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Fetal Diagn Ther. 2009;26(3):121-6. doi: 10.1159/000238122. Epub 2009 Sep 11.

Pregnancy outcome in maternal Crigler-Najjar syndrome type II: a case report and systematic review of the literature.

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  • 1Department of Obstetrics and Gynaecology, Johannes Gutenberg University of Mainz, Mainz, Germany.



To report a case of maternal Crigler-Najjar syndrome (CNS) type II in pregnancy, systematically review the literature for similar case reports, and to evaluate whether pregnancy is safe in patients with the disease. Data sources included the PubMed and up to date databases.


A 37-year-old mother with CNS type II was treated with phenobarbital during her pregnancy and her bilirubin levels were monitored. Her newborn had mild direct hyperbilirubinemia, did not require any treatment and his postnatal follow-up showed normal growth and development as well as normal hearing.


CNS type II is rare, and only a few pregnancies with this condition have been reported. Maternal treatment with phenobarbital lowers the unconjugated bilirubin and avoids fetal and newborn sequelae.

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