previous post ,some mrcp ques , discuss &downloadABXICIA

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    1. A 27-year-old man with a history of IV drug use was found to have abnormal liver function tests
    Further work-up including serologic tests for viral hepatitis show
    Hepatitis B surface antibody (HBsAb) negative
    Hepatitis B surface antigen (HBsAg) positive
    Hepatitis core antibody (HBcAb) positive
    Hepatitis B surface antibody (HBsAb) negative
    Hepatitis B e antibody (HBeAb) positive
    Hepatitis B e antigen (HBeAg) negative
    Which of the following statements is true regarding this patient?

    He is a chronic hepatitis B virus (HBV) carrier with high infectivity.
    He is in the incubation period of HBV.
    He is a chronic HBV carrier with low infectivity.
    He has recovered from HBV infection and is immune to HBV.

    Answer: C
    Explanation: In the interpretation of results of hepatitis B serologic tests, the following facts should be considered: during the incubation period (i.e., before the onset of clinical manifestations) HbsAg, HbeAg, and HBV DNA become detectable in the serum. At the onset of clinical symptoms (e.g., jaundice), an increase in the serum transaminases antibodies occurs and antibodies to HBc become detectable (HBc antibodies). Initially, the HBc antibodies are IgM and thereafter IgG; these latter antibodies persist for years. HBs antibodies become detectable late in convalescence. A rise in HBs antibodies in combination with a loss of HbsAg, HbeAg, and HBV DNA indicate the presence of immunity to HBV. HbeAg and HBV DNA are markers of active viral replication and thus indicate high infectivity. The loss of HbeAg and appearance of anti-HbeAb indicates a less infective stage.

    2. A 23-year-old woman experienced watery diarrhea, nausea, vomiting, and abdominal cramps 6 hours after eating a salad and a hamburger in a local restaurant. The most likely organism causing her disease is

    Vibrio vulnificus
    Listeria monocytogenes
    Yersinia enterocolitica
    Clostridium welchii
    Staphylococcus aureus

    Answer: E
    Explanation: Staphylococcal food poisoning is manifested 2 to 6 hours after eating food (salad, potato salads) contaminated by a preformed enterotoxin. Yersinia is most commonly associated with the ingestion of improperly cooked meat, but symptoms generally begin more than 1 day after ingestion of the contaminated food. Symptoms resulting from L. monocytogenes also occur more than 24 hours after the ingestion of contaminated foods (milk, ice cream, and poultry). V. vulnificus-associated food poisoning presents usually 24 to 48 hours after the ingestion of contaminated seafood (usually oysters). C. welchii is not associated with food poisoning. The two clostridia associated with food poisoning are C. perfringens and C. botulinum.

    3. A 35-year-old man presents with diarrhea for 10 days, characterized by frequent, low-volume stools with the presence of mucus. He also complained of subjective fever and lower abdominal pain. The presence of leukocytes in stool is consistent with which organism?

    Clostridium perfringens
    S. aureus
    Giardia lamblia
    Enterobius vermicularis
    Entamoeba histolytica

    Answer: E
    Explanation: The presence of large numbers of leukocytes in stool is diagnostic of colonic mucosal inflammation and should suggest infection with enteroinvasive organisms such as Shigella, E. histolytica, Salmonella, Campylobacter, invasive Escherichia coli, or Y. enterocolitica. Those organisms that cause diarrhea by a noninvasive mechanism (Giardia lamblia, enterotoxigenic E. coli, Vibrio cholerae) are not associated with leukocytes in the stool


    4. Acetaminophen is an important cause of acute hepatic failure. All of the following statements about acetaminophen toxicity are correct, except

    Significant liver injury usually occurs with doses of more than 10 - 15 g.
    Alcoholics are more susceptible to liver injury even with a low dose.
    N-acetylcysteine is most effective when administered within 10 hours of ingestion.
    Hemodialysis is effective in the management of hepatotoxicity.
    Survivors of acetaminophen-induced hepatotoxicity do not experience any progressive or residual liver damage.

    Answer: D
    Explanation: Acetaminophen overdose causes acute liver failure. Significant liver injury usually occurs with doses of >10 to 15 g, most frequently taken in a suicide attempt. The liver injury is caused by toxic metabolites of acetaminophen formed by the microsomal cytochrome P-450-dependent drug-metabolizing system. Because ethanol induces this cytochrome P-450 system, severe hepatotoxicity can be seen in alcoholics, even with lower dosages of acetaminophen. N-acetylcysteine administered early after ingestion (i.e., <24 hours) reduces the severity of liver necrosis. Acetaminophen and its metabolites are not cleared by hemodialysis. Survivors of acute acetaminophen toxicity usually recover completely without progressive or residual liver damage.

    5. Which statement about esophageal cancer is true?

    Dysphagia is an early manifestation.
    The most common type of esophageal cancer in the United States is adenocarcinoma.
    Esophageal cancer is most commonly located in the proximal third of the esophagus.
    Most esophageal cancers are not resectable at presentation.
    Barrett's syndrome is associated with squamous carcinoma.

    Answer: D
    Explanation: Most esophageal cancers are asymptomatic, and at the time of diagnosis most are unresectable. Barrett's syndrome is associated with adenocarcinoma of the esophagus. Despite the increasing incidence of adenocarcinoma, the most common type of esophageal carcinoma in the United States is squamous cell carcinoma, which generally is located in the distal third of the esophagus.

    6. A 42-year-old man presents with intermittent dysphagia to solids and liquids and regurgitation of food. He has lost 4 pounds in 2 months. His physical exam is normal. A barium swallow reveals a dilated esophageal body, with the distal esophagus terminating in a narrow end. Which one of the following options is the most appropriate long-term therapy?

    Isosorbide dinitrate
    Metoclopramide
    Dilation with balloon
    Nifedipine
    Dilation with rubber tube (bougie)

    Asnwer: C
    Explanation: Achalasia is best treated with mechanical disruption of the lower esophageal sphincter. Dilation with a large Hurst bougie may give temporary relief; a few patients have been maintained with weekly self-dilations, but this treatment is no longer recommended. Much more effective is dilation with a pneumatic balloon (bag) under radiographic control. A successful approach to long-term pharmacologic management of achalasia has not been established. Short-term improvement in clinical symptoms and in scintigraphic esophageal emptying may occur with isosorbide mononitrate, a long-acting nitrate, or with nifedipine, a calcium-channel blocker. Promotility agents like metoclopramide increase the lower esophageal sphincter pressure and thus are contraindicated in achalasia.


    7. A 45-year-old male executive comes to your office complaining of epigastric pain for 2 months. His primary physician prescribed him H2-blockers 3 weeks ago, which have produced only partial relief of his symptoms. His weight is stable. His physical exam is normal. An upper endoscopy reveals a 1-cm duodenal ulcer. Which of the following risk factors is not associated with the development of ulcer disease?

    Daily use of nonsteroidal anti-inflammatory drugs (NSAIDs)
    Gastric infection with H. pylori
    Emotional stress
    Cigarette smoking
    Gastrin-secreting tumors

    Answer: C
    Explanation: Although considered a risk factor in the past, several studies showed that emotional stress is not a risk factor for the development of duodenal ulcer. Daily NSAID use significantly increases the risk of ulcer disease (risk ratio, 10- to 20-fold). Gastric infection with H. pylori increases risk about five- to sevenfold. Cigarette smoking doubles the risk of duodenal ulcer. At least 90% of those patients with Zollinger-Ellison syndrome have duodenal ulcer.

