Management of pregnancy in Crigler Najjar syndrome type 2

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 th...

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Bibliographic Details
Published in:World journal of hepatology 2016-04, Vol.8 (11), p.530-532
Main Authors: Chaubal, Alisha Nitin, Patel, Ruchir, Choksi, Dhaval, Shah, Kaivan, Ingle, Meghraj, Sawant, Prabha
Format: Article
Language:English
Subjects:
2
Phenobarbitone
Folic
acid
Pregnancy
Kernicterus
Case Report
Crigler
Najjar
type
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Summary: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 neuro-logical damage in the newborn.Patient was carefully monitored during pregnancy and treatment with pheno-barbitone in low doses was adjusted such that the serum bilirubin levels were below 10 mg/d L.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.
ISSN:1948-5182
1948-5182
DOI:10.4254/wjh.v8.i11.530