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Case Reports
. 2023 Sep;16(3):184-186.
doi: 10.1177/1753495X221099443. Epub 2022 May 12.

Crigler-Najjar type II in pregnancy: A case report

Katherine Creeper  1 , Dorothy Graham  1   2
Affiliations
Case Reports

Crigler-Najjar type II in pregnancy: A case report

Katherine Creeper et al. Obstet Med. 2023 Sep.

Abstract

Crigler-Najjar is a rare, autosomal recessive disorder that results in mutations causing a complete absence (type I) or deficiency (type II) of the hepatic uridine diphospho-glucuronosyl transferase (UDPGT) enzyme. Both forms, however, result in unconjugated hyperbilirubinaemia which can lead to kernicterus and potentially death. Phenobarbitone can be used as an enzyme inducer in Type II to facilitate a reduction in total serum bilirubin. We report two consecutive pregnancies in a 29-year-old woman with Crigler-Najjar Type II syndrome. Phenobarbitone therapy was commenced in the first pregnancy at 16 weeks' gestation and was associated with favorable biochemical and clinical outcomes. There were no reports of long-term neonatal neurological sequelae. Tertiary center, multidisciplinary care is recommended for optimal pregnancy outcomes.

Keywords: Bilirubin; Crigler-Najjar; jaundice; phenobarbitone.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Total serum bilirubin by gestation.
See this image and copyright information in PMC

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