    8. A 20-year-old white woman presents with jaundice and malaise of 2 weeks' duration. Her boyfriend had some form of hepatitis several months before. Initial laboratory studies reveal alanine transaminase (ALT) of 211 U/L, aspartate transaminase (AST) of 194 U/L, and bilirubin of 5.4 mg/dL. HBsAg and anti-HBc IgM are positive. Which of the following statements regarding acute hepatitis B is false?

    About 90% of patients with acute hepatitis B will recover completely.
    About 1% of patients with acute hepatitis B can experience fulminant hepatic failure.
    Chronic hepatitis B carrier state will develop in 10% of patients.
    Interferon administration in the acute phase of infection prevents the development of the chronic hepatitis B carrier state.


    Answer: D
    Explanation: Ninety to 95% of otherwise healthy adult patients with acute hepatitis B recover completely and become HBsAg negative. About 1% experience massive necrosis, and 5 to 10% of patients who remain HBsAg positive beyond 6 months are at increased risk of chronic hepatitis. Interferon given during acute hepatitis B infection has not shown any benefit.

    9. A 51-year-old woman presents with abdominal pain, weight loss, early satiety, and night sweats. On physical exam she appears cachectic, multiple enlarged lymph nodes are present in her neck (supraclavicular area), and a mass is palpated in the epigastrium. Laboratory data reveal a hemoglobin of 8 g/dL and a normal WBC count. Which of the following is the most appropriate next step in establishing the diagnosis?

    Upper GI series
    Peripheral blood smear
    CT of the abdomen
    Upper endoscopy with biopsy
    Exploratory laparotomy

    Answer: D
    Explanation: This patient has lymphoma of the stomach. Lymphoma of the stomach can resemble superficially spreading carcinoma, linitis plastica, or solitary adenocarcinoma. Gastroscopy with directed biopsy and brush cytology gives a higher yield than was previously appreciated, especially in the presence of exophytic lesions. Lymphoma of the stomach frequently presents radiographically as a bulky mass and less frequently as a diffusely infiltrating tumor-the most common form of secondary lymphoma-giving the appearance of large folds on upper GI series, frequently associated with multiple nodular defects and ulcerations. Although CT may be useful to evaluate the extent of disease, it will not provide a specific diagnosis. Exploratory laparotomy is useful for staging and therapeutic resection where possible.



    10. Which of the following features best distinguishes Crohn's disease from ulcerative colitis?

    Oral ulcers
    Rectal bleeding
    Continuous colonic involvement on endoscopy
    Noncaseating granulomas
    Crypt abscesses

    Answer: D
    Explanation: Oral ulcerations can occur both in Crohn's disease and ulcerative colitis. Rectal bleeding and continuous involvement of the colon may be also seen in both Crohn's disease and ulcerative colitis. The presence of crypt abscesses does not distinguish ulcerative colitis from Crohn's disease; however, noncaseating granulomas, when present, are pathognomonic of Crohn's disease.

    11. A 49-year-old man presents to the emergency room because of melena of 3 days' duration. He denies abdominal pain. Vital signs reveal a resting pulse of 104 per minute and a 25-mm Hg orthostatic drop in BP. Physical findings include bilateral temporal wasting, pale conjunctivae, spider angiomas on his upper torso, muscle wasting, hepatosplenomegaly, and hyperactive bowel sounds without abdominal tenderness to palpation. His stool is melenic. Nasogastric tube aspiration reveals coffee grounds material. Hematocrit is 31%. The appropriate next step in the management of this man's illness would be to

    Pass a Sengstaken-Blakemore tube.
    Obtain an upper GI series.
    Insert a transjugular intrahepatic portosystemic shunt (TIPS).
    Obtain immediate visceral angiography.
    Perform upper endoscopy.

    Answer: E
    Explanation: After this patient has been hemodynamically stabilized, the next most important step is to perform a diagnostic/therapeutic upper endoscopy. If the source of his bleeding is from esophageal varices, then these can be obliterated with sclerosis or, preferably, endoscopic band ligation. The use of a Sengstaken-Blakemore tube should be reserved for patients in whom upper endoscopy was unsuccessful in controlling the hemorrhage. A TIPS should be considered in patients in whom medical and endoscopic therapy have failed. Barium studies have no role in the evaluation of patients with suspected variceal hemorrhage



    Haematology

    1. You are asked to see a 25-year-old white man who experienced marked weakness and dyspnea 4 days after being admitted for a compound arm fracture after falling from a tree. Estimated blood loss from the initial fracture episode was 600 mL, and the patient was transfused with one unit of packed erythrocytes. The initial crossmatch was reported as compatible by the transfusion service. The patient has never been transfused before this incident and has no other serious medical illnesses. The patient's arm fracture was treated with surgical pinning and prophylactic antibiotics consisting of cefotetan 2 g IV every 12 hours. On examination, the patient is febrile and mildly tachycardic, with no evidence of wound infection or compartment syndrome. Laboratory data show a hematocrit of 15%, absolute reticulocyte count of 600,000 μL, and total bilirubin of 70 umol/L with direct bilirubin of 9 umol/L. The peripheral smear shows many spherocytes. No hemoglobinemia or hemoglobinuria is seen on visual inspection of the plasma and urine. The transfusion service reports that the direct Coombs' test is now strongly positive using anti-IgG and only weakly positive with anti-C3d antisera. They further report that routine compatibility tests show no new erythrocyte antibodies in the patient's serum and that, when they attempted to elute antibody from the patient's RBCs and test against normal RBCs, the results were negative. What is the most likely diagnosis?

    Hemolytic transfusion reaction caused by an ABO incompatibility
    Delayed hemolytic transfusion reaction
    Autoimmune hemolytic anemia of warm antibody type
    Autoimmune hemolytic anemia of cold antibody type
    Drug-induced immune hemolytic anemia

    Asnwer: E

    Explanation: Recognize drug-induced immune hemolytic anemia of the hapten type, classically developing in patients exposed to high doses of penicillin. The other types of drug-induced immune hemolytic anemia are the [agr ]-methyldopa type (the most common) and the quinidine type (occurring with quinidine, quinine, stibophen, chlorpromazine, and sulfonamides). In this patient the strongly positive direct Coombs test shows that this is an immune hemolytic anemia. Three findings suggest the diagnosis of a drug-induced mechanism rather than an autoimmune mechanism: (1) the patient received a cephalosporin known to induce a hapten-type reaction, (2) routine tests for RBC antibodies in the patient's serum were negative even though the patient's RBCs were strongly coated for antibody, and (3) eluate from the patient's RBCs was not reactive with normal RBCs. In most cases of drug-induced immune hemolytic anemia, the RBC antibodies are detectable only if the offending drug is added to the in vitro system.


    2. Pregnancy-related microangiopathic hemolytic anemia is caused by all of the following, except

    Preeclampsia/eclampsia
    Pregnancy-related ITP
    HELLP syndrome (hemolysis, elevated liver enzymes, low platelet count)
    Postpartum hemolytic-uremic syndrome (HUS)

    Answer: B
    Explanation: Pregnancy-related ITP, by definition, affects only the platelets. Microangiopathic hemolytic anemia, which is caused by a variety of disorders, is an RBC fragmentation syndrome resulting from fibrin deposition in partially thrombosed microvasculature. RBCs are caught on the thin fibrin strands, and fragmentation of RBCs into various sizes and shapes results. Preeclampsia/eclampsia, HELLP, and postpartum HUS can give rise to microangiopathic hemolytic anemia. (Hoffman et al, Chs. 32-35; Lee et al, Ch. 49; Cecil, Ch. 169)

    3. All of the following cause microangiopathic hemolytic anemia, except

    TTP
    HUS
    Vasculitis
    Venoms
    Disseminated intravascular coagulation (DIC)

    Asnwer: D
    Explanation: Venoms cause intravascular hemolysis, but not by a mechanism of microangiopathic fibrin deposition. Other causes of intravascular hemolysis, but not by a mechanism of microangiopathic hemolytic anemia, include valve hemolysis, exertional hemolysis, chemical agents, osmotic lysis, thermal injury, infections, PNH, and cold agglutinin disease.

