cholestasis - NEONATAL CHOLESTASIS Gregory J. Semancik,...

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NEONATAL CHOLESTASIS Gregory J. Semancik, M.D. Major, Medical Corps, U.S. Army Fellow, Pediatric Gastroenterology and Nutrition Walter Reed Army Medical Center
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OBJECTIVES Know the differential diagnosis for neonatal cholestasis. Understand how to evaluate the neonate with conjugated hyperbilirubinemia. Know the therapeutic management of neonates with cholestasis.
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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.
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ETIOLOGIES Basic distinction is between: Extrahepatic etiologies Intrahepatic etiologies
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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
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INTRAHEPATIC ETIOLOGIES Idiopathic Toxic Genetic/Chromosomal Infectious Metabolic Miscellaneous
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INTRAHEPATIC ETIOLOGIES Idiopathic Neonatal Hepatitis Toxic TPN-associated cholestasis Drug-induced cholestasis Genetic/Chromosomal Trisomy 18 Trisomy 21
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INTRAHEPATIC ETIOLOGIES Infectious Bacterial sepsis (E. coli, Listeriosis, Staph. aureus) TORCHES Hepatitis B and C Varicella Coxsackie virus Echo virus Tuberculosis
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INTRAHEPATIC ETIOLOGIES Metabolic Disorders of Carbohydrate Metabolism Galactosemia Fructosemia Glycogen Storage Disease Type IV Disorders of Amino Acid Metabolism Tyrosinemia Hypermethioninemia
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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
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INTRAHEPATIC ETIOLOGIES Metabolic (cont.) Peroxisomal Disorders Zellweger syndrome Adrenoleukodystrophy Endocrine Disorders Hypothyroidism Idiopathic hypopituitarism
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cholestasis - NEONATAL CHOLESTASIS Gregory J. Semancik,...

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