PEDBASE.org - The Pediatric Database - Detailed information of LUCEY-DRISCOLL SYNDROME

LUCEY-DRISCOLL SYNDROME

DEFINITION:

A benign disorder of bilirubin metabolism characterized by transient unconjugated hyperbilirubinemia and jaundice in the first week of life.

EPIDEMIOLOGY:

incidence: rare (about 24 cases reported)
age of onset:
- newborn (first 4 days of life)
risk factors:
- familial or sporadic
- M = F

PATHOGENESIS:

1. Background

first reported by Lucey et al., Am. J. Dis. Child. 100:787 (1960)
it is thought that an inhibitor of UDP-glucouronosyltransferase activity is present in the mother's serum -> transient unconjugated hyperbilirubinemia as the inhibitor is cleared

CLINICAL FEATURES:

1. Gastrointestinal Manifestations

transient jaundice in the first week of life
kernicterus may occur

INVESTIGATIONS:

1. Serum

unconjugated hyperbilirubinemia

MANAGEMENT:

1. For Hyperbilirubinemia

phototherapy
exchange transfusion
phenobarbital to induce UDP-glucouronosyltransferase activity

Pediatric Database - LUCEY-DRISCOLL SYNDROME

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