    4. Which of the following statements is true?

    Thalassemias are quantitative disorders of hemoglobin, whereas hemoglobinopathies are qualitative disorders of hemoglobin.
    Thalassemias are qualitative disorders of hemoglobin, whereas hemoglobinopathies are quantitative disorders of hemoglobin.
    Thalassemias are always inherited disorders

    Asnwer: A
    Explanation: In the general classification schema, thalassemias can be classified as disorders of quantitative abnormalities of hemoglobin, wherein the morbidity of the disease is usually a result of the excess globin chains of the unaffected gene (e.g., [agr ]-thalassemia results in decreased [agr ] chains and excess β chains, with the precipitated excess β chains causing the problems). Hemoglobinopathies (e.g., hemoglobin SS, SC) are qualitative abnormalities of the hemoglobin chains usually as a result of point gene mutations. Thalassemias are generally inherited, but acquired cases have been reported.

    5. A 52-year-old black woman comes to you for another opinion regarding a history of anemia that has been unresponsive to oral iron supplementation. She sought your opinion because her other physician was recommending IV iron supplementation. She has been on nearly continuous iron supplementation therapy ever since her second child was born 23 years ago. Over the years she says her doctors have prescribed her to take anywhere from one to three pills daily, sometimes with vitamin C concomitantly. Although she has never needed a transfusion, she says she has been told that her RBC count has never completely normalized. She is otherwise healthy and has no unusual dietary habits. Her menstrual history reveals relatively normal menstrual periods until about 3 years ago, when she attained menopause. The patient believes that her mother was also iron deficient. Your physical exam is normal. Laboratory values show a hemoglobin of 11.6 g/dL; hematocrit, 33%; MCV, 70 fL; normal WBC with differential; normal platelet count; serum iron, 70 μg/L; iron-binding capacity, 255 μg/dL; and ferritin, 158 μg/L. At this point you should next

    Agree with the other physician and recommend IV iron supplementation because she does not appear to be absorbing enough oral iron to totally correct her anemia.
    Perform a hemoglobin electrophoresis.
    Obtain a serum EPO level.
    Discontinue iron supplementation.
    Perform a bone marrow aspirate and biopsy.

    Answer: D
    Explanation: Recognize a clinical history suspicious for two-gene [agr ]-thalassemia. Deletion of two [agr ] genes (-[agr ]/-[agr ] or -/[agr ][agr ]) results in mild to moderate microcytosis and mild anemia, rarely with any progression or development of other signs or symptoms. It is probably the most common hemoglobinopathy in the world, and the combination of one-gene or two-gene [agr ]-thalassemia has an incidence of 20% or more among blacks. It is often mistaken for iron deficiency anemia, and menstruating women with two-gene [agr ]-thalassemia are often treated for prolonged periods with iron supplementation because it is presumed that the mild microcytic anemia is due to iron deficiency. A hemoglobin electrophoresis is a useful test for β-thalassemia wherein one looks for increased levels of hemoglobin A2 and hemoglobin F. However, hemoglobin electrophoresis is generally not helpful for the diagnosis of an [agr ]-thalassemia disorder. A globin chain synthesis study is generally required for a conclusive diagnosis. Because these studies are not routinely available, [agr ]-thalassemias are often diagnosed presumptively by ruling out other possibilities


    6. A 25-year-old white woman presents to the emergency room with the complaint of extreme shortness of breath of acute onset. She was actually seen in the same emergency room 24 hours previously where she was diagnosed with a urinary tract infection and given prescriptions for phenazopyridine (Pyridium) and sulfamethoxazole. She is overweight and sedentary and smokes two packs of cigarettes a day. On physical exam she is markedly dyspneic and extremely cyanotic. Arterial blood gases fail to reveal any hypoxia, but a ventilation-perfusion scan is obtained anyway, which is read as low probability. What should be the next course of action?

    Repeat the arterial blood gas to look for progression and development of hypoxia.
    Proceed to pulmonary arteriography.
    Begin anticoagulation.
    Administer methylene blue.
    Transfuse two units of packed RBCs.

    Answer: D
    Explanation: Recognize an individual with methemoglobinemia who has been exposed to an offending agent. Rapid development of extreme dyspnea and cyanosis, in the setting of no hypoxia, should be the clue to consider methemoglobinemia. In this case, the patient was exposed to two different known medications (pyridium and sulfamethoxazole) associated with increased levels of methemoglobin in susceptible individuals. Methemoglobin is the derivative of hemoglobin, in which the iron of the heme group is oxidized from the ferrous to the ferric state. It is the oxidation status that determines the oxygen-carrying capacity of hemoglobin. When iron is in the ferrous form (deoxyhemoglobin), oxygen can easily bind, in contrast to the inability to bind to the ferric hemes of methemoglobin. Steady-state methemoglobin levels in the blood are usually <1% but can increase markedly when susceptible individuals (heterozygotes for methemoglobin reductase deficiency) are exposed to certain medications or chemicals. Correct therapy is prompt institution of methylene blue, to which individuals will respond rapidly with resolution of cyanosis.

    7. A 50-year-old white man comes to see you because he was told he had "high blood." Physical exam is normal except for a ruddy complexion, which he says he has had most of his adult life .He has smoked two packs of cigarettes per day since he was 16 years old. A CBC shows a normal WBC count and differential, normal platelet count, a hemoglobin of 18.4 g/dL, and a hematocrit of 57%. To work up this elevated hematocrit, what is the next most appropriate test to order?

    Serum EPO level
    Arterial blood gas analysis
    RBC mass study
    Bone marrow aspirate and biopsy
    Pulmonary function tests

    Asnwer: C
    Explanation: An RBC mass study is the next most appropriate test to order to determine whether the elevated hematocrit is a true polycythemia (erythrocytosis) or a spurious elevation (resulting from reduced plasma volume). Because of the significant smoking history, this patient may have evidence of chronic obstructive pulmonary disease with resultant abnormal arterial blood gases and pulmonary function tests, but these tests will not distinguish a true polycythemia from a spurious one. An EPO level may be indicated later in the work-up once a true polycythemia has been documented.

    8. For the patient described in question 7, the next set of tests to order after the preliminary assessment would include all of the following, except

    Serum EPO level
    Determination of venous P50 (partial pressure of oxygen at which the hemoglobin is 50% saturated)
    Arterial oxygen saturation determination
    Carbon monoxide determination
    Bone marrow aspirate and biopsy

    Answer: E
    Explanation: Once a true RBC mass elevation has been documented, a search for a cause must ensue. The patient's history of nearly lifelong ruddy complexion could be due to tobacco abuse but may also suggest a congenital polycythemia. Most congenital polycythemias are due to hemoglobin mutants with high oxygen affinity. These abnormal hemoglobin affinities as well as abnormal levels of 2,3-diphosphoglycerate (2,3-DPG) can be detected by measuring a P50 level on the oxygen saturation/desaturation curve. Tumors and other disorders can lead to elevated levels of endogenous erythropoietin. Arterial oxygen saturation and carbon monoxide determinations can rule out pulmonary and environmental conditions. A bone marrow exam is rarely useful in the work-up of erythrocytosis, even for a potential diagnosis of polycythemia rubra vera, in which culture of erythroid progenitor cells for the detection of erythropoietin-independent colony growth is currently the closest thing to a diagnostic test for this disease.

