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133 Cards in this Set

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Correct Answer
An impending myocardial infarction was successfully averted by thrombolytic (clot-dissolving) therapy in a 55-yo man. Which of the following biochem events most likely occurred during the period of hypoxia?
Inhibition of anaerobic glycolysis and glycogenolysis
The sequence of events in hypoxic cell damage is as follows: hypoxia results in failure of oxidative phophorylation, with resultant depletion of ATP and increase in AMP and ADP. Anaerobic glycoclysis and glycogenolysis are stimulated (not inhibited) through increased phophorfructokinase and phophorylase activities, respectively. THis resluts in accumulation of cell lactate, with a decrease in intracellular pH and depletion of cellular glycogen stores. Decreased availablility of ATP also results in failure of the Na+K+-ATPase pump, which then leads to increased cell Na+ an dwater and decreased cell K+
Which of the following would be the most likely cellular change to be found if it were possible to examine microscopic sections of the heart in a PT whose myocardium was entirely "saved" by immeditate thrombolytic therapy?
Swelling of the endoplasmic reticulum
If infarction is averted by immediate thrombolytic therapy, then indicators of necrosis, such as karyorrhexis, pyknosis, and karyolysis, which represent irreversible changes, would not be expected. Swelling of the ER from increased cell water, one of the earliest ultrastructural changes observed in injured cells, is reveresible and would be expected.
All of the following abnormal clinical manifestations are correctly paired with the appropriate compound or pigment deposited EXCEPT: a. CCl4 poisoining - fat, b. hemolytic anemia -bilirubin, c. herediatry hemochromatosis - hemosiderin, d. vitiligo - melanin, e. atrophy of hepatic and myocardial cells -lipfuscin.
Vitiligo is characterized by focal, patchy arease of depigmentation due to the loss of melanocytes with consequent decreased melanin. CCl4 poisoning is associated with hepatic fatty changed due to impaired apoliproprotein synthesis. Hemolytic anemia leads to jaundice (accumulation of bilirubin in the blood and tissues). Herediatary hemochromatiosis is characterized by massive accumulation of hemosiderin with consequent organ damage, especially in the liever, heart, and pancreas. An accumulation of lipfuscin in hepatic and myocardial cells in characteristic of the brown atrophy of these organs that is sometimes observed in the elderly.
Which of the following sites is an example of metastatic calcification?
The kidney in nephrocalcinosis
Nephrocalcinosis is caused by hypercalcemia and is thus an example of metastatic clacification. The other choices aer all examples of Dystrophic calcification, calcification in arease of prior tissue damage
An appendectomy is performed on a 16 yo boy who is hopitalized for right lower quadrant abdominal pain of 18 hours duration. The surgical specimen is edematous and erythematous. An infiltrate of which of the following cells would be most characteristic of the process occurring here?
The clinical scenario is illustrative of a PT with acute appendicitis, a classic example of acute inflammation. Neutrophils are the most characteristic cellular component of the early stages of acute inflammation
Which of the following is NOT a mediator of degranulation of basophils and mast cells? A. AGEPC, b. complement componenets, c. immunoglobin E, d. interleuking-1, e. physical stimuli
AGEPC, or platelet-activating factor (PAF) is a result of basophil and mast cell degranulation and is not a mediator of degranulation. PAF induces platelet aggregation; mediates vasoconstriction, vasodilation, and increased capillary permeability; activates arachidonic acid metabolism; and causes bronchospasm
A 38 yo HIV-pos man is hospitalized because of acough, hemoptysis, and weight loss. Chest roentgenogram reveals a right sided apical infiltrate with cavitation. Which of the following findings is an invariable hisotlogic feature of the probable causative lesion?
epithelioid cells
The clinical vignetted is a classic description of pulmonary TB, which is often characterized by otherwise unexplained weight loss, cough, and hemoptysis. The pulmonary apices are favored sites for TB, and cavitation is common. Ther has been a rectne upsurge in the incidence of TB, especially associated with HIV-pos. Granulomas always consist of groups of modified macrophages called epithelioid cells. Caseous necrosis and Langhans giant cells are especially suggestive of TB etiology but ar not always found. although a definitive diagnosis is facilitated by the deomstration of organisms, this is not always possible. A cuff of lymphocytes often (but not always ) surrounds each granuloma
Match the action with the mediator: Endothelial cell contraction. A. histamine, b. bradykinin, c. C3a and C5a, d. C3b
In the early stages of actue inflammation, histamine mediates the contraction of endothelial cells, which widesn the interendothelial gaps of postcaplillary veules, increasing vascularpermeability. Histamine is dervied mainly from the granules of basophils and mast cells and also from platelets.
A 40 yo woman died after a long history of illness characterized by dyspnea, or thopnea, hepatomegaly, ditended neck veins, and peripheral edema. The cut surfacte of the liver as it appeared at autopsy is shown in the illustration. Which of the following disorders is the most likely cause of these findings?
Right heart failure
The clinical findings described in the ? Are typical of right heart failures, as is the illlustration, which reveals the nutmeg-like appearance of hepatic chronic passive congestion. The gross morhpologic appearance is caused by congested centrilobular areas alsternating with pale portal areas
Which of the following causes of an increased tnedency to venous thrombosis is acquired rather than hereditary?
antiphospholipid Ab syndrome
Antiphopholipid abs are the most common acquired defects in blood proteins associated with venous thrombossi. Additionally, many others acquired factors (eg surgery, malignancy, oral contracteptie use) are associated with venous thrombosis. Seveal hereditary abnormalities are associated with increased thrombosis. Of thses, the factor V Leiden mutation resulting in resistance to activated protein is the most common.
Which of the following substances does NOT contribute to the antithrombotic (thrombo resistnat) properties of vascular endothelium? A. von Willebrand factor, b. prostacyclin, c. heparin-like molecules, d. thrombomodulin, e. protein C
von Willebrand factor
von Willebrand factor is important in the binding of platelets to surfaces such as subendothelial collagen and is thus prothrombotic and not a contributor to endothelial thromoresistance. In contrast, the other choices listed are antithrombotic. Heparin-like molecules inhibit coagulation by activation of antithrombin III, which in turn inhibits thrombin and factors IXa and Xa. The thrombomodulin-thrombin complex activates protein C. Activated protien C inhibits coagulation by proeolysis of factors Va and VIIIa.
The rational for low-dose aspirin prophylaxis of coronary artery disease is based on the resultant decreased synthesis of
Both TxA2 and PGI2 are products of the cylcooxygenase pathway of arachidonic acid ametabolism, and synthesis of both is hinibited by aspirin, a cyclooxygenase inhibitor. In this setting, TxA2 inhibition is desirable because platelet aggregation is an important early step in atherogenesis. Inhbititon of PGI2 synthesis is an undesirable side effcet of aspirin porphylaxis, because PGI2 is a potent platelet anti-aggregant.
Which of the following disorders I more liekly to be associated with an exudate rather than a transudate?
Edema resulting from inflammation is caused by increased capillary permeability, and the protein-rich, high-specific-gravity, glucose-poor, cell-laden edema fluid is called an exudate. IN contrast, a transudate results form increased intravascular hydrostatic pressure or from altered osmotic pressure. A transudate is charcterized by low protein content and low specific gravity. congesttive heart fialure, the nephortic syndrom, and chronic liver disease are classic causes of generalized transudation.
Which of the following conditions results in hypovolemic shock?
severe hemorrhage
The reduction in circulating blood volume, which occurs with severe hemorrhage, cuases shock from reduction in cardia output and is the classic example of hypovolmeic shock. Reduced volume is normal or even increased. The shock associated with gram neg sepsis (spetic shock) is marked by peripheral vasodilation and peripheral pooling, as is the neurogenic shock resulting from head injury.
All of the following disorders are transmitted by X-linked inheritance EXCEPT: a. fabry disease, b. leshc-nyhan syndrome, c. glucose 6 phophate dehydrogenase deficiency, d. classic hemophilia (hemophilia A), e. von Gierke disease
von Gierke disease
von Gierke disease is a form of glycogen storage disease inherited as an autosomal recessive characteristic
Characteristics or phenomena associated with acute rejection after renal transplantation include all of the following EXCEPT: a. occurs days to months after taransplantation, b. T cell-mediated, c. localized Arthus reaction, d. mononulcar infiltrates, e. arteritis with thrombosis
localized Arthus reaction
a localized Arthus reaction (localized immune complex reaction that occurs whne exogenous antigen is inserted in the midst of performed ab) is the cause of the almost immediate phenomenon called hyperacute rejection. In contrast, actue rejection occurs days to months after transplantation and is usually T cell-mediated
Bruton disease is characterizd by all of the folowing manifestations EXCEPT: a. absent circulating B cells, b. normal circulating T cells, c. absent plasma cells, d. low serum levels of immunoglobulin, e. impaired resistance to viral infections
impaired resistance to vrial infections
this disease of male children is X-linked and is decriptively called X-linked agammaglobulinemia of Bruton. T cells are unaffected, as are T cell functions such as cell-mediated immunity and resistance to viral infections. Failure of maturation of pre-B cells is associateed with absence of Bl ymphocytes and plasma cells, failure of ab synthesis with erslultant serum agammaglobulinemia, and recurrent bacterial infections
Each of the following disease stes is matched with the correct manifestation or association EXCEPT: a. severe combined immunodeficiency disease - adenosine deaminase deficiencey, b. isolated IgA dficiency - rxns to blood transfusions, c. Wiskott-Aldrich syndrome - thrombocytopenia, d. DiGeorge Syndrom - autosomal dominant inheritance, e. AIDS - reveresed CD4+:CD8+ ration
DiGeorge syndrome - autosomal domiant inhertiance
DiGeorge syndrome is a congenital developmental defect possibley caused by intrauterine infection
Each of the following disease entities is correctly paired with the appropriate ab EXCEPT: a. SLE - anti-Sm ab, b. Sjogren syndrom - anti-SS-B ab, c. mixed connective tissue disease - anti-nRNP ab, d. polyarteritis nodosea - ab to ds-DNA, e. progressive systemic sclerosis - anti-Scl 70 ab
polyarteritis nodosa - Ab to ds-DNA
Abs to ds-DNA and to the Sm ag are highly specific findins in SLE bu NOT in poyarteritis nodosa
Which of the following findings is characterisitc of secondary (reactivesystemic) amyloidosis?
