bronchoalveolar lavage is performed and yields atypical cells with scattered macrophages. The most likely diagnosis is: |
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Definition
| Bronchioloalveolar carcinoma, tumor spreads in a pnuemonia like fashion |
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Term
At autopsy, a 60 year old male is found to have a peripheral 7 cm area of golden-yellow consolidation on sectioning of the left lung. Microscopically, this area has alveoli filled with foamy macrophages. Which of the following conditions is most likely to be responsible for these findings:
A Mycoplasma infection
B Cystic fibrosis
C Adenocarcinoma
D Silicosis
E Squamous cell carcinoma |
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Definition
| CORRECT. Most squamous cell carcinomas are located centrally and arise in bronchi, leading to obstruction that can cause a lipid pneumonia. In this case, the breakdown of lung tissue distal to the mass yields an 'endogenous lipid pneumonia.'E |
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Term
A 40-year-old female develops fever, cough, and dyspnea over less than a day. A chest radiograph shows diffuse and nodular infiltrates. Her symptoms disappear after a month's vacation, but she had to come home to take care of her canaries, and her symptoms reappear. She probably has a condition caused by:
A Degranulation of mast cells
B Progressive interstitial fibrosis
C Antigen-antibody complexes
D Proliferation of Langerhans cells
E Infection with Mycobacterium kansasii |
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Definition
| CORRECT. Birds make a lot of organic dust from their feathers. The result is an extrinsic allergic alveolitis. Getting away from the antigen (such as a vacation) will improve the situation. Extrinsic allergic alveolitis is produced, which is mainly an interstitial process. Extrinsic allergic alveolitis, or hypersensitivity pneumonitis, is acutely mediated by activation of complement from exposure to an allergen, but there is usually no eosinophila and the symptoms last longer than for asthma.C |
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Term
A 51-year-old cardiac transplant recipient develops an abscessing pneumonia involving the left lower lobe. A sputum gram stain reveals normal upper respiratory tract flora. He does not respond to antibiotic therapy over 6 months. He develops multiple brain abscesses. A causative organism to consider in this setting is:
A Mycoplasma pneumoniae
B Aspergillus fumigatus
C Mycobacterium avium-intracellulare
D Nocardia brasiliensis
E Cytomegalovirus |
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Definition
CORRECT. Nocardia can persist and lead to chronic abscesses. It can appear in immunocompromised patients.D
(B) Incorrect. Aspergillus does not typically produce abscesses. |
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Term
Pulmonary atelectasis is seen by chest radiography to involve all of the right lung of a 44 year old male who had the sudden onset of severe dyspnea. This is most likely to have resulted from:
A Aspiration of a foreign body
B Pulmonary embolism
C Squamous cell carcinoma
D Penetrating chest trauma
E Bronchiectasis |
|
Definition
CORRECT. This would lead to pneumothorax with lung collapse. Such trauma is not infrequent, particularly in auto accidents.D
Atelectasis is defined as the lack of gas exchange within alveoli, due to alveolar collapse or fluid consolidation. It may affect part or all of one lung |
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Term
A newborn male develops increasing respirtory distress within an hour following delivery. A radiograph reveals near opacification of both lungs. Despite intubation and positive pressure ventilation, the baby dies within two days. At autopsy, the baby's lungs demonstrate extensive pink hyaline membranes. Of the following findings, the one that suggests the most significant risk for the baby's condition is:
A Maternal diabetes
B Female sex
C Increased lecithin/sphingomyelin ratio
D Maternal corticosteroid therapy
E Increased surfactant |
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Definition
CORRECT. The hyperinsulinism in the baby as a result of the high glucose impedes development of the type II pneumonocytes.A
(C) Incorrect. It is an increased L/S ratio >2 that indicates fetal lung maturity. Other similar tests include fluorescence polarization (fpol) and phosphatidyl glycerol (PG). |
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Term
| A neonate born at 30 weeks gestation has initial Apgar scores of 5 and 6 at 1 and 5 minutes following birth by normal vaginal delivery. However, increasing respiratory distress in the next hour requires intubation and positive pressure ventilation. Two months later, the infant is finally taken off the ventilator, but still does not oxygenate normally because he has developed: |
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Definition
