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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Published inWorld journal of hepatology Vol. 8; no. 11; pp. 530 - 532
Main Authors Chaubal, Alisha Nitin, Patel, Ruchir, Choksi, Dhaval, Shah, Kaivan, Ingle, Meghraj, Sawant, Prabha
Format Journal Article
LanguageEnglish
Published United States Baishideng Publishing Group Inc 18.04.2016
Subjects
2;Phenobarbitone;Folic
acid;Pregnancy;Kernicterus
Case Report
Crigler
Najjar
type
Crigler Najjar type 2
Pregnancy
Kernicterus
Folic acid
Phenobarbitone
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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.
Bibliography:Alisha Nitin Chaubal;Ruchir Patel;Dhaval Choksi;Kaivan Shah;Meghraj Ingle;Prabha Sawant;Department of Gastroenterology,LTMG Hospital
Telephone: +91-022-24063088 Fax: +91-022-24044154
Author contributions: Chaubal AN wrote the case report; Chaubal AN, Patel R, Choksi D, Shah K, Ingle M and Sawant P managed the case.
Correspondence to: Alisha Nitin Chaubal, Registrar, Department of Gastroenterology, LTMGH Hospital, Dr. Babasaheb Ambedkar Road, Sion West, Mumbai 400022, India. alishachaubal@gmail.com
ISSN:1948-5182
1948-5182
DOI:10.4254/wjh.v8.i11.530