Chaubal AN, Patel R, Choksi D, Shah K, Ingle M, Sawant P. Management of pregnancy in Crigler Najjar syndrome type 2. World J Hepatol 2016; 8(11): 530-532 [PMID: 27099654 DOI: 10.4254/wjh.v8.i11.530]
Corresponding Author of This Article
Alisha Nitin Chaubal, Registrar, Department of Gastroenterology, LTMGH Hospital, Dr. Babasaheb Ambedkar Road, Sion West, Mumbai 400022, India. alishachaubal@gmail.com
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant, Department of Gastroenterology, LTMG Hospital, Mumbai 400022, India
Author contributions: Chaubal AN wrote the case report; Chaubal AN, Patel R, Choksi D, Shah K, Ingle M and Sawant P managed the case.
Institutional review board statement: The institutional review board of LTMG hospital have reviewed and accepted the case report.
Informed consent statement: I am aware that my clinical problem is being reported without revealing my identity and I have no objections to the same. I have been explained in detail the procedure for the same and will not hold anyone responsible for the outcome.
Conflict-of-interest statement: The authors do not hold any conflict of interest with reviewers.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Alisha Nitin Chaubal, Registrar, Department of Gastroenterology, LTMGH Hospital, Dr. Babasaheb Ambedkar Road, Sion West, Mumbai 400022, India. alishachaubal@gmail.com
Telephone: +91-022-24063088 Fax: +91-022-24044154
Received: October 18, 2015 Peer-review started: November 12, 2015 First decision: January 4, 2016 Revised: February 22, 2016 Accepted: March 9, 2016 Article in press: March 14, 2016 Published online: April 18, 2016 Processing time: 151 Days and 9.1 Hours
Abstract
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.
Core tip: Crigler Najjar syndrome type 2 is a rare disorder causing indirect hyperbilirubinemia. In pregnancy placental crossing of unconjugated bilirubin can cause high bilirubin levels in the fetus with low Uridine Di Phospho Glucoronosyl Transferase activity causing permanent neurological impairment in the newborn. Hence timely diagnosis and treatment with low dose phenobarbitone is required.
