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Harrison's Principles of Internal Medicine, 18e | Part 14. Disorders of the Gastrointestinal System > Section 2. Liver and Biliary Tract DiseaseĀ > | Chapter 309. Genetic, Metabolic, and Infiltrative Diseases Affecting the Liver Sections: Genetic, Metabolic, and Infiltrative Diseases Affecting the Liver: Introduction, Further Readings. Topics Discussed: liver diseases. Excerpt:"There are a number of disorders of the liver that fit within the categories of genetic, metabolic, and infiltrative disorders. Inherited disorders include hemochromatosis, Wilson's disease, 1 antitrypsin ( 1AT) deficiency, and cystic fibrosis (CF). Hemochromatosis is the most common inherited disorder affecting Caucasian populations, with the genetic susceptibility for the disease being identified in 1 in 250 individuals. Over the past 15 years, it has become increasingly apparent that nonalcoholic fatty liver disease (NAFLD) is the most common cause of elevated liver enzymes found in the U.S. population. With the obesity epidemic in the United States, it is estimated that 20% of the population may have abnormal liver enzymes on the basis of NAFLD and 3% may have nonalcoholic steatohepatitis (NASH). Infiltrative disorders of the liver are relatively rare.Hereditary hemochromatosis (HH) is a common inherited disorder of iron metabolism (Chap. 357). Our knowledge of the disease and its phenotypic expression has changed since 1996, when the gene for HH, called HFE, was identified, allowing for genetic testing for the two major..."
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