    9. A 53-year-old woman comes to see you regarding a possible diagnosis of essential thrombocytosis. She says her gynecologist has noted a platelet count of >550,000/μL on three separate occasions over the past 2 years. Apart from two uneventful childbirths, the woman says she really has no significant medical history. She says she has never been told she was anemic. Lab values reveal a normal hemoglobin, hematocrit, and MCV. The platelet count is 580,000/μL. Your review of the peripheral smear reveals no microcytosis or hypochromia but does show RBC Howell-Jolly bodies. The platelet count on the smear appears elevated, but there are no giant platelets or platelet clumps. What is the next most appropriate step in your diagnostic work-up?

    Perform bone marrow aspirate and biopsy.
    Obtain a C-reactive protein and a sedimentation rate, looking for a state of chronic inflammation.
    Obtain a ferritin level to confirm that there is no iron deficiency.
    Go back and obtain a more thorough history and repeat the physical exam.
    Perform chest, abdominal, and pelvic CT scans, searching for an occult malignancy.

    Answer: D
    Explanation: The RBC Howell-Jolly bodies should be the tip-off that the patient has had a prior splenectomy. Further questioning would reveal that the patient failed to mention a splenectomy at the age of 14 after splenic injury in a motor vehicle accident. You missed the surgical scar on physical exam. Postsplenectomy patients can sometimes have lifelong mild elevations of either their WBC count or their platelet count. The gynecologist was correct to obtain several platelet counts over time to make sure the platelet elevation was persistently increased. Reasons for reactive thrombocytosis include iron deficiency, splenectomy, postsurgical state, infection or inflammation, and occult malignancy. There is currently no diagnostic test for essential thrombocytosis. It remains a diagnosis of exclusion and can be entertained only after all forms of reactive thrombocytosis have been ruled out.

    10. A 62-year-old woman with a platelet count of 1,350,000/μL has been diagnosed with essential thrombocytosis after an exhaustive search failed to reveal any reactive causes for the elevated platelet count. Her platelet count has been greater than 1 million for more than 6 months. The most appropriate therapy now that a diagnosis of essential thrombocytosis has been established is

    Platelet pheresis
    Aspirin
    Anagrelide
    Hydroxyurea
    Interferon-[agr ]

    Answer: C
    Explanation: Anagrelide is an oral imidazoquinazolin derivative that has been approved by the FDA as a platelet-lowering agent in essential thrombocythemia. It appears to lower the platelet count by interfering with the maturation of megakaryocytes. There are some side effects, but they are relatively mild in most cases. It should not be administered in cases of reactive thrombocytosis because their risk of complications from thrombocytosis is much less than in patients with thrombocytosis from inherent marrow disorder. Because essential thrombocytosis patients are at risk for hemorrhage as well as thrombosis, aspirin is not indicated in all cases. Hydroxyurea has a potential leukemogenic risk because it is a chemotherapeutic, although this risk has not been substantiated. Anagrelide lacks this potential risk because it is not a chemotherapeutic agent. Interferon has many more associated side effects with less efficacy. Thus, anagrelide appears to offer the best therapeutic window with the fewest risks and is the treatment of choice for essential thrombocythemia as long as it is tolerated by the patient.



    11. A 54-year-old white man is admitted to the hospital because of abdominal pain and "black stools." He has not seen a doctor in years. He smokes two packs of cigarettes daily. Physical exam reveals poor dentition, normal cardiovascular exam, moderate splenomegaly with mild epigastric and left upper quadrant tenderness, and a guaiac stool test positive for occult blood. Laboratory values reveal a hemoglobin of 9.5 g/dL, hematocrit of 29%, WBC count of 14,500/μL with a fairly normal differential, a platelet count of 540,000/μL, and a ferritin level of 4 μg/L. Serum vitamin B12 levels are elevated. A bone marrow exam shows hypercellularity without other specific findings, and chromosomes are reported as normal. Endoscopy reveals a gastric ulcer and biopsies are negative for malignancy but positive for Helicobacter pylori infection. Appropriate management at this stage should be

    Splenectomy
    Transfusion of two units of packed RBCs
    Observation
    Antibiotic treatment for the H. pylori infection and iron supplementation for the iron deficiency anemia
    Antibiotic treatment for the H. pylori infection

    Answer: E
    Explanation: Recognize that this patient has all of the manifestations of polycythemia rubra vera except that his bleeding gastric ulcer has masked the development of polycythemia. Because of his bleeding gastric ulcer, he has already become iron deficient, which is the goal of the cornerstone phlebotomy therapy for polycythemia vera. Instituting iron supplementation at this point may very well give the patient more morbidity because it could cause a rebound erythrocytosis. Rather, the H. pylori infection should be treated to cure the gastric ulcer, and a further work-up for a probable diagnosis of polycythemia vera should ensue, including culture of the patient's erythroid progenitor cells looking for EPO-independent colony growth, a hallmark for the diagnosis of polycythemia vera.


    Endocrinology

    1. A 51-year-old white man was recently diagnosed with a solitary 2.7-cm papillary cancer of the thyroid with no invasion of the capsule, no lymphadenopathy, and no distant metastases. He denies a history of head and neck irradiation, hoarseness, pain, dysphagia, or hemoptysis. His physical exam is otherwise normal, with no lab abnormalities. Which of the following measures is most appropriate for his management?

    Partial thyroidectomy followed by radioactive iodine (RAI) treatment
    Near-total thyroidectomy followed by RAI treatment
    Thyroid hormone treatment
    A and C
    B and C

    Answer: E
    Explanation: Thyroid cancer remains a significant medical problem in the United States; 12,000 new cases are diagnosed and 1000 deaths are reported each year. Differentiated thyroid cancer is classified into follicular and papillary (derived from the follicular cells) and medullary thyroid carcinoma (derived from the C cells). Rarely, the thyroid is the site of involvement by lymphoma. Anaplastic cancer arises from the papillary and follicular cancers. The most common type of thyroid cancer is papillary cancer, which accounts for approximately 70% of all thyroid cancers. It is two to three times more common in females and peaks in the third and fourth decades of life. Papillary cancer is usually nonencapsulated and sometimes multifocal and tends to spread by the lymphatic route. Follicular cancer is the second most common form of thyroid cancer, accounting for 15% of all thyroid cancers. It affects a slightly older age group and is more commonly diagnosed in females than in males. Follicular cancer tends to be encapsulated, is usually unifocal, and tends to spread via the hematogenous route; early metastases are seen with small lesions. Thyroid cancer is now diagnosed at an early stage, and its slow rate of growth makes for a favorable outcome in a majority of cases. Sometimes, however, thyroid tumors are encountered that display aggressive features leading to early death despite aggressive treatment. Moreover, the treatment modalities themselves can sometimes be attended by significant complications, making the optimum treatment of thyroid cancer a highly controversial issue. Therefore, an understanding of the factors that affect prognosis should guide selection of treatment modalities. In papillary cancer, prognosis is affected by tumor size, presence or absence of metastases, patient age, and degree of differentiation. Generally, the smaller tumors (<1.5 cm) carry an excellent prognosis in the absence of metastasis, whereas larger tumors (>2.5 cm) tend to carry a poorer prognosis. Patient age greater than 40 years at diagnosis tends to carry a poor prognosis in part because of poor concentration of iodine by most tumors. Poorly differentiated tumors tend to run a more aggressive course. The first line of treatment of thyroid cancer consists of surgical resection. Although the optimum procedure is not known, the more aggressive tumors should be managed with more extensive procedures (near-total or total thyroidectomy with or without lymph node dissection). RAI ablation should be considered when residual or metastatic disease is present. Finally, thyroid hormone treatment should be used with a goal of keeping the TSH level as low as possible without causing overt hyperthyroidism. RAI ablation and thyroid hormone suppression have been shown to reduce recurrence of thyroid cancer. In this patient, age and tumor size predict a poor outcome. Treatment should, therefore, consist of near-total thyroidectomy, RAI ablation, and thyroid hormone treatment.

