WBR0626

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Author [[PageAuthor::Mugilan Poongkunran M.B.B.S [1]]]
Exam Type ExamType::USMLE Step 3
Main Category MainCategory::Primary Care Office
Sub Category SubCategory::Gastrointestinal
Prompt [[Prompt::A 40 year old female, comes to the office with complaints of fatigue, lethargy, malaise and pruritus for the past few weeks. Her past history is insignificant and her family history is unremarkable. She has no recent travel exposure outside the country. She denies smoking, alcohol and drugs. Her menstrual cycles are irregular with increased duration of cycles. Her temperature is 36.7 C, blood pressure is 130/70 mmHg, pulse is 102/min and respirations are 16/min. On examination there is no jaundice, but xanthelasmas and excoriations from scratches are present on skin examination. Abdomen is soft and non-tender with no organomegaly. Other system examinations are normal . Her lab results shows Hb:7 g/dl, hematocrit : 33, Na:135 mEq/L, K: 3 mEq/L, Cl:104mEq/L, Bi: 24 mEq/L, BUN: 30 mg/dl, glucose:176 mg/dl, Mg:1mg/dl and Ca: 8mg/dl. The liver fuction test results are as follows:

Alanine aminotransferase : 100 U/L Aspartate aminotransferase : 90 U/L Alkaline phosphatase : 600 U/L Total bilirubin : 1 mg/dl Direct bilirubin : 0.6 mg/dl Hepatits A IgM : negative HbsAg : negative HBc Antibody : negative HCV Antibody : negative

This patient is at the risk of developing which of the following ?]]

Answer A AnswerA::Hypogondism
Answer A Explanation [[AnswerAExp::Incorrect : Hypogonadism is seen in patients with hemochromatosis and not in primary biliary cirrhosis.]]
Answer B AnswerB::Osteoporosis
Answer B Explanation [[AnswerBExp::Correct : Bone disease is a frequent complication of PBC, due to malabsoption of vitamin D. Thus it is important to screen patients for osteoporosis and osteomalacia in these patients.]]
Answer C AnswerC::Hypothyroidism
Answer C Explanation [[AnswerCExp::Incorrect : Primary biliary cirrhosis is associated with other autoimmune diseases like rheumatoid arthritis, Hashimoto’s thyroidism but does not lead to it as a complication.]]
Answer D AnswerD::Coronary artery disease
Answer D Explanation [[AnswerDExp::Incorrect : The risk of atherosclerosis in patients with PBC and hypercholesterolemia is low, despite the severity of the disease.]]
Answer E AnswerE::Endometrial cancer
Answer E Explanation [[AnswerEExp::Incorrect : Primary biliary cirrhosis has no relation to endometrial cancer.]]
Right Answer RightAnswer::B
Explanation [[Explanation::Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts (bile canaliculi) within the liver. When these ducts are damaged bile builds up in the liver (cholestasis) and over time damages the tissue. Serum alkaline phosphatase concentration is almost always elevated, often to striking levels with serum levels of 5'-nucleotidase and gammaglutamyl transpeptidase paralleling alkaline phosphatase levels. Bone disease is a frequent complication of PBC, due to malabsoption of vitamin D.

Educational Objective:
References: ]]

Approved Approved::Yes
Keyword WBRKeyword::Primary biliary cirrhosis
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