Association with crhonic inflammatory disease
Reactive systemic amyloidosis occures as a secondary phemomenon to long-standing chronic innflammatory disorders such as rheumatoid arthritis, TB, and osteomylelitis. The characteristic amyloid protein si formed from a rprecursor, serium amyloid-associated protein. Organ distruibution is primarily in parenchymatous organs
All of the following disease that predispose to amyloidosis are correctly mathced with the apporoprieate amyloid protein EXCEPT: a. rheumatoid arthritis - AA amyloid, b. multiple myeloma - AL amyloid, c. medullary carcinoma of the thyroid - AA amyloid, d. Alzheimer disease - A4 amyloid, e. senile amyloidosis of the heart - transthyretin
medullary carcinoma of the thyroid - AA amyloid
IN medullary carcinoma of the thyroid, amyloid is apparently a catabolic product of caclitonin and is NOT the AA amyloid associated with reheumatoid arthritis and other chronic inflammatory dosrders. The AL amyloid associated with plasmas cell doriders usch as multiple myeloma is derived from immunoglobulin light chains. Alzheimer disease is noted by the pressence of amyllid plaquies containing A4 amyloid. Senile amyloidosis, when it occurs in the heart, is characterized by amyloid deposits dervied from transthyretin.
A biopsy of the stomach reveals an area of normal-appearing pancreatic tissue. This is an example of which of the following lesions?
An area of normal-appearing pancreatic tissue in the stomach is an example of a choristoma, which is an area of normal tissue withing another organ
Clinical evidence supporting the monoclonalilty of tumors can be dreived from evaluation of all of the following substances EXCEPT: a. G6PD isoenzymes, b. immunoglublin light chains, c. immunoglublin or T cell receptor genes, d. hormone production, e. serum proteins
hormone production
Although production of specific homrones, such as prolactin, parathryoid hormone, or human chorionic gonadotropin, may be a highly specific characteristic of certain neoplasms, it has no relationship to monoclonality. Clinical evidence supporting the monoclonality of tumors can be derived from evaluating substances such as G6PD isoenzymes, immunoglobulin light chains, immunoglobulin or T cell receptor genes, and serum proteins
All of the following statements about carcinogensis are true EXCEPT: a. chemical carcinogenesis is usually a multistep phenomenon, b. initiators cause irreversible damage ot DNA, c. promoters of carinogensis induce cll proliferation, d. promoters of carcinogenesis exert their effect before intiation
promoters of carcinogenesis exert their effect efore intiation
Carcinogenesis occurs in multiple steps. Initiation, the first carcinogenic event, si almost always a reaction between a dirct-recating, or ultimate, carcinogen and DNA. Promoters act at a later stage by stimulating increased cellular replication
Which of the following neoplasms is associated with gene amplification?
Neuroblastoma, a frequently occurring adrenal tumor of early childhood, is associated with marke amplification of the N-myc oncogen. The reduplication is so marked that it can often be cisualized in karyotypic preparations
Which of the following charaterisitcs is the most reliable indicator of prognosis of malignant tumors?
degree of localization
The degre of tumor localization is the basis of staging whtich, in general, correlates better with the prognosis than does histopathologic grading which is bsed on differentiation nad other morphologic changes
Which one of the following malgnant neoplasms is corerctly paired with the appropriateparaneoplastic manifestation?
squamous cell carcinoma of the lung - hypercalcemia
Squamous cell carcinoma of the lung is often associated with secretion of parathyroid hormone-like substances, with resultant hypercalcemia.
Each of the following associations of an injury and a consequence is correct EXCEPT: a. head trauma - contrecoup injury, b. contact gunshot wound to skull - exit wound larger than entry wound, c. multiple factures - fat embolism, d. crush injury of skeletal muscle - acute tubular necrosis, e. sever bruns - acute gastric ulcer.
contact gunshot wound to skull - exit wound larger than entry wound
In contact gunshot wounds overlying unyielding bony surfaces such as the skull, the entry wound is often larger than the exit wound, due to the effect of expanding gases under the skin. This is in contrast to through-and-through gunshot wounds, where the reverse is true. Brain contusion in head injuries can occur at the point of impact (coup injury) or on the opposite side of the brain (contracoupd injury). Multiple factures can be complicated by fat emboli. Skeletal muscle crush injury can reslut in a acute tubular necrosis with myoglobin casts. Severe burns may be associated with acute ulceration of the gastric mucosa referred to as a Curling ulcer.
Each of the following is a characteristic of kwashiorkor EXCEPT: a. severe edema, b. scant subcutaneous fat, c. muscle wasting, d. growth retardation, e. fatty liver
scant subcutaneous fat
Kwashiorkor is the result of severe protein deprivation in the face of adequate caloic intake. Subcutaneous fat tissue is generally unaffected because sufficient calories are supplied for fat synthesis. Protein deficiency results in edema (largely secondary to hypoproteinemia; it is also caused by decreased cardiac output and renal blood flow iwth compensatory activation of the angiotensin-aldosterone system), muscle wasting, growth retardation, and fatty liver (due to failure of apoprotein synthesis).
Glossitis, corneal vscularization, cheilosis and seborrheic dermatitis are manifestations of dietary deficiency of:
Glossitis and dermatitis are manifestations common to deficienceis of the B complex group of water-soluble vitamins. Cheilosis and corneal vascularization are especially suggestive of riboflaving deficiency.
Diarrhea, dermatitis, and dementia are characteristics of deficiency of:
Diarrhea, dermatitis, and dementia (the 3 Ds) are the classic triad of pellagra (clinicla niacin deficiency). The dermatitis tends to involve areas exposed to sunlight.
Monckeberg arteriosclerosis is marked by all of the following EXCEPT: a. it occurs most often inelderly PTs, b. caclifaications occur in the arterial media, c. is predominantly seen in readial and ulnar arteries, d. significatn vascular obsturction occurs
significant vascular obstruction occurs
Monckerberg arteriosclerosis is usually a nonobstructing lesion because the arterial intima is not involved. THe condition is often seen in elderly persons in whom medium-sized muscular arteries, typically the radial and ulnar arteries, become stiffenedby calcification withing the media, resulting in so-called "pipestem" arteries.
The lesion shown in the illustration below is associated with which one of the following disorders?
The incidence of atherosclerosis is strongly associated with hypercholesterolemia. The figure shows a large atheromatous plaque narrowing the lumen of an artery. The plaque consists of a mixture of fibrous tissue and cleft-like spaces indicating the presence of cholesterol crystals
All of the following vascular lesions are correctly matched with thea ppropriate clinical association EXCEPT: a. hemangiosarcoma - admin of Thorotrast, b. cystic hygroma - cervical and axillary masses, c. glomus tumor - painful subungual nodule, d. herediatry hemorrhagic telangiectasia - X-linked inheritance, e. Kaposi sarcoma - AIDS.
Hereditary hemorrhagic telangiectasia - X-linked inheritance
Hereditary hemorrhagic telangiectasia is an important autosomal dominant condition often presenting as bleeding of obscure origin
Hepatitis B virus is frequently associated with which of the following diseases?