    2. You saw a 71-year-old white woman nursing home resident who was brought in by her daughter for a complete physical exam. Her complaints include a poor appetite, weight loss, cramps, and weakness. She was diagnosed with Crohn's disease 10 years ago but is not taking any medications. Five months ago she had a mammogram and flexible sigmoidoscopy, both of which were normal. Because her exam was normal, she was given a 1-month return appointment and sent for blood work. At the end of the day, your lab calls to report a panic value of calcium of 1.4 mmol/L (normal range, 2.2 – 2.6 mmol/L) with an inorganic phosphate of 0.58 mmol/L (normal range, 0.8 – 1.5 mmol/L). She has a creatinine of 80 umol/L (normal range, 60 – 110 umol/L), albumin is 35 g/L (normal range, 37 – 49 g/L), and alkaline phosphatase is 250 U/L (normal range, 42-98 U/L). Which of the following diagnoses is compatible with these lab data?

    Hypoparathyroidism
    Hypomagnesemia
    Vitamin D deficiency
    Renal failure

    Answer: C
    Explanation: The causes of hypocalcemia, an abnormal reduction of serum calcium, can quickly be determined by examining the serum phosphorus, creatinine, and calcium. In hypoparathyroidism, there is reduced mobilization of calcium from bone, reduced renal reabsorption of calcium (along with decreased phosphaturia), and reduced formation of 1,25-hydroxyvitamin D, resulting in reduced intestinal absorption of calcium. Consequently, the hypocalcemia is accompanied by hyperphosphatemia. Hypoparathyroidism can be congenital or acquired; the latter is accounted for by transient or permanent disorders. Hypomagnesemia causes deficient secretion of PTH and consequent functional hypoparathyroidism. In vitamin D deficiency, decreased intestinal absorption of calcium leads to secondary hyperparathyroidism, which increases renal tubular loss of phosphate. Vitamin D deficiency can result from inadequate dietary intake, lack of sun exposure, and malabsorption. Renal failure impairs hydroxylation of 25-hydroxyvitamin D, which results in the malabsorption of calcium. The body compensates by increased secretion of PTH, leading to increased mobilization of calcium from bone. Renal failure is characterized by an abnormal serum creatinine, whereas renal dysfunction is not the critical pathogenetic feature of the other forms of hypocalcemia.

    3. A 38-year-old black woman draws your attention to a swelling in her neck, which she noticed 2 days ago. She denies palpitations, diaphoresis, and weight loss. There is no pain, hoarseness, or dysphagia. Her medical history is notable only for hypertension. Medications include only atenolol 50 mg once daily. On exam, blood pressure is 150/80 mm Hg; pulse is 70. There is a 2 × 1-cm nontender nodule on the right lobe of the thyroid. No lymphadenopathy is detected. The remainder of the exam is unremarkable. Electrolytes, blood urea nitrogen (BUN), creatinine, liver function tests, calcium, phosphorus, and CBC are normal. What would you do next?

    Elicit history of head and neck irradiation.
    Elicit a family history of thyroid cancer.
    Obtain thyroid function tests.
    Perform fine-needle aspiration.
    All of the above

    Answer: E
    Explanation: The clinically apparent (>1 cm) thyroid nodule is a common clinical finding; up to 5% of the population is affected. It is more common in women than in men, and a majority (85%) are hypofunctional or cold nodules. The likelihood of malignancy in a solitary thyroid nodule is low (4%); cold nodules carry a higher risk than hot nodules (20% vs. 1%). Evaluation of a solitary nodule should be aimed at detecting potentially malignant lesions so that as many cancers are removed with as few operations as possible. A history of head and neck irradiation raises the likelihood that a thyroid nodule is malignant, as does the presence of a family history of differentiated thyroid cancer or medullary cancer of the thyroid (which can be a component of multiple endocrine neoplasia <MEN> type IIA or IIB). Fine-needle aspiration of the thyroid gland is a cost-effective procedure with a high sensitivity and specificity for malignancy. Fine-needle aspiration allows the nodule to be characterized cytologically as benign, malignant, suspicious for malignancy, or indeterminate.

    4. A 60-year-old white man comes to see you for chronic back pain, which worsened 1 week ago. He has been wheelchair bound for 6 months because of severe osteoporosis with multiple lumbosacral spine fractures. He has severe asthma, which has required large doses of glucocorticoids for many years. The patient reports progressive loss of height and kyphosis over the past year. Other medications include albuterol and ipratropium inhalers and long-acting theophylline 300 mg twice a day. Significant physical findings include bilateral cataracts, multiple ecchymoses, and a prolonged expiratory phase with bilateral wheezes. Which of the following measures may be helpful?

    Testosterone replacement (only if he is deficient)
    Physical therapy
    Vitamin D replacement (only if his vitamin levels are low)
    Calcium supplementation
    Hydrochlorothiazide
    All of the above

    Answer: F

    5. Regarding the patient in question 4, which of the following underlies his osteoporosis?

    Decreased bone formation
    Increased bone loss
    Decreased calcium absorption from the GI tract
    Increased calcium loss in urine
    All of the above

    Answer: E
    Explanation: Glucocorticoids are used in the treatment of chronic inflammatory diseases of the lungs, connective tissue, and intestines as well as in transplantation because of their anti-inflammatory effect. When long-term treatment is required, several complications (e.g., cataracts, truncal obesity, skin-thinning, hyperglycemia) may be seen. A particularly disabling complication is bone loss, which can lead to fracture; it can occur with or without the other complications of chronic steroid treatment. The incidence of steroid-induced osteoporosis is unknown, but it appears to be related to the duration of treatment, half-life of the steroid, and its dose. Risk factors associated with increased bone loss include age, body mass index, and duration of use. Steroid-induced osteoporosis proceeds rapidly in the first 6 months of steroid use and slows thereafter. Trabecular bone and the cortical rim of the vertebral body are most susceptible to the effects of steroids. Steroids induce bone loss by several mechanisms. First, they inhibit calcium absorption in the GI tract while enhancing calcium loss in the kidneys. These effects induce secondary hyperparathyroidism, which leads to increased bone resorption. Second, they lower sex hormone levels through an effect on the gonadotropin levels and a direct effect at the gonadal level, as well as by decreasing adrenal sex steroid synthesis by inhibiting ACTH release. Third, they have a direct inhibitory effect on osteoblast proliferation, activity, and half-life, leading to decreased bone formation. Fourth, they induce proximal muscle weakness. Short-term studies showed that steroid-induced osteoporosis can be prevented or treated by using measures aimed at minimizing the negative effects of steroids on calcium and bone metabolism. Deficiency of sex steroids should be corrected. Physical therapy should be encouraged to prevent steroid-induced myopathy. Calcium and vitamin D supplementation and diuretics have been used to enhance calcium absorption and minimize calcium loss in urine, thereby preventing secondary hyperparathyroidism. Regular monitoring is recommended to prevent hypercalcemia.



