tailieunhanh - Metabolic Liver Disease in Children

In utero, the placenta is the main site for gas exchange for the developing foetus and the blood flow to the foetal lung is minimal. Blood from the right ventricle bypasses the lungs and passes directly from the pulmonary artery to the aorta via a foetal vessel called the arterial duct. After birth, a number of changes occur in transition from the foetal to the newborn circulation including expansion of the lungs (which reduces pulmonary vascular resistance) and closure of the arterial duct, so that blood now perfuses the lungs. There are certain severe congenital cardiac lesions that are only. | LIVER TRANSPLANTATION 14 713-733 2008 ERRATUM Due to a clerical oversight the following article by Hansen and Horslen published in Liver Transplantation 14 391-411 did not include revisions made by the authors after its acceptance. The publisher regrets this error and apologizes to Drs. Hansen and Horslen as well as the Liver Transplantation readership. Metabolic Liver Disease in Children Keli Hansen1 and Simon Horslen1 Division of Transplant Surgery and Division of Gastroenterology Children s Hospital and Regional Medical Center Seattle WA The aim of this article is to provide essential information for hepatologists who primarily care for adults regarding liver-based inborn errors of metabolism with particular reference to those that may be treatable with liver transplantation and to provide adequate references for more in-depth study should one of these disease states be encountered. Liver Transpl 14 713-733 2008. 2008 AASLD. Received January 11 2008 accepted February 4 2008. Inborn errors of metabolism are caused by single enzyme defects that result in abnormalities in the synthesis or catabolism of proteins carbohydrates or fats. Most are due to a defect in an enzyme or transport protein that alters a metabolic pathway. This group of diseases differs from what is called metabolic disease in the adult or more accurately metabolic syndrome which includes visceral obesity elevated triglycerides elevated fasting blood sugar high blood pressure and a decrease in high-density lipoprotein cholesterol levels. This group of diseases can be divided into 1 diseases that lead to structural liver damage with liver failure or cirrhosis with or without injury to other tissues such as alpha-1-antitrypsin deficiency A1ATD and cystic fibrosis CF and 2 diseases due to a metabolic defect expressed solely or predominantly in the liver but lead Abbreviations A1ATD alpha-1-antittypsin deficiency AGT alanine glyoxylate aminotransferase AL argininemia ALT alanine aminotransferase AS .

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