新生儿和婴儿胆汁淤积(英文版）.ppt

Neonatal and Infantile Cholestasis Ying-kit Leung, MD, FAAP President, Hong Kong Society of Paediatric Gastroenterology, Hepatology and Nutrition, Yantai, Shandong, July 2006 DEFINITION Neonatal cholestasis is defined as conjugated hyperbilirubinemia developing within the first 90 days of extrauterine life. Conjugated bilirubin exceeds 1.5 to 2.0 mg/dl. Conjugated bilirubin generally exceeds 20% of the total bilirubin. Bilirubin Production BilirubinUptake, Conjugation, Excretion EXTRAHEPATIC ETIOLOGIES Extrahepatic biliary atresia Choledochal cyst Bile duct stenosis Spontaneous perforation of the bile duct Cholelithiasis Inspissated bile/mucus plug Extrinsic compression of the bile duct INTRAHEPATIC ETIOLOGIES Idiopathic Toxic Genetic/Chromosomal Infectious Metabolic Miscellaneous INTRAHEPATIC ETIOLOGIES Idiopathic Neonatal Hepatitis Toxic TPN-associated cholestasis Drug-induced cholestasis Genetic/Chromosomal Trisomy 18 Trisomy 21 INTRAHEPATIC ETIOLOGIES Infectious Bacterial sepsis (E. coli, Listeriosis, Staph. aureus) TORCHES Hepatitis B and C Varicella Coxsackie virus Echo virus Tuberculosis INTRAHEPATIC ETIOLOGIES Metabolic Disorders of Carbohydrate Metabolism Galactosemia Fructosemia Glycogen Storage Disease Type IV Disorders of Amino Acid Metabolism Tyrosinemia Hypermethioninemia INTRAHEPATIC ETIOLOGIES Metabolic (cont.) Disorders of Lipid Metabolism Niemann-Pick disease Wolman disease Gaucher disease Cholesterol ester storage disease Disorders of Bile Acid Metabolism 3B-hydroxysteroid dehydrogenase/isomerase Trihydroxycoprostanic acidemia INTRAHEPATIC ETIOLOGIES Metabolic (cont.) Peroxisomal Disorders Zellweger syndrome Adrenoleukodystrophy Endocrine Disorders Hypothyroidism Idiopathic hypopituitarism INTRAHEPATIC ETIOLOGIES Metabolic (cont.) Miscellaneous Metabolic Disorders Alpha-1-antitrypsin deficiency Cystic fibrosis Neonatal iron storage disease North American Indian cholestasis INTRAHEPATIC ETIOLOGIES Miscellaneous Arteriohepatic dysplasia (Alagille s