    6. A 35-year-old black woman comes to see you for a complete physical exam. She has experienced cold intolerance, weakness, and constipation for 3 months. Her menses are regular but scanty. Her history is significant for hypertension and peptic ulcer disease, and her family history includes hypertension and diabetes. The patient is married but has never been pregnant and takes cimetidine 400 mg at bedtime, sustained-release nifedipine 60 mg daily, and docusate sodium 100 mg three times a day. Her pulse is 58 beats/minute with a blood pressure of 135/90 mm Hg. Her skin is dry and scaly, and she has hung-up reflexes. The rest of her exam is normal, and the following labs are obtained: serum chemistries are normal except for a creatine kinase of 300 U/L (normal range, 26-140 U/L); CBC is normal, free thyroxine (T4) is 6.4 pmol/L (normal range,10 – 22 pmol/L), and thyroid stimulating hormone (TSH) is 1.5 mIU (normal range, 0.3-5.0 mIU). Which of the following tests would you order?

    Free triiodothyronine (T3)
    Thyroid scan
    Thyroid uptake
    Pituitary magnetic resonance imaging (MRI)
    Antithyroid antibodies

    Answer: D
    Explanation: This patient has central hypothyroidism and should be evaluated for pituitary and end-organ function as well as the presence of a pituitary tumor. The prolactin level should be measured and the pituitary-adrenal, gonadal, and growth hormone axes assessed. The presence of a pituitary tumor can be determined by imaging the pituitary gland with MRI or CT scan. Where appropriate, this should be followed by evaluation of the visual fields. Measurement of the α subunit, a glycoprotein shared by FSH, LH, and TSH, may also be useful because some pituitary tumors secrete only this peptide.

    7. A 38-year-old black woman comes to you for renewal of her medications. She has had hypertension since her last pregnancy at age 30 and has been maintained on clonidine 0.2 mg twice a day. She gets headaches, dyspnea on exertion, swelling of her feet, and orthopnea but denies chest pain. Her father is also being treated for hypertension. She is married and does not smoke. She is five feet seven inches tall and weighs 257 pounds. Her blood pressure is 180/110 mm Hg; pulse is 92 beats/minute. The rest of her exam is remarkable for hypertensive retinopathy, bibasilar rales, and 1+ pitting edema bilaterally. Initial labs were normal except for a serum potassium of 3.0 mEq/L (normal range, 3.5- 5.0 mEq/L) and serum bicarbonate of 33 mEq/L (normal range, 22-28 mEq/L). You correct hypokalemia and obtain a random serum aldosterone level of 25 ng/dL (normal range, 5-30 ng/dL) with a plasma renin activity of 0.5 ng/mL/hour (normal range, 1.6- 7.4 ng/mL/hour) while the patient is on a normal diet. What additional tests might be appropriate?

    Adrenal computed tomography (CT) scan
    Adrenal vein sampling
    18-hydroxycorticosterone
    Saline loading test
    A, B, and C

    Answer: E
    Explanation: Primary aldosteronism, a disorder characterized by hypertension, hypokalemia, suppressed plasma renin activity, and increased aldosterone secretion, affects 0.05 to 2% of the hypertensive population. This disorder should be suspected in hypertensive patients in whom spontaneous or easily provoked hypokalemia develops that is slow to correct after discontinuation of diuretics. As important as recognizing the presence of primary aldosteronism is the differentiation of lesions that are surgically curable (60-70% of the cases in some series) from those that are best treated medically. In this patient, the presence of hypertension, hypokalemia, and alkalosis appropriately triggered screening for hyperaldosteronism, which led to the findings of an aldosterone-renin ratio of greater than 30, which constitutes a positive screening test. Aldosteronism can be confirmed by the finding of a 24-hour urine aldosterone secretion of 12 μg in the salt replete state. Adrenal imaging is the next step to differentiate adrenal adenoma from adrenal hyperplasia, although adenomas smaller than 1.5 cm can be missed and thus mistaken for hyperplasia. In confusing cases, adrenal vein sampling for aldosterone measurements is used to localize adenoma with a 95% accuracy. The finding of a lateralizing 10:1 aldosterone ratio in the presence of a symmetrical ACTH-induced cortisol rise diagnoses and localizes an adenoma. Other features suggestive of adenoma include plasma 18-hydroxy corticosterone of 100 ng/dL or more, spontaneous hypokalemia of less than 3 mEq/L, and an anomalous postural decrease of plasma aldosterone concentration. Saline loading is inappropriate in this patient because of heart failure and hypertensive retinopathy.



    8. A 27-year-old white woman was admitted 2 days ago through the emergency room for seizures. She has a history of moderate alcohol use. Two weeks ago she received benzathine penicillin for secondary syphilis. She is complaining of muscle cramps, weakness, and headache. She received 1 g of phenytoin on the day of admission and is now taking 100 mg three times a day. She is also taking acetaminophen, multivitamins, and tapering doses of chlordiazepoxide. There is a history of seizures in her family. She is 5 feet tall and weighs 120 pounds. Her blood pressure is 130/80 mm Hg; pulse is 90 beats/minute. The rest of the physical exam is normal except for a round face, a short neck, short fourth and fifth metacarpals, and bilateral cataracts. Abnormal labs include a calcium of 1.5 mmol/L (normal range, 2.2-2.6 mmol/L), phosphorus of 1.7 mmol/L (normal range, 0.8-1.4 mmol/L), and an intact parathyroid hormone (PTH) of 200 pg/mL (normal range, 15-65). Which of the following is most likely?

    Hypothyroidism
    Hypogonadism
    Basal ganglia calcification
    Mental retardation
    All of the above

    Answer: E
    Explanation: The findings of Albright's hereditary osteodystrophy (short stature, brachydactyly, and soft tissue calcification) along with severe hypocalcemia and elevated PTH are diagnostic of pseudohyperparathyroidism (Type IA). This is an autosomal-dominant disorder resulting from a G protein (Gs) defect, which leads to PTH resistance. Hypothyroidism and ovarian failure are also seen because Gs also couples to TSH and gonadotropin receptor signaling, respectively. Mental retardation is seen in 70% of cases.

    9. All of the following thyroid conditions are amenable to RAI treatment, except

    Papillary cancer
    Follicular cancer
    Graves' disease
    Thyroid lymphoma
    Multinodular goiter

    Answer: D
    Explanation: Iodine 131 is a radioactive isotope of iodine (RAI) that is selectively concentrated in the thyroid tissue and metabolized by the same pathways as naturally occurring iodine. This, together with its long half-life (8 days), allows it to deliver high doses of radiation to the thyroid gland (β-radiation) sufficient to destroy thyroid follicular cells. Thus, 131I is used in the treatment of Graves' disease, toxic multinodular goiter, and differentiated thyroid cancer. The doses of RAI used in the treatment of Graves' disease and toxic multinodular goiter are relatively low compared with those used in the treatment of thyroid cancer (in which it is used in conjunction with surgery). RAI has no place in the treatment of thyroid lymphoma because lymphoma cells do not concentrate iodine.