| Bronchopulmonary dysplasia. The BPD is a complication of the treatment for neonatal respiratory distress. The positive pressure ventilation with the higher FIO2's, and the prolonged intubation, all contribute. Generally, these are premature infants who had a respiratory complication following birth, such as hyaline membrane disease. |
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Term
A 19-year-old male notes hematuria and sees his physician, who also notes mild proteinuria on a urinalysis specimen. There is no blood, glucose, or ketones noted in the urine. Additional examination, including audiometry, reveals only hearing loss. Which of the following findings is LEAST likely to be present:
A Other affected family members
B Basement membrane abnormalities
C Immune complexes in the mesangium
D Increased risk for renal failure in 10 years
E Visual problems |
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Definition
| CORRECT. Hereditary nephritis (Alport's syndrome) is not associated with immune complexes. Mesangial matrix, however, is increased, and epithelial cells may appear foamy. |
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Term
A hyperplastic arteriolosclerosis with fibrinoid necrosis, petechial hemorrhages, and microinfarcts seen in the kidneys in conjunction with a markedly elevated plasma renin is most consistent with which of the following patient histories:
A A 56 year old male with an acute myocardial infarction
B A 6 year old male with albuminuria
C A 62 year old female with end stage renal disease
D A 15 year old male with recent streptococcal infection
E A 45 year old female with scleroderma |
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Definition
CORRECT. The diffuse form of scleroderma can be associated with hyperplastic arteriolosclerosis and malignant hypertension.E
(C) Incorrect. End stage kidneys all look alike: thickened arteries, globally sclerotic glomeruli, and interstitial scarring with chronic inflammation. |
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Term
A 20 year old previously healthy male has been feeling tired for several days. He goes to his physician when he passes dark-colored urine. His serum creatinine is 4.4 mg/dL. A urinalysis reveals pH 6, specific gravity 1.011, 3+ blood, 1+ protein, no glucose, and no ketones. There are numerous RBC casts. Which of the following pathologic findings on renal biopsy is most likely to be present:
A Glomerular crescents
B Widened proximal tubules
C Polymorphonuclear infiltrates
D Lipiduria
E IgA deposited in glomerular capillaries |
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Definition
| CORRECT. Crescents are characteristic for a rapidly progressive glomerulonephritis; they form when there is leakage of fibrinogen into Bowman's space.A |
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Term
| Malaise for several weeks prompts a 43 year old male to visit his family physician. The physical examination is normal, except for a blood pressure of 150/95 mmHg and 1+ pitting edema of the lower extremities to the knees. Dipstick urinalysis shows no glucose, blood, ketones, nitrite, or urobilinogen, and the microscopic urinalysis revealed no RBC/hpf and only 1 WBC/hpf. Additional laboratory testing revealed a 24 hour urine protein of 4.1 gm. His serum creatinine was 2.2 mg/dL with BUN of 40 mg/dL. His hepatitis B surface antigen is positive. Which of the following conditions is he most likely to have: |
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Definition
| CORRECT. Membranous GN is the most common cause for nephrotic syndrome in adults. Some cases are associated with underlying infections or malignancies, but in most cases the cause is unknown. |
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Term
A 50 year old man was diagnosed at age 15 with type 1 diabetes mellitus. His disease is poorly controlled, as evidenced by elevated hemoglobin A1C levels. He develops a non-healing ulcer of his foot at age 35. At age 45, he is at risk for each of the following complications involving the kidneys EXCEPT:
A Nodular glomerulosclerosis
B Arteriolar nephrosclerosis
C Papillary necrosis
D Glomerular crescents
E Diffuse glomerulosclerosis |
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Definition
| CORRECT. This is an infrequent finding with diabetic nephropathy.D |
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Term
A 53-year-old female has taken 2 gm of phenacetin a day for her arthritis pain for the past 3 years. She now has an increasing serum BUN and creatinine. Her kidneys are most likely to show:
A Papillary necrosis
B Focal segmental glomerulosclerosis
C Nephrocalcinosis
D Interstitial nephritis
E Arteriolosclerosis |
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Definition
| CORRECT. She has analgesic abuse nephropathy which leads to papillary necrosis and tubular atrophy (though the renal columns are spared).A |
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Term
Which statement regarding drug-induced interstitial nephritis is INCORRECT:
A It usually follows a generalized allergic reaction.
