tommyk
10-05-2006, 11:14 AM
Hy 2418 Liver Pathology. You gotta know this one…MUST KNOW TV!
55-year-old man named Hayden Christensen presents to you with severe abdominal pain. Physical examination demonstrates tender, smooth hepatomegaly and mild jaundice. Serum chemistries demonstrate an aspartate aminotransferase (AST) and alanine aminotransferase (ALT) of over ten times the upper limit of normal. The hematocrit is 70%. The patient has no known history of smoking or lung disease or alcohol abuse. Complete blood count shows an increased number in all cell lines, with the most marked elevation in the erythrocyte line (Biggie Fry hint). The morphology of the cells in the peripheral smear is normal. Which of the following is the most likely cause of this patient's liver function abnormalities? What is the red blood cell disease called???
1-Budd-Chiari syndrome
2-Alcoholic hepatitis
3-Alcoholic cirrhosis
4-Hepatitis A
5-Hep B
6-Hep C
7-Hep D
8-Hep E
9-Hep F (Just kidding)
10-Cholecystiis
11-Cholangitis
12-Cholelithiasis
13-Fulminant Hepatitis
14-Parasitic infection
a) ans is #1, Budd-Chiari syndrome. This patient has probable polycythemia vera (a below normal erythropoietin level would be a helpful confirmatory study) which is presenting with Budd-Chiari syndrome. Budd-Chiari syndrome is a rare disorder with high mortality rate usually caused by the thrombosis of major hepatic veins. It can present either acutely, as in this case, or more slowly with chronic vague abdominal pain and portal hypertension. Underlying predisposing causes include myeloproliferative disorders (notably polycythemia vera, as in this case); other hematologic disorders (sickle cell disease, paroxysmal nocturnal hemoglobinuria); defective inhibition of coagulation (antiphospholipid antibodies, low antithrombin III, low protein C, low protein S, and low factor V Leiden); possibly estrogens (oral contraceptives, pregnancy); and local liver disease (malignancies, trauma, or suppurative lesions). Alcoholic cirrhosis can cause jaundice, but AST and ALT elevations are usually not very high. Further, nothing in this man's history suggests significant alcohol use. Cholelithiasis and the other stuff can cause obstructive jaundice when a small stone occludes a bile duct, but AST and ALT elevations are usually not very high. No risk factors are noted for either hep A or chronic hepatits B, C, D, etc. or fulminant hepatitis. Chronic viral hepatitis does not usually have very high elevations of AST or ALT, but fulminant hepatitis, which Budd-Chiari syndrome mimics, can. KNOW your liver inside and out!
55-year-old man named Hayden Christensen presents to you with severe abdominal pain. Physical examination demonstrates tender, smooth hepatomegaly and mild jaundice. Serum chemistries demonstrate an aspartate aminotransferase (AST) and alanine aminotransferase (ALT) of over ten times the upper limit of normal. The hematocrit is 70%. The patient has no known history of smoking or lung disease or alcohol abuse. Complete blood count shows an increased number in all cell lines, with the most marked elevation in the erythrocyte line (Biggie Fry hint). The morphology of the cells in the peripheral smear is normal. Which of the following is the most likely cause of this patient's liver function abnormalities? What is the red blood cell disease called???
1-Budd-Chiari syndrome
2-Alcoholic hepatitis
3-Alcoholic cirrhosis
4-Hepatitis A
5-Hep B
6-Hep C
7-Hep D
8-Hep E
9-Hep F (Just kidding)
10-Cholecystiis
11-Cholangitis
12-Cholelithiasis
13-Fulminant Hepatitis
14-Parasitic infection
a) ans is #1, Budd-Chiari syndrome. This patient has probable polycythemia vera (a below normal erythropoietin level would be a helpful confirmatory study) which is presenting with Budd-Chiari syndrome. Budd-Chiari syndrome is a rare disorder with high mortality rate usually caused by the thrombosis of major hepatic veins. It can present either acutely, as in this case, or more slowly with chronic vague abdominal pain and portal hypertension. Underlying predisposing causes include myeloproliferative disorders (notably polycythemia vera, as in this case); other hematologic disorders (sickle cell disease, paroxysmal nocturnal hemoglobinuria); defective inhibition of coagulation (antiphospholipid antibodies, low antithrombin III, low protein C, low protein S, and low factor V Leiden); possibly estrogens (oral contraceptives, pregnancy); and local liver disease (malignancies, trauma, or suppurative lesions). Alcoholic cirrhosis can cause jaundice, but AST and ALT elevations are usually not very high. Further, nothing in this man's history suggests significant alcohol use. Cholelithiasis and the other stuff can cause obstructive jaundice when a small stone occludes a bile duct, but AST and ALT elevations are usually not very high. No risk factors are noted for either hep A or chronic hepatits B, C, D, etc. or fulminant hepatitis. Chronic viral hepatitis does not usually have very high elevations of AST or ALT, but fulminant hepatitis, which Budd-Chiari syndrome mimics, can. KNOW your liver inside and out!