    10. Which of the following statements is/are true regarding PTH?

    Secretion is stimulated by hypocalcemia.
    Secretion is inhibited by hypercalcemia.
    The effect of magnesium on secretion is the same as that of calcium.
    Secretion is stimulated by low 1,25-hydroxyvitamin D and inhibited by high levels of 1,25-hydroxyvitamin D.
    A, B, and D
    All of the above

    Answer: D
    Explanation: PTH, an 84-amino-acid peptide synthesized and secreted by the parathyroid gland, is a potent regulator of the serum calcium level. Hypocalcemia stimulates the secretion of PTH acutely (with increased PTH synthesis and parathyroid cell hypertrophy and hyperplasia after chronic hypocalcemia), whereas hypercalcemia leads to decreased secretion of PTH. Hypomagnesemia inhibits PTH secretion. Elevated 1,25-dihydroxyvitamin D affects PTH synthesis and secretion by directly inhibiting the parathyroid gland and indirectly via hypercalcemia. Low levels of 1,25-dihydroxyvitamin D have the opposite effect.


    Rheumatology

    1. 43-year-old woman presents with a 3-year history of progressive rheumatoid arthritis that has been partially responsive to various nonsteroidal anti-inflammatory drugs (NSAIDs) and to low-dose oral corticosteroids. After the examination, you decide to treat her active arthritis with methotrexate, currently the most widely used and effective agent for rheumatoid arthritis. Some of the facts to tell her about methotrexate therapy include

    Therapeutic effects are delayed so that clinical improvement is not generally seen for 3 to 6 weeks after initiation of treatment.
    Adverse effects may include oral ulcers, nausea, vomiting, pneumonitis, bone marrow suppression, and cirrhosis.
    CBC, platelet count, alkaline phosphatase level, and serum glutamic-oxaloacetic transaminase (SGOT) level should be obtained every 4 to 6 weeks to monitor therapy.
    Birth control measures must be in use before methotrexate is started.
    All of the above.

    Answer: E
    Explanation: All of the answers are correct. Methotrexate is currently the best drug used to treat rheumatoid arthritis, with initial improvement seen in 3 to 6 weeks and peak efficacy in 4 to 6 months. Adverse effects such as nausea, abdominal pain, and diarrhea are frequently seen, but serious toxicity is rare. Methotrexate is taken orally (7.5-15 mg/week), and tolerance may be increased by spacing the oral doses over 1 to 2 days, giving a single intramuscular injection each week and daily folic acid (1 mg/day) supplementation. Laboratory tests such as CBC, platelet count, alkaline phosphatase, and SGOT are done every 4 to 6 weeks. The most toxic drug-related side effects are pancytopenia, neutropenia, thrombocytopenia, pneumonitis, and cirrhosis; all are reasons to stop the medications. Transient or sustained (1.5-2 times normal values) elevations in alkaline phosphatase and SGOT are commonly seen and, in the majority of patients, generally do not portend the development of hepatic fibrosis. Methotrexate is known to be teratogenic and should not be given to women with childbearing potential unless they are using an adequate method of birth control. Because of its potential effect on sperm, men should discontinue methotrexate 3 to 4 months before attempting conception.

    2. 54-year-old woman complains of severe right shoulder pain localized mainly to the midhumerus but also diffusely around the anterolateral shoulder. The onset was sudden and not precipitated by trauma. Physical examination reveals limited abduction with point tenderness over the subacromial bursa and the greater tuberosity of the humerus. A radiograph reveals a linear calcific density in the supraspinatus tendon. All of the following statements are true, except

    Treatment consists of cortisone injection into the subacromial bursa, NSAIDs, and physical therapy.
    The calcific density is most likely calcium urate.
    The diagnosis could not be made by an arthrocentesis.
    Local tendon injury may be the major cause.

    Answer: B
    Explanation: The clinical features and radiographic pattern are characteristic for calcific tendinitis, an extremely common rheumatic syndrome characterized by deposits of hydroxyapatite crystals within injured rotator cuff muscles near the humeral attachment region. It most commonly involves the supraspinatus tendon, but the infraspinatus and subscapularis tendons may also be involved. Conservative treatment is indicated and is successful in the vast majority of cases.

    3. A 74-year-old woman complains of worsening left knee pain with weight-bearing and ambulation. Examination of the knee reveals a small effusion without warmth, bony enlargement, and crepitus with flexion and extension of the knee. A diagnostic arthrocentesis is performed. Each of the following characteristics of the synovial fluid would be expected, except

    Pale yellow color
    Good viscosity
    Routine culture negative
    WBC count 800/mm3
    Glucose 22 mg/dL


    Answer: E
    Explanation: Clinically, the patient has osteoarthritis of the left knee. Synovial fluid in patients with osteoarthritis is typically "noninflammatory," meaning that the leukocyte count is less than 2000/mm3. A low level of glucose in the synovial fluid would not be found in this patient but is suggestive of septic arthritis.

    4. A 42-year-old woman with seropositive rheumatoid arthritis has become disabled by pain and tightness behind the right knee. Physical examination reveals cystic swelling over the popliteal fossa and semimembranous tendon. Which of the following is the most appropriate next step?

    Arthrogram of the right knee
    Synovial biopsy of the right knee
    Ultrasound study of the right knee popliteal fossa
    Venogram of right lower extremity
    None of the above

    Answer: C
    Explanation: The physical examination is suggestive of a distended Baker's cyst, but physical examination alone is not diagnostic, particularly if there has been a dissection or rupture. Ultrasonography has been found to be very useful in making a diagnosis of popliteal cyst with or without dissection. An arthrogram could also demonstrate a popliteal cyst but is less desirable because it is an invasive procedure. A venogram of the right lower extremity could be performed if a deep vein thrombosis was suspected clinically but would not be indicated in this case

    5. All of the following conditions involve the distal interphalangeal (DIP) joint, except

    Multicentric reticulohistiocytosis
    Erosive osteoarthritis
    Psoriasis with nail changes
    Juvenile chronic arthritis
    Rheumatoid arthritis

    Answer: E
    Explanation: Although hand involvement is very common in rheumatoid arthritis and occurs in approximately 95% of patients, DIP joint involvement is distinctly unusual. The most commonly involved joints in the rheumatoid hand are the PIPs, MCPs, and wrist joints in a symmetric manner.

    6. Rheumatoid factor may be present in each of these conditions, except

    Adult Still's disease
    Subacute bacterial endocarditis
    Vasculitis syndromes
    Sarcoidosis
    Sjögren's syndrome

    Answer: A
    Explanation: Patients with adult Still's disease are "seronegative" and lack serum rheumatoid factor. Rheumatoid factors are antibodies specific for the region of the Fc portion of human IgG. Although present in 75 to 80% of rheumatoid arthritis patients, primarily those with HLA-DR4 haplotype, they are by no means specific for this disorder and are found in normal individuals as well as patients with a variety of other inflammatory illnesses.

    7. An 82-year-old woman was hospitalized for treatment of congestive heart failure. She experienced a warm, painful right knee on the 3rd hospital day. The most appropriate procedure would be

    Blood cultures followed by IV antibiotics
    Arthrocentesis for diagnostic/therapeutic purposes
    IV colchicine
    Allopurinol
    Ultrasound study of right knee, including popliteal fossa

    Answer: B
    Explanation: Clinically, the patient has a monoarthritis most likely crystal induced, such as pseudogout or gout. She could also have septic arthritis, although this would be less likely. Gout and pseudogout can be rapidly and definitively diagnosed by proper examination of joint fluid, and infection can also be ruled out in this manner.