Polyarteritis nodosa
Polyarteritis nodosa is an immune complex disease. The ag is most often unkown. Hepatitis B surface ag is found in approximately 30% of cases and may well be the ag involved int eh immune complexes
A child with fever, arthralgias, gastrointestinal and renal involvement, and hemorrhagic urticaria-like lesions of the skin most liekly is exhibiting
Henoch-Schonlein purpura
The hemorrhagic urticaria-like lesions (palpable purura) place this disorder within the hypersensitivity vasculitis group. Associated GI and renal abnormalities are particulartly characterisitc of Henoch-Schonlein purpura
Match complication with most likely associated: Aortic valvular insufficiency. A. congenital vascular muscle weakness, b. atherosclerosis, c. syphilis, d. hypertension, e. penetrating wounds
Syphilitic aortitis characteristically involces the proximal thoracic aorta, resulting in dilation, often with aneurysim formation. Dilation of the adjacent arotic valve results in aortic insufficiency. Although once the most common cause of these lesions, syphilis rarel ycauses this now because of treatment and control
Match complication with most likely associated: Aneurysm of abdominal aorta. A. congenital vascular muscle weakness, b. atherosclerosis, c. syphilis, d. hypertension, e. penetrating wounds
Ahteroslcerosis is the most common cause of aortic aneurysm, and the abdominal aorta is the most frequent site of involvement
Match complication with most likely associated: High-output cardiac failure. A. congenital vascular muscle weakness, b. atherosclerosis, c. syphilis, d. hypertension, e. penetrating wounds
Penetrating wounds
Penetrating wounds can result in arteriovenous fistula formation, which in turn is associated with hypervolemia and high-output cardiac failure
Match complication with most likely associated: Dissecting arotic aneurysm. A. congenital vascular muscle weakness, b. atherosclerosis, c. syphilis, d. hypertension, e. penetrating wounds
Hypertension predisposes to disseicting aneurysm, as does cystic medial necrosis
A myocardial infarct that exhibits early granulation tissue hs most likely occurred
within 1 week
By the end of the 1st week afer a MI, early granulation tissue is seen at the periphery of the necrotic area, followed shortly by the appearance of newly formed collagen. Granulation tissue is gradually replaced by scar tissue withing several weeks
Following acute MI, rupture of the L ventricle is most likely to occur within
4-7 days
Rupture of the L ventricle, a catastrophic complication of acute MI, usually occurs when the necrotic area has the least tensile strength, which is at about 4-7 days after an infarction when repair is just beginning. The anterior wall of the heart is the most frequent site of rupture, usually leading to fatal cardia tamponade. Internal rupture of the interventricular septum or of a papillary muscle may also occur.
The most common cause of death immediately after the onset of actue MI is
Arrythmias are the most IMP early complication of acute MI, accounting for almost 50% of deaths shortly after MI.
The myocardial lesions shown in the illustration below can be observed in PTs with which one of the following conditions?
The figure illustrates an Aschoff body, the characteristic lesion of rheumatic fever. This myocardial lesion is most often oval in shape and cahracterized by swollen framgented collagen and fibrinoid material and y characterisitc large mesenchymal cells (Anitschkow myocytes) and multinucleated cells (Aschoff cells). sydenham chorea is a major namifestation of rheumatic fever
Most of the deaths that occur during acute rheumatic fever are c/b
The most common cause of death that occurs during acute rheumatic fever is cardiac failure secondary to myocarditis
Which of the following conditions characteristically results in aortic valvular insufficiency?
syphillitic heart disease
In syphiltic aortitis, the elastica of the aorta is replaced by fibrous tissue, resulting in dilation of the ascending aorta and separation of the aortic commissures, with resultant aortic insufficiency.
Mitral stenosis most often results from
rheumatic endocarditis
Mitral stenosis is most often a late sequela of rheumatic fever. Bacterial endocarditis is a complication, no a cause, of mitral stenosis. Congenital heart disease is sometimes complicated by mitral stenosis, which is often of rheumatic origin. Marfan syndrom is sometimes associated with mitral valve prolapse. syphilitic heart disease is characterized by widening of the aortic commissures, resulting in aortic insufficiency.
Cyanosis in congenital heart disease is c/b
a R to L shunt
Cyanosis, which occurs when the arterial concentration of reduced Hgb exceeds 5 mg/ml is seen with a R to L shunt in which venous blood gains direct access to the arterial circulation.
Which of the following conditions is associated with paradoxic embolism?
atrial septal defects
The term paradoxic embolism denotes the passage of an embolus of venous origin into the arterial circulation through a R to L shunt (ex patent foramen ovale, atrial septal deftct). The likelihood of R to L passage of the embolus is foten enhanced by pulmonary hypertension secondary to pulmonary thromboembolism.
A 42 yo man is seen because of a long hx of slowly developing congestive heart failure. His blood pressure is normal. Coronary artery angiography reveals no vascualr disease. No heart mrumurs are heard. The WBC count, diff and erythrocyte seidmentation rate are normal. THe most likely diagnosis is
Cardiomyopathies are noninflammatory myocardial disroders not associated with coronary artery obstruction, hypertension, valvular disease, congenital heart diesease, or infectious disease. They are most often charcterized by otherwise unexplained ventricular dysfxn such as cardiac failure, ventricular enlarement, or ventricular arrythmias.
Which of the following diseases is classified as a restrictive cardiomyopathy?
amyloid heart disease
Restrictive, or obliterative -restirctive, cardiomyopathy is characterized by restricted ventricular filling, most often associated with amyloidosis. Congestive, or dilated, cardiomyopathy is characterized by biventricular dialtion associated with alcoholism, thiamine deficiency (beriberi), and cobalt toxicity. Hypertrophic cardiomyopathy is characterized by marked increase in ventricular wall thickness with small ventricular cavities, often with outflow tract obsttruction, as in asymmetric septal hypertrophy.
All of the following pairings of red cell Rh types and ABO groups of mother and child may lead to hemolytic disease of the newborn EXCEPT: A. mother cde, A; child cDe, O. B. mother CDe, AB; child cdE, O. C. mother cde, O; child CDe, A. D. mother cDE, O; child Cde, A. E. mother Cde, B; child cDe, B.
mother CDe, AB; child cdE, O.
Hemolytic disease of the newborn does not occur when the mother is D (Rh-positive) and the child is d (Rh-negative) or when the mother is AB and the child is O. Rh-system incompatibility is seen when the mother is d (Rh-negative) and the child is D (Rh-positive). ABO system incompatibility is seen when the mother is O and the child is A, B, or AB. FROM TEXT: Hemolytic disease of the newborn (erythroblastosis fetalis) --results from maternal alloimmunization to fetal red cell antigens, classically the D antigen of the Rh blood group system. In the most frequently occurring form of Rh-mnediated hemolytic disease of the newborn, the mother is typed as d and the fetus as D. --can also result from ABO incompatibility. In most instances of ABO incompatibility, the mother is blood group O and the child is blood group A or group B. Other incompatible combinations include: mother A, child B or AB; mother B, child A or AB. --occurs when maternal antibodies cross the placenta and react with fetal red cells, resulting in fetal hemolytic anemia. --can result in stillbirth or in hydrops fetalis, fetal heart failure with massive generalized edema. --can result in kermicterus, staining of the basal ganglia and other centra nervous system structures by unconjugated bilirubin. Kernicterus with resultant neurologic damage is the most significant long-term consequence of hemolytic disease of the newborn. --has been markedly reduced in incidence by preventive measures. Routine administration of anti-D IgG antiserum to D-negative mothers at the time of deliver (or at the time of termination of pregnancy) of a D-positive child results in the antibody-mediated removal of fetal red cells from the maternal circulation, preventing maternal alloimmunization.
Ineffective erythropoiesis is characteristic of which one of the following conditions?
beta-Thalassemia major
Ineffective erythropoiesis, red cell destruction in marrow prior to red cell release, is a major characteristic of beta-thalassemia major and is caused by aggregation of excess alpha-chains. FROM TEXT: BETA-THALASSEMIAS (A TYPE OF HEMOLYTIC ANEMIA): --are the most common forms of thalassemia found in Mediterranean areas and in the United States. --are caused by defects in the promoter sequence, in introns, or in coding regions of the beta-globin gene. Beta-Thalassemia major: --is also known as Mediterranean anemia or Cooley anemia. --results from compound heterozygosity or homozygosity for thalassemic variants of the beta-globin gene. --is characterized clinically by: (1) marked anemia resulting from: (a) Modest decrease in hemoglobin synthesis (b) Marked shortening of red cell life span due to aggregation of insoluble excess alpha-chains (c) INEFFECTIVE ERYTHROPOIESIS (FROM QUESTION STEM) (d) Relative folate deficiency (2) Marked splenomegaly (3) Distortion of skull, facial bones, and long bones because of erythroid marrow expansion (4) Thalassemic red cell morphology (marked microcytosis, hypochromia, target cells, extensive changes in size and shape) (5) Increased hemoglobin F (alpha2gamma2) throughout life (6) Generalized hemosiderosis due to chronic hemolysis, ineffective erythropoiesis, and repeated transfusions *MY INCORRECT ANSWER: ANEMIA OF CHRONIC DISEASE --is a common form of anemia, second in incidence to iron deficiency anemia. --can be secondary to a wide variety of primary disorders, including rheumatoid arthritis, renal disease, or chronic infection. --is often normochromic and normocytic --when associated with renal disease, may be moderately macrocytic. --when associated with chronic inflammatory states (e.g., rheumatoid arthritis), may be accompanied by decreased serum iron and hypochromia and microcytosis, mimicking iron deficiency anemia; however, IN CONTRAST TO IRON DEFICIENCY ANEMIA, TIBC IS CHARACTERISTICALLY DECREASED IN ANEMIA OF CHRONIC DISEASE.
