WBR0624

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 34 year old Caucasian female, comes to the office with complaints of fatigue, lethargy, malaise, anorexia, nausea and abdominal pain for the past few weeks. She has been having fever on and off and an increase in the frequency of stools of 4-5 times a day over the past month with a single episode of blood in stools. Her past history is insignificant and her family history is unremarkable. She has no recent travel exposure outside the country. She denies smoking and drugs, but consumes alcohol occasionally. Her menstrual cycles are normal. Her temperature is 36.7 C, blood pressure is 130/70 mmHg, pulse is 102/min and respirations are 16/min. The physical examination is normal except for mild abdominal pain on the right upper quadrant and epigastric area. Serum biochemistry results shows Na:135 mEq/L, K: 3 mEq/L, Cl:104mEq/L, Bi: 24 mEq/L, BUN: 30 mg/dl, glucose:226 mg/dl, Mg:1mg/dl and Ca: 8mg/dl. The liver fuction test results are as follows: Alanine aminotransferase : 110 U/L Aspartate aminotransferase : 100 U/L Alkaline phosphatase : 130 U/L Total bilirubin : 1.3 mg/dl Direct bilirubin : 1.3 mg/dl Hepatits A IgM : negative HbsAg : negative HBc Antibody : negative HCV Antibody : negative What is the most likely diagnosis in this patient ?]]
Answer A	AnswerA::Alcoholic hepatitis
Answer A Explanation	[[AnswerAExp::Incorrect : Alcoholic liver disease usually have highly elevated AST and ALT levels with a very clear history of heavy alcohol intake.]]
Answer B	AnswerB::Primary sclerosing cholangitis
Answer B Explanation	[[AnswerBExp::Incorrect : Primary sclerosing cholangitis (PSC) is usually a progressive disorder that ultimately leads to complications of cholestasis and hepatic failure with underlying ulcerative colitis. Liver biochemical tests usually demonstrate a cholestatic pattern, with elevation of the serum alkaline phosphatase predominating in most patients. The serum aminotransferases are typically less than 300 IU/L. The serum alkaline phosphatase and bilirubin may fluctuate substantially, possibly indicating transient blockage of strictured bile ducts by biliary sludge or small stones.]]
Answer C	AnswerC::Viral hepatitis
Answer C Explanation	[[AnswerCExp::Incorrect : Negative serology rules out viral hepatitis, which would have elevated AST, ALT and bilirubin.]]
Answer D	AnswerD::Primary biliary cirrhosis
Answer D Explanation	[[AnswerDExp::Incorrect : Primary biliary cirrhosis (PBC) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. The signs and symptoms of PBC usually include pruritus, hyperpigmentation of the skin, and hepatomegaly in a woman with serum alkaline phosphatase concentration is almost always elevated, often to striking levels. Serum levels of 5'-nucleotidase and gammaglutamyl transpeptidase parallel those of alkaline phosphatase. The serum levels of aminotransferases may be normal or slightly elevated and antimitochondrial antibodies are the serologic hallmark of PBC.]]
Answer E	AnswerE::Autoimmune hepatitis
Answer E Explanation	AnswerEExp::'''Correct''' : Autoimmune hepatitis is characterized by elevated AST and ALT with normal bilirubin and ALP levels. Patients are associated with other autoimmune conditions like ulcerative colitis as in this patient.
Right Answer	RightAnswer::E
Explanation	[[Explanation::Autoimmune hepatitis (AH) is characterized by circulating autoantibodies and a high serum globulin level. They of two types : type 1 autoimmune hepatitis ( classic autoimmune hepatitis), characterized by circulating antibodies to nuclei (ANA) and/or smooth muscle (ASMA) and type 2 autoimmune hepatitis, defined by the presence of antibodies to liver/kidney microsomes (ALKM-1) and/or to a liver cytosol antigen (ALC-1). Diseases commonly seen with autoimmune hepatitis include hemolytic anemia, immune thrombocytopenia (ITP), type 1 diabetes mellitus, thyroiditis, celiac sprue, ulcerative colitis (which is more often associated with primary sclerosing cholangitis) and a polyglandular autoimmune syndrome can occur in children with type 2 autoimmune hepatitis. It is important to distinguish AH from other forms of liver disease, as a large percentage of patients respond to therapy and therapy has been shown to delay transplantation, improve the quality of life as well as prolong survival. Lupoid hepatitis (also called Autoimmune hepatits) is an auto-immune disease which causes liver cirrhosis. It may be associated with systemic lupus erythematosus (SLE) or other connective tissue disorders. Patients may have chronic hepatitis that mimic viral hepatitis, but without serologic evidence of a viral infection. The disease usually affects women and although the disease is chronic, many patients with autoimmune hepatitis present acutely ill with jaundice, fever and sometimes symptoms of severe hepatic dysfunction, a picture that resembles acute hepatitis. Educational Objective: References: ]]
Approved	Approved::Yes
Keyword	WBRKeyword::Autoimmune hepatitis, WBRKeyword::Primary biliary cirrhosis, WBRKeyword::Primary sclerosing cholangitis
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