Open AccessManagement of pregnancy in Crigler Najjar syndrome type 2
Author(s) -
Alisha Chaubal,
Ruchir Patel,
Dhaval Choksi,
K. L. Shah,
Meghraj Ingle,
Prabha Sawant
Publication year - 2016
Publication title -
world journal of hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.913
H-Index - 55
ISSN - 1948-5182
DOI - 10.4254/wjh.v8.i11.530
Subject(s) - medicine , pregnancy , fetus , glucuronosyltransferase , bilirubin , kernicterus , obstetrics , pediatrics , enzyme , microsome , biochemistry , chemistry , genetics , biology
Crigler Najjar syndrome is associated with indirect hyperbilirubinemia due to a deficiency of enzyme Uridine Di Phospho Glucoronosyl Transferase (UDPGT). Presented here is a case of a female in the first trimester of pregnancy, who was diagnosed to have type 2 Crigler Najjar syndrome. We also discuss the management of this rare disease especially in pregnancy. Unconjugated bilirubin can cross the placental barrier causing neurological damage in the newborn. Patient was carefully monitored during pregnancy and treatment with phenobarbitone in low doses was adjusted such that the serum bilirubin levels were below 10 mg/dL. Crigler Najjar syndrome being rare needs to be diagnosed early in pregnancy to avoid adverse fetal outcomes. Phenobarbitone being an inducer of enzyme UDPGT is used as the first line of treatment and is not teratogenic in the low doses used. Treatment protocol followed was on the basis of previous reported cases and successful perinatal outcome was achieved.