increased erythropoietin (false)
thrombocytosis (true)
The combination of erythrocytosis, leukocytosis, and thrombocytosis along with splenomegaly is highly characteristic of polycythemia vera. Polycythemia vera is a clonal proliferative disorder characterized by a decreasxe in erythropoietin concentration. FROM TEXT: MYELOPROLIFERATIVE DISEASE: B. POLYCYTHEMIA VERA 1. Clinical characteristics a. Marked erythrocytosis b. Moderate increase in circulating granulocytes and platelets c. Splenomegaly d. Decreased erythropoietin 2. Other features: a. Sluding of high hematocrit blood often leads to thrombotic or hemorrhagic phenomena b. Polycythemia vera often progresses to a late phase, in which anemia supervenes. This phase is often marked by bone marrow fibrosis and extramedullary hematopoiesis and a rising white bloood cell count, and it can mimic CML. c. Acute leukemia may supervene in approximately 3% of patients, most of whom have received antimitotic drugs or radiation therapy. 3. Diagnosis--polycythemia vera: --is marked by DECREASED ERYTHROPOIETIN (MY INCORRECT ANSWER), which distinguishes it from other forms of polycythemia, all of which are associated with increased erythropoietin. --must be distinguished from secondary polycythemia, which is associated with the following: a. Chronic hypoxia, associated with pulmonary disease, congenital heart disease, residence at high altitudes, and heavy smoking. b. Inappropriate production of erythropoietin, associated with androgen therapy, adult polycystic kidney disease, and tumors, such as renal cell carcinoma, hepatocellular carcinoma, and cerebellar hemangioma. c. Endocrine abnormalities, prominently including pheochromocytoma and adrenal adenoma with Cushing syndrome.
Chronic Lymphocytic Leukemia (CLL)
Chronic Myelogenous Leukemia (CML)
The illustration shows predominance of mature-appearing lymphocytes and several cells distorted mechanically in preparation of the blood smear (smudge cells), both characteristic of Chronic Lymphocytic Leukemia (CLL). CLL most often affects older persons, many of whom are asymptomatic for many years. Generalized lymphadenopathy and hepatosplenomegaly are frequent findings. FROM TEXT: C. Chronic Leukemias: --are characterized by proliferations of lymphoid or hematopoietic cells that are more mature than those of the acute leukemias. --have a longer, less devastating clinical course than the acute leukemias but are less responsive to therapeutic intevention. 1. Chronic Lymphocytic Leukemia (CLL) a. General Considerations (1) CLL is characterized by a proliferation of neoplastic lymphoid cells (almost always B cells) with widespread infiltration of the bone marrow, peripheral blood, lymph nodes, spleen, liver, and other organs. (2) Leukemic cells are less capable of differentiating into antibody-producing plasma cells. (3) CLL most often occurs in persons older than 60 years of age and more frequently in men. b. Characteristics (1) The leukemic cells closely resemble normal mature peripheral blood lymphocytes and, like these cells, express surface immunoglobulin and pan-B cell markers, such as CD19 and CD20. They are also CD5 positive and CD10 negative. (2) The cells are susceptible to mechanical disruptioni and often appear on the peripheral blood smear as smudge cells. (3) The peripheral white blood cell count varies from 50,000/ul to 200,000/ul, with a preponderance of leukemic cells. (4) Leukemic cells diffusely infiltrate the bone marrow. c. Complications (1) Warm antibody autoimmune hemolytic anemia. (2) Hypogammaglobulinemia and increased susceptibility to bacterial infection, often occurring early in the course of this disorder. d. Clinical features (1) The clinical course is usually described as indolent, often with few symptoms and minor disability for protracted periods. (2) Generalized lymphadenopathy and moderate hepatosplenomegaly are frequent features. (3) Mean survival is 3 to 7 years; treatment relieves symptoms but has little effect on overall survival. C. Chronic Leukemias: 3. Chronic Myelogenous Leukemia (CML) --is a neoplastic clonal proliferation of myeloid stem cells, the precursor cells of erythrocytes, granulocytes, monocytes, and platelets. --is one of the myeloproliferative syndromes. a. Molecular changes (1) CML is characterized by a reciprocal chromosomal translocation between chromosomes 9 and 22. The Philadelphia chromosome represents a remnant of chromosome 22 with the addition of a small segment of chromosome 9. This cytogenetic change is found in all blood cell lineages (erythroblasts, granulocytes, monocytes, megakaryocytes, B and T cell progenitors) but not in the majority of circulating B or T lymphocytes. (2) The c-able proto-oncogene on chromosome 9 is transposed to an area on chromosome 22, adjacent to an oncogene referred to as bcr (for breakpoint cluster region), forming a new hybrid, or fusion, gene bcr-abl. (3) Bcr-able codes for a protein (p210) with tyrosine kinase activity, which plays a critical role in the etiopathogenesis of CML. b. Characteristics. (1) Marked leukocytosis, with white blood c ell counts varying from 50,000/uL to 200,000/uL. (2) Leukemic cells in the peripheral blood and bone marrow, mainly middle-to-late myeloid (granulocytic) precursor cells, including myelocytes, metamyelocytes, bands, and segmented forms (3) Small numbers of blasts and promeylocytes (4) The Philadelphia chromosome, found in granulocytic and erythroid precursor cells and in megakaryocytes. (5) Marked reduction in leukocyte alkaline phosphatase activity in the leukemic leukocytes. c. Clinical Features (1) Prominent splenomegaly and modestly enlarged liver and lymph nodes. (2) Peak incidence in middle-age group (ages 35-50) (3) Terminates, in most cases, in an accelerated phase leading to so-called blast crisis marked by increased numbers of primitive blast cells and promyelocytes.
cell surface markers indicating T cell lineage (false)
8;14 translocation (true)
Burkitt lymphoma is a neoplasm of mature B cells. FROM TEXT: b. Non-Hodgkin lymphomas --are malignant neoplasms arising from lymphoid cells or other cells native to lymphoid tissue. --originate most frequently within lymph nodes or in other lymphoid areas. Tumor involvement of the periaortic lymph nodes is frequent. 8. Burkitt lymphoma --is an aggressive B cell lymphoma. The African form frequently involves the maxilla or mandible; the American form usually involves abdominal organs. --is closely linked to EBV infection (especially in the African variety). --is characterized histologically by a "starry-sky" appearrance. As a result of rapid cell turnover, the lesions contain abundant cellular debris that is taken up by non-neoplastic macrophages, resulting in this appearance. --is closely related to B-ALL (Acute Lymphoblastic Leukemia of late-stage B cell origin), which is called Burkitt cell leukemia in the WHO classification. --is associated with a characteristic cytogenic change, t(8; 14) a. In this translocation, the c-myc proto-oncogene located on chromosome 8 is transposed to a site adjacent to the immunoglobulin heavy-chain locus on chromosome 14. b. Increased expression of the c-myc gene, presumably caused by the proximity of regular sequences of the immunoglobulin heavy chain gene, is characteristic.
No missed answers!
All of the following disorders are correctly paired with realted charactersitics EXCEPT: a. atelectasis - bronchial obstruction, b. adult resp distress - hyaline membranes, c. cystic fibrosis - a1 antitrypsin deficiency, d. acute epiglottitis - Haemophilus influenzae, e. asbestosis - ferruginous bodies
cystic fibrosis - a1 anti-trypsin deficiency
CF is associated with a mutation in the CF transmembrane conductance regulator (CFTR) gene; it is not associated with a- anti-trypsin deficiency
Adult resp distress sydnrome is c/b all of the following EXCEPT: a. shock, b. sepsis, c. inhalation of toxic irritants, d. bronchial obsturction by foreing body, e. head injury
bronchial obstruction by foreign body
Although bronchial obstruction by a foreign body might result in atelectatic collapse of the pulmonary parenchyma distal to the obstruction, the diffuse alveolar damage and increased capillary permeability characteristicof ARDS would be an unlikely complication of this occurrence. Vearious conditions can cause this syndrom, including shock, sepsis, inhalation of toxic irritants, and heady injury
All of the following disorders are correctly paired with realted charactersitics EXCEPT: a. sarcoidosis - npncaseating granulomas, b. idiopathic pulmonary hemosiderosis - antiglomerular basement membrane abs, c. idiopathic pulmonary fibrosis - immune complex disease, d. eosinophilic granuloma - Birbeck granules, e. goodpastrue syndrome - renal lesions
idiopathic pulmonary hemosiderosis - antiglomerular basement membrane abs
Idiopathic pulmonary hemosiderosis is characterized by hemorrhagic lung involvement similar to Goodpasture syndrome, but notable diffences include the absence of antiglomerular basement memberane abs or renal involvement
A patchy distribution of lesions throughout the lungs is characteristic of pneumonia c/b all of the following agents EXCEPT: a. staph aureus, b. viruses, c. mycoplasma pneumonia, d. Stre pneumoniae, e. Haemophilus influenzae
Strep pneumoniae
Patchy distribution of lesions throughout the lungs is charcaterisitc of bronchopneumonia, which is c/b a nubmer of organisms, most notably S. arueaus and H. influenzae. A similar distribution is observed I the interstitial penumonias c/b viruse and mycoplasma. In contrast, involvement of a single lobe, often in its entirety (lobar pneumonia), is chactertisitc of infection with S. pneumoniae.