B Methicillin can cause it.
C Symptoms subside if the drug is stopped.
D Eosinophils may appear in the urine.
E It is more frequent with increasing dosage. |
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Definition
| CORRECT. The appearance of this complication is unrelated to the dosage of the drug.E |
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Term
A 53 year old woman has fever and flank pain on the right. A urinalysis reveals sp. gr. 1.010, pH 7.5, no glucose, no protein, no ketones, 1+ blood, and many WBCs and WBC casts. An abdominal radiograph reveals a radiopaque calculus that forms a cast of a dilated right renal collecting system. A urine culture grows Proteus vulgaris. Which of the following crystals is most likely to be seen in large numbers on microscopic urinalysis:
A Calcium oxalate
B Cystine
C Calcium phosphate
D Uric acid
E Magnesium ammonium phosphate |
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Definition
CORRECT. She has a staghorn calculus and acute pyelonephritis. These 'infection stones' are typically the 'triple phosphate' stones whose formation is aided by infection with urea-splitting bacteria such as Proteus.E
(A) Incorrect. Calcium oxalate crystals are quite common in small numbers in urine, even of persons without any renal disease.
(D) Incorrect. Uric acid stones form in acid urine and may be seen in some persons with hyperuricemia and gout. |
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Term
A 60 year old male was diagnosed last year with adenocarcinoma of the lung, and a 4 cm mass lesion was treated with a right lower lobectomy. He now has an abdominal CT scan that reveals scattered hepatic mass lesions and hilar lymphadenopathy. For several weeks, he has had increasing malaise. A urinalysis reveals 4+ proteinuria, and his 24 hour urine protein is 2.7 gm. His serum urea nitrogen is 55 mg/dL with creatinine of 6.1 mg/dL. A renal biopsy is performed, and there is focal deposition of IgG and C3 with a granular pattern. He is most likely to have:
A Membranous glomerulonephritis
B Rapidly progressive glomerulonephritis
C Nodular glomerulosclerosis
D Goodpasture's syndrome
E Membranoproliferative glomerulonephritis, type II |
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Definition
CORRECT. Most cases of membranous GN are idiopathic, but in some patients there is a history of an infection or a malignancy (usually lung) with antigenemia.A
(B) Incorrect. RPGN is more typical for a disease such as Goodpasture's, with anti-glomerular basement membrane antibody |
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Term
Of the following congenital urinary tract anomalies, which carries the greatest significant risk for the most morbidity for the patient:
A Unilateral renal agenesis
B Bladder exstrophy
C Bilateral ureteral duplication
D Horseshoe kidney
E Medullary sponge kidney |
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Definition
| CORRECT. This is serious, and requires surgical repair. The exposed bladder predisposes to infection. There is a long-term risk for carcinoma.B |
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Term
Renal papillary necrosis is most likely to be a complication of all of the following conditions EXCEPT:
A A 55-year-old male with diabetes mellitus who develops a urinary tract infection
B A 60-year-old male who has ingested large quantities of acetaminophen and phenacetin
C A 19-year-old African-American male who has sickle cell anemia
D A 25-year-old pregnant female who has bacteriuria
E A 48-year-old male who has a blood pressure of 180/100 |
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Definition
| CORRECT. Hypertension does not lead to papillary necrosis.E |
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Term
| renal biopsy reveals Subepithelial electron dense deposits |
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Definition
| Such deposits are more typical for post-streptococcal glomerulonephritis. |
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Term
A 70-year-old male with hematuria has a 2 cm filling defect in the left renal pelvis on an intravenous pyelogram. This is most consistent with a lesion that led to:
A Polycythemia
B An elevated white blood cell count
C Nephrotic syndrome
D Hypertension
E Atypical cells on urine cytology |
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Definition
CORRECT. The mass is probably a transitional cell carcinoma, and atypical cells would appear in the urine.E
(A) Incorrect. Polycythemia would be more of a feature expected with a renal cell carcinoma. |
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