10 años. Un 66,8% estaba eutrófico (IMC p10-85), SP 13,9% (IMC p85-95), O 12% (IMC > p95) y bajo peso (BP) 7,3% (IMC < p10), sin diferencias según sexo ni grupo etario. Conclusiones: La prevalencia de BP, SP y O en CLC es similar a la observada en niveles socioeconómicos medio-bajos chilenos. La mayor proporción entre 6-10 años hace indispensable su prevención desde la etapa de lactante]]>
Familial Intrahepatic Cholestasis (FIC) includes a heterogeneous group of recessive autosomic alterations characterized by hepatocellular cholestasis secondary to the interruption of the normal process of synthesis of bilis. Objective: A description of FIC in 3 of 5 children of an index family. Clinical case: a 5 y.o. child with hepatosplenomegaly increased serum hepatic enzymes and biliary acids. Abdominal echography showed alterations compatible with hepatic fibrosis. Biopsy showed bridge fibrosis, duct proliferation, minimal chronic cholestasis. These findings were compatible with a phenotype FIC-3 with elevate levels of Gamma-glutamyl transferase. A mutation of MDR3 gene is responsible for the absence of biliary phospholipids, allowing a detergent effect of biliary acids upon the duct epithelium, developing cholangitis, fibrosis and later cirrhosis. Among four brothers, the mutation was found in two twin sisters. Three affected brothers were treated with ursodeoxicolic acid, 30 mg/Kg. Excellent results were obtained in the twin girls not in the index boy. The clinical expression of this illness is variable, and an elevation of aminotransferase must call attention to this possibility. Early diagnostic and treatment could avoid the development of hepatic damage and cirrhosis.
Acute Fulminant HB s Ag Hepatitis in an Infant Born to a Chronic HBV Carrier Mother The incidence of Hepatitis B Virus (HBV) chronic asymptomatic carriers is low (0.35%) among the Chilean population. This is the clinical report of an infant, born to a HB s Ag carrier mothej, that developed a fulminant form of hepatitis at the age of three months. His mother was asymptomatic, but a brother died at the same age, nine years before, from acute fulminant HB s Ag hepatitis. A maternal aunt also died, three years before, from an hepatic carcinoma. The advise to routinely test for HB s Ag those mothers known to be at high risk of HBV infection is stressed. A recently recommended prophylactic schedule with anti HBV inmuneglobuline and HBV vaccine is described.
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