All of the following types of pneumonia are correctly paired with related findings EXCEPT: a. Strep pyogenes pneumonia - lobar involvement, b. staphylococcal pneumonia - absecess formation, c. Mycoplasma pneumonia - cold agglutinins, d. Haemophilus pneumonia - infants and childresn, e. Klebsiella pneumonia - high mortality in the elderly
Strep pyongenes pneumonia - lobar involvement
The most frequent etiologic agent of lobar pneumonia is S. penumoniae, not S pyogenes. Staphylococcal penumonia is often compilcated by pulmonary or exrapulmonary abscess formation as well as by empyema, bronchopleural fistula, or baterial endocarditis. Diagnosis of Myocplasma penumonia, the most common nonbacterial pneumonia, is facilitated by lab demonstration of nonspecific cold agglutinins directed against red cells as well as by complement-fixing ab s and microbiologic culture techniques. Haemophilus pneumonia is most often observed in infants and young kids. Klebsiella pneumonia has a high mortality rate among hte elderly.
All of the following cuasative microorganisms or disease enttitles are correctly paired with a frequent assoc or effect EXCEPT: a. bronchiectasis -abundatn sputum, b. bronchogenic carcinom - lung abscess, c. Mycoplasma pneumoniae - cold agglutinins, d. Staph aureus - lobar penumonia, e. Strep pneumoniae - exudate in pleural space
Staph aureus - lobar pneumonia
Lobar pneumonia is typically c/b S. pneumoniae. IN contrast, pulmonary infxn with S. aureus is mot often associated with bronchopneumonia, often with fromation of multiple small abscesses.
All of the following disorders are corerctly paired with related charcteristics EXCEPT: a. legionnaries disease - AC systems, b. Q fever - livestock workers, c. ornithosis - richkettsial disease, d. measles - giant cells
Ornithosis - rickettsial disease
Ornithosis, c/b an organism of the genus Chlamydia, is not a rickettsial disease. Legionnaires disease, Legionella pneumophila infxn, has often been traced to contamination of AC systems. Q fever, the most common rickettsial pneumonia, often affects live-stock workers who handle cattle of sheep. Pneumonia fro the measle virus can take the form of giant cell pneumonia
All of the following terms are correctly paired EXCEPT: a. actinomycosis - sulfur granules, b. cyrptococcosis - India ink preps, c.histoplasmossis - overwhelming pneumonia, d. coccidioidomycosis - spherules with endospores, e. nocardiosis - opportunistic infection
Histoplasmosis - overwhelming pneumonia
Histoplasmosis exhibits pulmonary manifestations similar to those of TB, and overwheming pneumonina does not occur. The most frequent form is the asymptomatic primary pulonary disease, marked by a POS skin test result and residual calcifications. Secondary pulmonary histoplasmosis can reslut in cavitary disease, and disseminated histoplasmosis involves the lymphoreticular system.
Select the disorder with its association: Charcot-Leyden crystals. A. bronchial asthma, B. chronic bronchitis, C. emphysema, D. Bronchiectasis
Bronchial asthma
Charcot-Leyden crystals, thought to be derived from eosinophil membran proteins, are found in the sputum in bronchial asthma
Select the disorder with its association: Bronchial submucosal gland hyperplasia. A. bronchial asthma, B. chronic bronchitis, C. emphysema, D. Bronchiectasis
Chronich bronchitis
The characteristic pathologica change in chronic bronchitis is hyperplasia ofbronchial submucosal glands and bronchial smooth muscle hypertorphy, which can be quantified by the Reid index, a ration of landular layer thickness to bronchial wall thickness.
Match the pulmonary disorder with its association: TB. A. progressive massive fibrosis, B. Silicosis, C. asbestosis, D. anthracosis
Pulmonary TB is a major complication of silicosis. The cause of this association is unknown but may be due to altered macrophage activity.
Match the pulmonary disorder with its association: Black fibrotic nodules. A. progressive massive fibrosis, B. Silicosis, C. asbestosis, D. anthracosis
Progressive massive fibrosis
Progressive massive fibrosis, a generalized pulmonary fibrotic change, soemtimes complicates simple coal workers' pneumoconiosis. Progressive massive fibrosis is characterized by fibrotic nofules filled with necrotic black Inida ink-like liquied. The lesions ma yblead to ronchiectasis and pulmonary hypertension.
Which of the following phrases best characterizes ameloblastoma?
Most often involves the mandible
Ameloblastoma, a true neoplasm, most commonly originates within the mandible and less often within the maxilla. The origin is from the epithelial tissue involved in the development of teeth. The tumor is benign and does not metastasize; however, local expansion can have serious consequences
All of the following conditions are associated with Sjogren syndrome EXCEPT: a. mumps parotits, b. keratoconjunctivitis sicca, c. malignant lymphoma, d. rheumatoid arthritis, e. xerostomia
mumps parotitis
Ther is no relation of mups parotitis to Sjogren syndrome. Sjogren syndrome is an autoimmune disorder characterized by keratoconjunctivitis sicca and xerostomia, due to lymphocytic infiltration and parenchymal desturction of the parotid and lacrimal glands in assoc with a connective tissue disorder such as rheumatoid arthritis. Maligant lymphoma is a frequent complication.
All of the following salivary gland tumors are correctly matched with the appropriate characteristic EXCEPT: a. pleomorphic adenoma - most common salivary gland tumor, b. oncocytoma - eosinophilic-staining cells; c. adenolymphoma - Warthin tumor; d. mucepidermoid tumor - "mixed tumor"; e. adenoid cystic carcinoma - presents with pain
mucoepidermoid tumor - "mixed tumor"
"Mixed tumor" properly refers to pleomorphic adenoma, which often deomstrates myxoid and cartilage-like elements in addition ot stellate or fusiform epithelial cells. Oncocytoma is characterized by cells with granular eosinophilic cytoplasm. Adenolymphoma (papillary cystadenoma lymphomatosum) is often called Warthin tumor. Adenoid cystic carcinoma often presents with pain due to infiltration of perinerual spaces.
All of the following conditions are correctly matched wit hthe appropriate phrase EXCEPT: a. achalasia - acid gastric reflux; b. Mallory-Weiss syndrome - eophageal mucosal tears and hemorrhage c/b retching; c. esophageal varices - portal hypertension; d. Barrett esophagus - lesion predisposing to malignancy.
achalasia - acid gastric reflux
Achalasia is characterized by failure of relaaxation of the lower eophageal sphincter; acid gastric reflux is not an expected finding. The Mallory-Weiss syndrome (hemorrhage from esophagogastric mucosal tears) and bleeeding eosphageal varices (due to portal hypertension) are important causes of upper GI hemorrhage. Barret esophagus is a well-recognized precursor of adenocarcinoma.
Predisposing factors that may lead to gastroesophageal reflux include all of the following EXCEPT: a. hiatal hernia, B. pernicious anemia; C. pregnancy, d. scleroderma, e. incompeten esophageal sphincter
pernicious anemia
Gastroesoph reflux of HCl does not occur in PTs with pernicious anemia, which is assoc with achlorhydria, total absence of gastric HCl. Hiatal hernia, pregnacy, scleroderma, and incompetence of the esophageal sphincter can all lead to gastroesph reflux
An esophageal malignancy affecting the upper third of the esophagus is most likely to be:
squamous cell carcinoma
On a worldwide basis, squamous cell carcinoma of the esophagus Is much more common thatn adenocarcinoma. However, in the SU, adenocarcinoma and squamous cell carcinoma of the esophagus occur with equal freq because of a decrease in squamous cell carcinoma thought to be c/b decreased use of tobacco. In the upper 2/3s of the esophagus, squamous cell carcinoma is more common. Conversly, in the lower thrid, adenocarcinoam is more common. The other lesions listed above are rare.
All of the following disorders are correctly matched with the appropriate hcaracteristic or causative agaent EXCEPT: a. congenital pyloric stenosis - palpable mass, b. adenocarcinoma of the stomach - H pylori, c. acute (erosive) gastritis - alcohol, d. autoimmune gastritis - lesion predisposing duodenal ulcer, e. Menetrier disease - gastric rugalhypertrophy
autoimmune gastritis - lesion predisposing to duadenal ulcer
Autoimmune gastritis is assoc with reduced secretion of proteolytic enzymes and HCl, as well as with antiparietal cell abs. It predisposes to pernicous anemia, other autoimmune disorders, and carcinoma of the stomach. Duodenal ulcer is unleikely in the presence of hyposecretion of gastric HCl. the hypertrophied pyloric circular muscle layer of congenital pyloric stenosis often results in a palpable mass. Adenocarcinoma of the stomach is often associated with H. pylori infection, as are chronic gastritis and peptic ulcer of the somach and duodenum. Acute gastritis is c/b various toxic agents, including alcohol, aspirin, and other analgesic, and cigarett smoking. Menetrier disease is defiene by the cahracterisitc enlargement of the gastric rugae.
