[The neonatal hepatitis syndrome].

Abstract

Syndrome of Neonatal hepatitis and atresia of extrahepatic bile ducts are the most frequent causes of jaundice with conjugated bilirubin in the small infant. The neonatal hepatitis is diagnosed by its plurietiologic character, onset in the first 3 months, subacute or chronic, potentially cirrhogenous, evolution, conjugated hyperbilirubinemia and mainly, gigantocellular hepatic transformation--the essential characteristic of the diagnosis. Etiology of neonatal hepatitis has extremely diverse causes: infectious causes, genetic diseases of metabolism, toxic causes, post-hemolytic states, neonatal acute hepatic necroses, parenteral nutrition, chromosomal anomalies, familial syndromes, etc.; there exists also a form with nonspecific cause (idiopathic form). Practically, neonatal hepatitis might often be mistaken for atresia of the extrahepatic bile ducts. In the latter case, the temporization of the surgery (bilidigestive anastomosis) for more than 2 months of cholestasis evolution leads to the appearance of hepatic cirrhosis lesions. The authors analyze various clinical, biological and histopathological elements which permit the differentiation in due time of the two situations that require different therapeutic attitudes. Except for certain situations, which allow an etiologic treatment, the main therapeutic element (pathogenic) is corticotherapy and several additional measures. The paper concludes with appreciations on the evolution, prognosis and prophylaxis possibilities.