RELATIONSHIPS BETWEEN CLINICAL AND HISTOLOGICAL PROFILES OF NONFAMILIAL IDIOPATHIC NEONATAL HEPATITIS

Idiopathic neonatal hepatitis (INH) is a syndrome characterized clinic ally and histologically but there is little information concerning the relationship between the clinical features and histological findings. In the present study, sixty-two patients clinically diagnosed as non- familial INH were histologically classified into four groups according to a provisional definition based on predominant lesions and examinat ion of their clinical features. Patients of cholestasis (n = 23) and g iant cell hepatitis (GCH, n = 21) were most frequent (37% and 33%, res pectively), and patients of fatty liver (n = 10) and hepatitis (It = 8 ) were less common (16% and 13%). The GCH group showed a dominance of male, low birthweight, older and breast-fed babies. The cholestasis gr oup demonstrated a dominance of male, low birthweight, younger and bot tle-fed babies. The hepatitis group had the highest frequencies of hig h-grade hepatomegaly and splenomegaly. Fifty six cases completely reco vered. Two died of hepatic failure in early infancy and four had chron ic liver diseases at the age of 12 months. The fatty liver group had t he worst outcome. Histological features in non-familial INH were varia ble and typical giant cell hepatitis was seen in only one-third of pat ients. Characteristic clinical features in each histologically classif ied group may suggest heterogenous etiologies underlying non-familial INH.