All of the following phrases concerning peptic ulcer are correctly mathced EXCEPT: a. peptic ulcer of jejunum - pancreatic neoplasm; b. Meckel diverticulum - ectopic gastric mucosa; c. gastric peptic ulcer - extreme hypersecretion of HCl; d. dudenal ulcer - most common peptic ulcer; e. blood group 0 - increased incidnec of duodenal ulcer
gastric peptic ulcer - extreme hypersecretion of HCl
IN contrast to peptic ulcer of the duodenum, gastric acid secretion is often not increased in gastric pepitc ulcer. It is reare, however, for gastric peptic ulceration ot occur in the total absence of HCl.
Risk factors assoc with carcinoma of the stomach include all of the following EXCEPT: a. use of nitrites as food preservatives, b. high intake of animal fat, c. blood group A, d. chronic gastritis, e. pernicious anemia
high intake of animal fat
High animal fat intake is assoc with a secreased incidnec of carcinoma of the stomach. The ue of nitrites as food presrevatives, blood group A, crhonic gastritis, and pernicous anemia are all well-known asoc of gastric cancer.
All of the following are characteristics of ulcerative colitis EXCEPT: a. bleeding being the most freq clinical presentation; b. crypt abscess formation, c. increased risk of adenocarcinoma; d. inflammatory changes limited to the mucosa and submucosa; e. involvement of any portion of the GI tract
involvement of any portion of the GI tract
Ulcerative colitis is limited to the large intestine, affecting principally the rectum and left colon and sometimes the entire colon
The lesion shown below may occur in all of the following syndromes EXCEPT: a. Gardner, b. Turcot, c. Familial polyposis, d. Peutz-Jeghers
The illustration shows a tubular adenoma, which is the most common form of adenomatous polyp. These lesions can be single or multiple, or they can occur as componenets of various multiple polyposis sydnromes. Notable among these syndromes are Garnder (assoc with osteomas and soft tissue tumors), Turcot (assoc with CNS tumors), and familiial polyposis. All of the foregoing are asoociated with an increased incidence of colon malignancy. In contrast, the Peutz-Jeghers polyp is a non-neoplastic hamartomatous lesion and is not assoc with colon cancer
Match oral infection with association: Activated by febrile illness. A. vincent infection, b. thrush, c. aphthous stomatitis, d. herpes labialis
Herpes labialis
Herpes labialis is a recurrent vesicular infxn of the oral mucosa and admacent skin, c/b herpes simplex verus type 1. The virus most often resides in the trigeminal ganglion, where it remains latent until activation by stresses such as fever, trauma, sunshine, and menstruation.
Match disease with association: Frequently complicated by malignancy. A. Ulcerative colitis, B. Crohn disease, C. Both
Ulcerative colitis
After a decade of disease, approximately 1/3 of PTs with ulcerative colitis develop colon cancer. Although there is some increased risk for malgnancy, carcinoma is an unusual compliation of Crohn disease.
Match disease with association: All layers of intestinal wall are involved. A. Ulcerative colitis, B. Crohn disease, C. Both
Crohn disease
In marked contrast to ulcerative colitis, which is primarily a mucosal disease, Crohn disease affects all layers of the intestinal wall.
Each of the following disorders is approximately matched with the corresponding hepatic change EXCEPT: a. neonatal hepatitis - giant cells; b. cytomaegalovirus infection - owl's eye inclusions; c. yellow fever - cholestatis; d. Echinococcus infection - large cysts; e. schistosomiasis - portal hypertension
yellow fever - cholestasis
Yellow fever characteristically results in midzonal hepatic necrosis, not cholestasis
The most freq hepatic malignancy is
metatatic tumor
Although hepatocellular carcinoma is the most common primary hepatic malgnancy, metastatic tumors account for most malgnant tumors of the liver
Complications or well-established assoc of gallstones include all of the following EXCEPT: a. biliary obstruction; b. cholesterolosi; c. pancreatitis; d. intestinal obstruction; e. malignancy
Cholesterolosis (strawberry gallbaldder), a clinically insignificant aberration characterized by yellow, cholsterol-containing flecks in the mucosal surface of the gallbladder, hs no special ass with gallstones. Well-known complications of gallstones include biliary obstruction, pancreatitis, and intestinal obstruction (gallstone ileus), often occurringlate in life. Crarcinoma of the gallbladder is more greq in the presence of gallstones.
Match hepatic tumor with associations: Polyvinyl chloride. A. adenoma; b. hepatocellular carcinoma; c. cholangiocarcinoma; d. hemangiosarcoma
Industrial exposure to polyvinyl chloride is assoc with increased incidence of hemangiosarcoma of the liver.
A renal biopsy was taken from a 50 yo man with hypertension and the nephrotic syndrome. The appearance of the biopsy was similar to the illustration shown below. Of the following possible additional lab findings, which one is most charcateristcally associated with the lesion?
The illustration shows nodular glomerulosclerosis (Kimmelstiel-Wilson nodules), the most characteristic glomerular finding in dibabetes mellitus. The nodules are accumulations of mesangial matrix-like material
A 3 yo girl presents with generalized edema shortly after recovery from an upper resp infection. Lab studies reveal marked albuminuria as well as hypoalbuminemia nad hyperlipidemia. Prior similar epsiodes responded to adrenal steroid medication. The most likely diagnosis is:
minimal change disease
The combo of genealized edema, massive proeinuria, hypoalbuminemia, and hyperlipidemai constitues the nephrotic snydrome, the prototype of which is minimal change disease (lipoid nephrosis). This disorder charcteristically occurss in young children and demonstrates intracytoplasmic lipid in the proximal convoluted tubules, a paucity of glomerular abnormalities by light micorscopy, nad "fusing" (absence) of the epithelial foot processes by electron microscopy.
A 28 yo woman presented with fever, dysuria, urinary frequency, and flank tenderness. The urine contained numerous neutrophils nad many white cell casts. Urine protein was moderatly increaed. A quantitative urine culture revealed more than 10^5 bacteria/ml. The most likely causative organism was:
E. coli
Organisms involved in most urianry tract infections are most often normal flora of the colon, and the most freq of these is E. coli.
Associations of renal papillary necrosis include all of the following EXCEPT: a. impaired glucose tolerance, b. long-term use of pheacetin and aspirin compounds, c. myoglobinuria, d. fever and pyuria
Renal papillary necrosis (ishcemic necrosis of the tips of the renal papillae) is most often a compication of diabetes mellitus, chroniic analgesic nephritis, or acute pyelonephritis. It has no ascoc with myoglobinuria.
A kidney demonstrating coarse asymmetric renal corticomedullary scarring, deformity of the renal pelvis and calycces, interstitial fibrosis, and atrophic tubules containing eosinophilic proteinaceous casts is most suggestive of
chronic pyelonephritis
The combo of coarse asymmetric corticomedullary scarring, deformity of the renal pelvis nad calyces, and tubular atrophy is characterisitc of chronic pyelonephyritis. When the atrophic tubules contain eosinophillic proetien casts, the resultant similarity in appearance to theyroid follicles is referred to as "thyoidization." Although an ifectious etiology is assumed, the infectiouis agent is often not demonstrable.
A 2 yo boy with visible abdominal distention is found to have an enormous left-sided flank mass apparently arising form ,but dwarfing, the left kidney. Associations of the lesion include all of the following EXCEPT: a. congenital aniridia, b. subtle or gross deletions of the short arm of chrom 11, c. berry aneurysm of the circle of Willis, d. mental-motor retardation, e. hemihypertrophy
berry aneurysm of the circle of Willis
Bery aneurysm of the circle of Willis is a freq assoc and complication of adult polycystic kideny. Assoc of Wilms tumor noitably include gene deletions localized to the short arm of chromosome 11. In some instances, a "2-hit" mechanism of cancer suppressor gene inactivation, simlar to that potulated for retinoblastoma, appears ot be operative. Other associations of Wilms tumor include congeniatl aniridia, genitourinary malformations, and mental-motor retardation. Another unusual assoc is "hemihypertrophy" as part of the Beckith-Wiedemann syndrome; yet another is the Denys=Drash syndrom, which is charcteriized by intersexual disorders, nephropathy, and Wilms tumor.
A 55 yo man presents with painless hematuria. On cystoscopy, a papillary mass is ofund in the bladder. All of the following are characteristic of the lesion EXCEPT: z. the incidnece is increaesd in cigarette smokers, b. the majority are adenocarcinomas, c. the incidnece is increased in aniline dye workers, d. it is more likely to be malignant than benign, e. similar leisions with similar histology can occur thorughout the urinary tract
the majority are adenocarcinomas
The majority of urinary tract tumors are transitional cell carcinomas. Adenocarcinoma of the bladder is rare.