    8. A 46-year-old man on hemodialysis for 12 years complains of insidious onset of painful nocturnal dysesthesias involving the thumb and three fingers, relieved by shaking the hand. Physical examination of the hand reveals thenar wasting and numbness over the fingers. Each of the following statements is true, except

    Deposits of β2-microglobulin AH (amyloidosis associated with hemodialysis) amyloid compressing the median nerve could produce these findings.
    An entrapment neuropathy could explain these findings.
    Paresthesias involving the radial side of the thumb, second, third, and fourth fingers suggest compression of the medial nerve.
    Carpal tunnel syndrome could explain these findings.
    Deposits of amyloid of the primary type AL (amyloidosis associated with light chains) would be typical.

    Answer: E
    Explanation: Clinically, the patient has carpal tunnel syndrome, an entrapment neuropathy in which the median nerve is compressed within the carpal tunnel area. A new type of amyloid protein identified as β2-microglobulin has been demonstrated in bone and carpal tunnel tissue of patients undergoing long-term (usually greater than 10 years) hemodialysis. It is hoped that modifications of the dialysis membranes may result in improved β2-microglobulin clearance with diminished tissue deposition.

    9. Ophthalmologic manifestations of rheumatoid arthritis may include all of the following, except

    Secondary Sjögren's syndrome with sicca complex
    Scleritis
    Episcleritis
    Corneal melts
    Ischemic optic atrophy

    Answer: E
    Explanation: Ischemic optic atrophy is not routinely seen in patients with rheumatoid arthritis but may be a major ophthalmic manifestation of giant cell arteritis, Wegener's granulomatosis, and, less commonly, SLE.

    10. All of the following are characteristic patterns of joint involvement in rheumatoid arthritis, except

    Polyarticular involvement
    Oligoarticular involvement
    Symmetrical involvement
    Involvement of the proximal interphalangeal (PIP), metacarpophalangeal (MCP) wrist, and metatarsophalangeal (MTP) joints
    Frequent cervical spine involvement

    Answer: B
    Explanation: Clinically, rheumatoid arthritis is a symmetrical polyarthritis especially involving the PIP, MCP, wrist, and MTP joints. In many of these joints, definite articular deformities will develop over time. Cervical spine involvement is common. Rarely is an oligoarticular pattern observed except in the early course of this illness.

    11. A 32-year-old woman presents with left inguinal and groin pain of 1 week duration that is worse with weightbearing and ambulation. Physical examination reveals full range of motion of the left hip. She walks with a limp. She had previously been treated with mechlorethamine, vincristine, procarbazine, and prednisone therapy for Hodgkin's disease. An anteroposterior film of the pelvis demonstrates no osseous abnormality. Which of the following tests would be most useful in making the diagnosis?

    Serum rheumatoid factor
    Erythrocyte sedimentation rate
    Magnetic resonance imaging (MRI) of the left hip
    Arthrogram of the left hip
    Blood alcohol level

    Answer: C
    Explanation: Osteonecrosis is one of the most common causes of hip pain and incapacity in patients with a variety of diseases who have been treated with corticosteroids. A major problem in diagnosing osteonecrosis relates to the lag between the onset of symptoms (pain and limp) and defined radiographic changes. MRI has been shown to be extremely valuable in evaluating high-risk patients who are symptomatic but radiographically normal.

    12. Extra-articular manifestations of rheumatoid arthritis that may be associated with severe morbidity or mortality include

    Rheumatoid vasculitis
    Pericarditis
    Cachexia
    Rheumatoid nodule within the aortic valve
    All of the above

    Answer: E
    Explanation: All of the answers are correct. Rheumatoid arthritis may be associated with a number of systemic features that may be associated with severe morbidity or mortality.

    13. A 50-year-old white man is transferred to your hospital with a presumptive diagnosis of tuberculosis. His chest radiograph shows nodular cavitary lesions in both lung fields. His urinalysis shows 50 RBCs per high power field and 3+ proteinuria. He is scheduled for bronchoscopy with transbronchial lung biopsy in the morning. That evening he has a sudden deterioration consisting of massive hemoptysis and progressive renal failure. The most appropriate therapeutic intervention at this point would be supportive management and

    IV corticosteroids
    Antituberculous medications
    IV cyclophosphamide 4 mg/kg
    Oral cyclophosphamide 2 mg/kg
    IV corticosteroids and IV cyclophosphamide 4 mg/kg

    Answer: E
    Explanation: The involvement of the lower respiratory tract as well as renal involvement suggests Wegener's granulomatosis. Treatment of Wegener's granulomatosis with cyclophosphamide has resulted in marked improvement in outcome of this condition. Because of the severity and sudden deterioration, IV corticosteroids and IV cyclophosphamide would be indicated.

    14. Each of the following is characteristic of polymyalgia rheumatica, except

    Mild joint inflammation
    Stiffness of the shoulder and hip girdles
    Weakness of the shoulder and hip girdles
    Elevated erythrocyte sedimentation rate
    Normochromic normocytic anemia

    Answer: C
    Explanation: Polymyalgia rheumatica is a common clinical syndrome in patients older than 55 years and is characterized by stiffness and soreness in the shoulder and hip girdle areas. It is sometimes associated with mild joint swelling. Laboratory findings include mormocytic/normochromic anemia and elevated sedimentation rate. Weakness of the proximal upper and lower extremity muscles is distinctly unusual and suggests a proximal myopathy such as polymyositis.

    15. A 74-year-old man is noted to have purplish-discolored right third and fourth toes 4 days after coronary angiography and a creatinine level of 2.4 mg/dL (creatinine level was normal on admission). He has a history of adult-onset diabetes mellitus, hypertension, and 50 pack-years of smoking. Cholesterol crystal atheromatous embolization is suspected. Which of the following may be present?

    Livedo reticularis
    Elevated erythrocyte sedimentation rate and/or leukocytosis and/or eosinophilia
    Prominent gastrocnemius pain or claudication
    Source(s) of the cholesterol emboli are usually the abdominal aorta or iliofemoral arteries rather than the more distal arteries.
    All of the above

    Answer: E
    Explanation: All of the answers are correct. Cholesterol crystal (atheromatous) embolization is a common occurrence in patients with advanced atherosclerotic disease but is frequently either not recognized or misdiagnosed as "vasculitis." The exact incidence is currently unknown, but it is associated with significant morbidity and mortality. With a rise in the number of geriatric patients with arthrosclerosis, the recognition of this disorder is critical to prevent unnecessary diagnostic studies and treatment with high-dose corticosteroids/cytotoxic agents, which are of no benefit. The source of most cholesterol emboli is the abdominal aorta or iliofemoral arteries, but cardiac and thoracic aorta sources have been described


    mak Posted: Mon Apr 18, 2005 5:38 pm Post subject: thanks

    --------------------------------------------------------------------------------
    thanks for q


    Anonymous Posted: Wed Apr 20, 2005 2:47 pm Post subject: Great work!

    --------------------------------------------------------------------------------
    Dear ABCIXIMAB,
    Thanks a lot for all the questions and the detailed explanations. Great way to learn and prepare for the exam.
    Allaboutielts


    babu145145 Posted: Thu Apr 21, 2005 12:31
  2. drkash

    drkash Guest

    dear friend ;well done & thanks a lot for this great effort
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