Wilson’s Disease (Hepatology. 2003;37:1475-92) DEFINITION: Autosomal recessive inherited disorder of copper overload secondary to deficiency of an enzyme derived from liver cells (↓ secretion) Other inheritable forms of liver disease:…
Transplantation (Hepatology. 2005;41:1-26) REFERRAL: Decision ultimately rests with the transplant center Transplant when the natural history of liver disease suggest a better survival with transplant United Network for Organ Sharing…
Transplant Immunosuppression Steroids: broad immunosuppressive antibodies Suppress antibody production and ability to recognize antigen, inhibit IL-2 release from macrophages Used for both acute rejection and maintenance Dose varies widely; As…
Pulmonary Complications HEPATOPULMONARY SYNDROME (HPS) Definition: HPS triad: History of chronic liver disease; Arterial hypoxemia on room air; Presence of intrapulmonary vascular dilation Types of HPS: Type 1: Diffuse pulmonary…
Pregnancy Pearls (Am J Gastroenterol. 2004;99:2479-88 & 1999;94:1728-32) GENERAL Liver histology and function do not change during pregnancy Maternal blood volume and cardiac output increase, without corresponding increase in hepatic…
Primary Sclerosing Cholangitis (PSC) DEFINITION: Idiopathic cholestasis with fibrosis, stricturing and dilation of intra- and extrahepatic bile ducts (small and large bile ducts) EPIDEMIOLOGY: Young men (age 20-50) May be…
Primary Biliary Cirrhosis (PBC) (NEJM 2005;353:1261-73. Hepatology. 2000;31:1005-13) DEFINITION: PBC: Autoimmune destruction of intrahepatic bile ducts (small bile ducts) Autoimmune Cholangitis or AMA-Negative PBC: (may see positive ANA and/or ASMA)…
Non-Alcoholic Fatty Liver Disease (NAFLD)/Non-Alcoholic Steatohepatitis (NASH)/Non-Alcoholic Fatty Liver (NAFL) (Dig Dis Sci 2005:50;171-80. Clin Gastroenterol Hepatol. 2004;2:1048-58. Gastroenterology. 2002:123: 1702-04 & 1705-25) DEFINITION: The presence of macrovesicular fatty changes…
Mass (Focal Liver) Evaluation DEFINITION: Benign or Malignant, Primary (originating in the liver) or Secondary (metastases), lesions of the liver EPIDEMIOLOGY: See Etiologies below for specific conditions ETIOLOGIES: Differential Diagnosis…
Liver Function Tests (Gastroenterology. 2002;123:1364-1384; Clin Chem 2000;12:1-60) Adapted, in part, from Pocket Medicine 2nd ed. 2004. Lippincott Wilkins & Williams TESTS OF HEPATIC FUNCTION: Albumin: general marker for liver…