A glomerular immunofluorescent pattern for IgG similar to that shown in this illustration would be expected in which of the following PTs?
a 25 yo man with hemoptysis and hematuria
The illustartion demonstrates linear immunofluorescence, whichi is characterisitic of disease caused by antigolmerular basement memberane abs. In Goodpasture syndrome, abs directed against ags in the basement membranes of the glomeruli as well as the pulmonary alveoli result in both hemorrhagic penumonitis with hemoptysis and glomerular disease with hematuria.
An enormously enlarged kidney similar to that shown in the illustration below was found at autopsy in a 65 yo man. Well known assocaiations or characteristics of this disease process include all of the following EXCEPT: a. cysts in the liver, b. berry aneurysm of the circle of Willis, c. hypertension, d. autosomal dominant inheritance, e. polycythemia vera
polycythemia vera
Secondary polycythemia, not polycythemia vera, is a complication of adult polycysitc kidney disease and is c/b increaed secretion of erythropoientin.
Which of the following charcteristics is LEAST frequently observed in renal cell carcinoma? A. ectopic hormone production, b. fever, c. flank pain, d. hematuria, e. tumor cells in urine
tumor cells in urine
Malignant cells are only rarely detected in the urine in renal cell carcinoma
For each condition, may type of renal stone associated: Leukemias nad myeloproliferative syndromes. A. calcium stones, B. ammonium magnesium phophate stones, C. uric acid stones, D. cystine stones.
uric acid stones
Hyperuricemia due to gout or increaesd urate excretion secondary to the increased cellular turnover of leukemias and myeloproliferative syndromes accounts for the presence of many uric acid stones.
All of the following testicular tumors are correctly matched with the apropriate definition or assoc EXCEPT: a. seminoma - most common germ cell tumor; b. endodermal sinus tumor - occurs in infancy and early childhood; c. teratoma - almost always malignant; d. choriocarcinoma - increased human chorionci gonadotropin; e. sertoli cell tumor - freq precocious sexual development
Sertoli cell tumor - freq precocious sexual development
The Sertoli cell tumor, a benign non-germ cell tumor of sex crod derviation, is most often assoc with few, if any, clincally observalbe endocrine effects. This is in cotnratst to the Leydig cell tumor, another benign sex cord tumork which is often assoc with profound endocrine manifestations
Match disease with associations: Visceral malignancies. A. Bowen disease of the penis, B. Erythroplasia of Queyrat, C. Carcinoma of the penis
Bowen disease of the penis
Bowen disease of the penis is assoc with an increaed risk for visceral malignancy, in contrast to erythroplasia of Queyrat, which is similar lesion that is not assoc with visceral malignancy.
A 24 yo woman is seen because of high fever, prostration, vomiting, and diarrhea. Her pulse is rapid and thready, and her BP is 60/40. A diffuse generalized macular rash is noted. Culture of which of the following specimens will most likely lead ot the corrct diagnosis?
Cervical secretions
The assoc of a severe febrile illness with signs of GI dysfxn and a diffuse macular rash is strongly suggestive of TSS. This diagnosis is especially liekly if clinical abnormalities are asooc with concomitant mentruation and the use of high absorbent tampons. The clinical abnormalities are due to Staph infection of the tampon, demontstrable in vaginal secretions, and the elaboration of Staph exotoxins. The differential diagnosis includes strep infection, usually pharyngeal, with a scarlatiniform eruption; meningococcemia; endotoxemia assoc with various gram Neg organisms; rickettsial infections, and so forth. Except for Strep infection, all of these conditions are less likely than TSS.
All of the following conditions or findings are correctly matched with the appropriate etiologic agents EXCEPT: a. cervicitis - Chlamydia trachomatis; B. condyloma lata - Treponema pallidum; c. condyloma acuminatum - human papillomavirus; d. multinucleated giant cells - herpes simplex virus, e. granuloma inguinale - Haemophilus ducreyi
granuloma inguinale - Haemophilus ducreyi
The etiologic agent of granuloma inguinale is Calymmatobacterium granulomatis, not Haemophilus ducreyi, which is the etiologic agent of chancroid, a STD common in the tropics but rare in the US
All of the following conditions are correctly matched with the appropriate assoc EXCEPT: a. endometriosis - sever menstrual pain, b. endometrial hyperplasia - excess estrogen stimulation, c. leiomyoma - postmenopausal decrease in size; d. endometrial carcinoma - multiparity; e. ectopic pregnancy - hematosalpinx
endometrial carcinoma - multiparity
Endometrial carcinoma is assoc with nulliparity, no multiparity. Other assoc include obesity, diabetes, and hypertension, all predisposing conditions commonly assoc with excess estrogen stimulation
A 20 yo woman presents with a solitary discrete, freely movable, firm, rubbery, non-tender, well-circumscribed breast lesion. On resection biopsy, the lesion appear similar to that shown in the illustrtion below. The most likely diagnosis is:
Fibroadenoma is a benign tumore most often presenting as a single discrete, freely movable lesion, often demonstrating a pattern of compressed glands and young fibrous stroma similar to that shown in the illustration. Fibroadenoma is the most freq cause of palpable mass in the breast in women younger than age 25.
Match the condition with the association: Fluid accumulations in serous cavities. A. Dermoid cyst, B. Struma ovarii, C. choriocarcinoma, D. Fibroma
The triad of hydrothorax, hydroperitoneum, and ovarian fibroma is known as Meigs syndrome. The cause of this assoc is unknown.
Match the condition with the association: Reduplication of meiotic chromosomes. A. Dermoid cyst, B. Struma ovarii, C. choriocarcinoma, D. Fibroma
Dermoid cyst
All benign ovarian teratomas are of 45,XX karyotype, and both isoenzyme and chormosome banding techniques suggest that these tumors are aberrant fetuses solely of maternal origin, arising afer the first meiotic division.
All of the following characteristics are asooc with somatotropic adenoma EXCEPT: a. gigantism, b. acromegaly, c. basophilic staining, d. hyperglycemia, e. hypertension
basophilic staining
Somatotropic adenoma most often exhibits eosinophilic staining. This growht hormone-secreting tumor resluts in gigantism if hypersecretion occurs before epiphyseal closure, or in acromegaly if hyperfunction begins in adolescence or adult life. In etiher case, hypersomatotropism is often complicated by hyperglycemia and by hypertension
Adrenal hypercorticism may be c/b all of the following EXCEPT: a. adrenal crotical adenoma, b. adrenal cortical hyperplasia, c. pituitary basophilic microadenoma, d. ptuitary basophilic hyperplasia, e. pituitary eosinophilic adenoma
pituitary eosinophilic adenoma
Hypercorticotropic ptiuitary tumors or hyperplasias are assoc with basophilic, not eosinophilic, staining.
Which of the following findings is NOT an assoc of primary hyperparathyroidism? A. "brown tumor" of bone, b. dystrophic calcification, c. hypertension, d. peptic duodenal ulcer, e. multiple endocrine neoplasia syndrome
dystrophic calcification
Dystrophic calcification occurs in precviously damaged tissues and has no special assoc with hypercalcemia or primary hyperparathyroidism, in contrast to metastatic calcification, which is the most signifaicatn complication of the chronic hypercalcemia of perimary hyperparathyroidism.
A tumor similar to that shown in the illustaration below has observed in a biopsy specimen from the thyroid of a 50 yo woman. An adjacent lymph node was also involved. Which one of the following descriptions of this tumor is most appropriate?
slow-growing lesion with relatively good prognosis
The lesion shown is a papillary carcinoma of the thyroid, which is the most common form of thyroid cancer. This tumor most often remains localized to the thyroid and adjacent tissues for many years, even whne local lymph nodes are involved. Papilaary carcinoma is almost always nonfunctional.
Type 1 diabetes mellitus is asoc with all of the following characteristics EXCEPT: a. focal islet cell fibrosis with amylin deposits, b. linkage ot HLA-D alleles, c. precipitation by viral infection, d. onset in childhood, e. propensity to ketoacidosis
focal islet cell fibrosis with amylin deposits
Islet cell focal fibrosis with deposition of amylin is characteristic of type 2 diabetes mellitus. In type 1, the pancreatic islets are diffuesly atrophic, bea cells are greatly decreaed, and insulitis marked by diffuse lymphocytic infiltration may be prominent early in the disease.
Familial premalignant condition is associated with what nevus?
Dysplastic nevus
Intradermal nodular foci of dendritic melanocytes associated with what type of nevus?
Blue nevus
The blue nevus, a lesion present at birth, appears blue because of the presence of foci of highly pigmented dendritic melanocytes deep in the dermis. BRS TEXT: BLUE NEVUS IS PRESENT AT BIRTH. BLUE NEVUS IS CHARACTERIZED BY NODULAR FOCI OF DENDRITIC, HIGHLY PIGMENTED MELANOCYTES IN THE DERMIS; BLUE EXTERNAL APPEARANCE RESULTS FROM THE DERMAL LOCATION.
Childhood lesion with spindle-shaped cells associated with what type of nevus?
Spitz nevus
Nonfamilial malignant precursor lesion, associated with what type of nevus?
