CRIGLER-NAJJAR SYNDROME

Course overview

CRIGLER-NAJJAR SYNDROME IS AN AUTOSOMAL RECESSIVE INHERITED DISORDER THAT LEADS TO CONGENITAL NON-HEMOLYTIC JAUNDICE. CRIGLER-NAJJAR SYNDROME IS CAUSED BY AN ABSENCE OR PROFOUNDLY DECREASED LEVEL OF THE ENZYME UDP-GLUCURONOSYLTRANSFERASE DUE TO A GENETIC DEFECT IN THE UGT1A1 GENE. SEVERE HYPERBILIRUBINEMIA HAS THE POTENTIAL TO CAUSE IRREVERSIBLE BRAIN DAMAGE. HENCE, PROMPT DIAGNOSIS AND TREATMENT IS OF UTMOST IMPORTANCE. THIS ACTIVITY REVIEWS THE EVALUATION AND MANAGEMENT OF CRIGLER-NAJJAR SYNDROME AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN THE CARE OF NEWBORNS WITH THIS CONDITION.