Lentigo maligna
Lentigo maligna, or Hutchinson freckle, is characterized by atypical melanocytes at the epidermal-dermal junction and is considered to be a precursor lesion to lentigo maligna melanoma, a form of malignant melanoma. BRS TEXT: LENTIGO MALIGNA (HUTCHINSON FRECKLE): LENTIGO MALIGNA IS A NONFAMILIAL PRECURSOR TO LENTIGO MALIGNA MELANOMA. LENTIGO MALIGNA IS AN IRREGULAR MACULAR PIGMENTED LESION ON SUN-EXPOSED SKIN. LENTIGO MALIGNA (HUTCHINSON FRECKLE) IS CHARACTERIZED BY ATYPICAL MELANOCYTES AT THE EPIDERMAL-DERMAL JUNCTION.
Describe the nevocellular nevus (common mole):
*Nevocellular nevus is variably classified as a benign tumor or hamartoma; nevus cells are derived from melanocytes and ordinarily occur in clusters or nests.* The three most common types of nevocellular nevi are: a. Junctional nevus--nevus cells confined to the epidermal-dermal junction. b. Compound nevus--nevus cells both at the epidermal-dermal junction and in the dermis. c. intradermal nevus--nevus cells confined to clusters within the dermis (these cells are often nonpigmented).
Angular atrophy predominantly involving type II muscle fibers
Angular atrophy predominantly involving type I muscle fibers.
Disuse atrophy is histologically characterized by angular atrophic changes predominantly involving type II muscle fibers. Similar changes involving both type I and type II fibers, fiber-type grouping, and target fibers are all associated with denervation atrophy. BRS TEXT: DISUSE ATROPHY: IS ASSOCIATED WITH PROLONGED IMMOBILIZATION. DISUSE ATROPHY IS CHARACTERIZED HISTOLOGICALLY BY ANGULAR ATROPHY, PRIMARILY OF TYPE II FIBERS.
autosomal recessive inheritance (false)
partial or segmental gene deletion mutations (true)
All of the following statements concerning cerebrovascular disease aer true EXCEPT: a. most common cuase is emoblization from cardiac mural thrombi, b. infarction is more freq than hemorrhage, c. hemorrhage is freq assoc with hypertension, d. subarachnoid hemorrhage is freq assoc with bery aneurysm of the circle of Willis, e. atherosclerosis is the most freq underlying babsis of arterial thrombossi and occlusion
most common cuase is emoblization form cardiac mural thrombi
Embolic obstruction of cerebral vessels with resultant infarction is less freq than local thrombotic occlusion assoc with atherosclerotic disease. However, in PTs such as those with chronic atrial fibrillation, the devleopment of atrial mural thrombi and subsequent cerebral embolization is a threat. Cerebral hemorrhage is often assoc with hypertension; when hemorrhage is subarachnoid in distribution, rupture of a berry aneurysm of the circle of Willis is a freq finding
A 75 yo woman appeared well after slipping on wet pavement and striking the right side of her head. When questioned, she said tha tshe did not remember the fall. Subsequently she complained of persistent headache and confusion. MRI studies revealed a subdural hematoma ove rthe lateral aspectof the reight cerebral hemisphere. Which of the following is a well-known assoc or characteristic of this disorder?
characteristically c/b venous hemorrhage
Subdural hematoma is charcteristically c/b venous bleeding, most often from veins that join the cerebrum to venous sinuses withing the dura. The venous hemorrhage typically arrests early, but the volume of the hematoma gradually incrases because of osmotic imbibement of water. This results in a slowly enlarging tumor-like mass characterized clinically by gradual signs of cerebral comkpression occurring hours to days or even weeks after head injury.
A 2 yo child presents with fever, headache, prostration, and nuchal rigidity. The CSF is cloudy, and microscopic examination reveals innumerable neutrophils. The CSF protein is increased, and glucose is decreased. The most likely etiologic agent is:
Strep pneumoniae
Pyogenic meningitis in older infants and youn kids is most freq c/b Strep pneumoniae or Neisseria meningitidis. In newborns, the most likely agents aer group B streptococci, E. coli, and Listeria; in youg adults, the most freq agaent is N. Meningitidis. In older adults, especially those with impaired reisstance to infection, the most common etiologic agents are S. pneumoniae and Gram neg rods.
All of the following charcterisitcs are asoociated with infantile toxoplasmosis EXCEPT: a. periventricular calcifications demonstrable by readiograph, b. common occurrence of hydrocephalus, mental reatrdation, and other neuroligic abnormalities; c. favored sites of involvement include the cerebral cortex, basal ganglia, retinae, heart, lungs, and liver;, d. charatceristically spread by a child ingesting foods contaminated by the urine of household pets; e. cats are frequent reservoir
characteristically spread by a child ingetsting food s contaminated by the urine of household pets
In the infantile form of toxoplasmosis, Toxoplasma gondii is characteristically transmitted transplacentally from an infected mother and not by ingestion of food contaminated by animal urine or feces, as occurs in older children
All of the following viral diseases or viruses are correctly matched with the approprieate association or characteristic EXCEPT: a. St. Louis encephalitis - reservoir is horses and birds; b. herpes simplex encephalitis - common compication of herpes simplex virus infection; c. poliomyelitis - degeneration and necrosis of anterior horn cells of spinal cord; d. rabies - Negri bodies; e. cytomegalovirus infection - immunosuppressed persons
herpes simplex encephalitis - common compication of herpes simplex virus infection
Herpes simplex encephalitis is an uncommon complication of HSV infection but is, nonetheless, the most common form ofsevere viral encephalitis.
All of the following diseases of viral or suspected vrial etiology are correctly mathced with the appropriate association or charcaterisitc EXCEPT: a.AIDS - CD4+ T lymphocytes probable vehicle for viral entry into the CNS; b. kuru - ingestion of human brain; c. Creutzfeldt-Jakob disease - possible danger to health workers; d. subacute sclerosing panencephalitis - measles virus; e. progressive multifocal leukoencephalopathy - leukemia or lymphoma
AIDS - CD4+ T lymphocytes probable vehicle for viral entry into the CNS
Cells of monocyte-macrophage origin are considered to be the probable vehicle by which HIV infection enters the CNS, and these cells most likely function as a viral reservoir.
All of the following are characteristics of amyotrophic lateral sclerosis EXCEPT: a. association with a papovavirus, b. invollvement of anterior motor neurons, c. involvement of lateral corticospinal tracts, d. symmetric hyperreflexia and spasticity, e. symmetric muscle atrophy and fasciculation
association with a papovavirus
The JC polyoma papovavirus is associated with progressive multifocal leukoencephalopathy. The cause of amyotrophic lateral sclerosis (Lou Gehrig disease) remains unknown.
All of the following disorders are correctly matched with the appropriate association or characteristic EXCEPT: a. guillain-Barre syndrome - albumiocytologic dissociation, b. Alzheimer disease - focal spherical collections of swollen nerve cell processes with a centeral amyloid core, C. Huntington disease - hereditary disorder with delay of clinical onset utnil age 30-40, d. Pick disease - Lewy Bodies, e. Parkinson disease - depigmentation of cells of substantia nigra
Pick disease - Lewy Bodies
Lewy bodies are characteristic eosinophilic intracytoplasmic inclusions observed in idiopathic Parkinson disease, not Pick disease, and are noted within the depigmented cells of the substantia nigra, whciih are alos characteristic of this disorder
The ability of a lab test to correctly ID individuals with a specific disease is reffered to as
Sensitivity is defined as the abililty of a test of be Pos in PTs with a specific disease. It is calcualted by dividing the number of Pos resluts in PTs by the total numbre of PTs.
The ability of a lab test to correctly ID individuals who are free of a specific disorder is reffered to as
Specificity is defninde as the abillity of a test ot be negative in healthy persons and is calculated by dividing the number of neg results in healthy persons by thte total number of healthy persons
A new test for disease A is found to have a sensitivity of 99% and a specificity of 99%. The test is further evaluated in 3 highly selecte population subgroups with differing prevalances of the disease. Match the likelihood that the new test correctly IDs a PT with disease A. with the appropriate subgroup. A subgroup in which the prevalence is 1 in 1000. A. 1%, B. 9%, C.50%, D. 92%, E. 99%
A population afrbitrarily set at 100,000 will include 100 PTs with the disease A and 99,900 individuals free of disease A. Of the 100 PTs, 99 will test pos. Of the 99,900 unaffected persons, 999 will test pos. The total number of pos results is thus 99 + 999. The predictive value of a pos result is then 99/(99+999), or 9%.
A new test for disease A is found to have a sensitivity of 99% and a specificity of 99%. The test is further evaluated in 3 highly selecte population subgroups with differing prevalances of the disease. Match the likelihood that the new test correctly IDs a PT with disease A. with the appropriate subgroup. A subgroup in which the prevalence is 1 in 10. A. 1%, B. 9%, C.50%, D. 92%, E. 99%
In this very high prevalence subgroup, the predictive value of a pos reslut is 92%. These 3 examples illustrate the profound effcect of prevalence on the usefulness of a diagnostic test. A test procedure is more likely to be useful when directed to PTs who have a high likelihood of the disorder, as dedictated by good clincal judment, than when used in an indiscrminate, unselective fashion.