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Recommendations for Pregnancies in Patients with Crigler-Najjar Syndrome

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JIMD Reports - Case and Research Reports, 2012/4

Part of the book series: JIMD Reports ((JIMD,volume 7))

Abstract

During pregnancy, the developing foetus in mothers with Crigler-Najjar type 1 and 2 is exposed to raised levels of unconjugated bilirubin, with the risk of neurotoxicity. We describe two pregnancies in a patient with Crigler-Najjar type 2, who was carefully monitored prior to and during pregnancy and phototherapy adjusted to maintain serum bilirubin levels below 200 μmol/l and the bilirubin/albumin molar ratio below 50%. Both pregnancies resulted in normal delivery of healthy infants who had normal neurological development. A review of all reported pregnancies in Crigler-Najjar patients and a set of recommendations are presented.

Competing interests: None declared

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References

  • Arora N, Choudhary S (2009) Pregnancy with Crigler-Najjar syndrome type II. J Obstet Gynaecol 29:242–244

    Article  PubMed  CAS  Google Scholar 

  • Cahill DJ, McCarthy CF (1989) Pregnancy and the Crigler-Najjar syndrome. J Obstet Gynecol 9:213

    Article  Google Scholar 

  • Cotton BD, Brock BJ, Schifrin BS (1981) Cirrhosis and fetal hyperbilirubinemia. Obstet Gynecol 57(6 suppl):25–27S

    Google Scholar 

  • Davis DR, Yeary RA (1979) Impaired fertility in the jaundiced female (Gunn) rat. Lab Anim Sci 29:739–743

    PubMed  CAS  Google Scholar 

  • Davis DR, Yeary RA, Lee K (1983) Improved embryonic survival in the jaundiced female rat fed activated charcoal. Pediatr Pharmacol 3:79–85

    CAS  Google Scholar 

  • Furhoff AK (1974) Fate of children born to women with jaundice in pregnancy. Arch Gynäk 217:165–172

    Article  CAS  Google Scholar 

  • Gajdos V, Petit F, Trioche P et al (2006) Successful pregnancy in a Crigler-Najjar type I patient treated by phototherapy and semimonthly albumin infusions. Gastroenterology 131:921–924

    Article  PubMed  CAS  Google Scholar 

  • Hannam S, Moriaty P, O'Reilly H et al (2009) Normal neurological outcome in two infants treated with exchange transfusions born to mothers with Crigler-Najjar Type 1 disorder. Eur J Pediatr 168:427–429

    Article  PubMed  Google Scholar 

  • Holstein A, Plaschke A, Lohse P, Egberts EH (2005) Successful photo-and phenobarbital therapy during pregnancy in a woman with Crigler-Najjar syndrome type II. Scand J Gastroenterol 40:1124–1126

    PubMed  Google Scholar 

  • Holstein A, Bryan CS (2011) Three consecutive pregnancies in a woman with Crigler-Najjar syndrome type II with good maternal and neonatal outcomes. Dig Liver Dis 43:170

    Article  PubMed  Google Scholar 

  • Ito T, Katagiri C, Ikeno S, Takahashi H, Nagata N, Terakawa N (2001) Phenobarbital following phototherapy for Crigler-Najjar syndrome type II with good fetal outcome: a case report. J Obstet Gynaecol Res 27:33–35

    Article  PubMed  CAS  Google Scholar 

  • Macias RI, Marin JJ, Serrano MA (2009) Excretion of biliary compounds during intrauterine life. World J Gastroenterol 15:817–828

    Article  PubMed  CAS  Google Scholar 

  • McDonagh AF (2007) Movement of bilirubin and bilirubin conjugates across the placenta. Pediatrics 119:1032–1033

    Article  PubMed  Google Scholar 

  • McDonagh AF (2010) Controversies in bilirubin biochemistry and their clinical relevance. Semin Fetal Neonatal Med 15:141–147

    Article  PubMed  Google Scholar 

  • Mreihil K, McDonagh AF, Nakstad B, Hansen TW (2010) Early isomerization of bilirubin in phototherapy of neonatal jaundice. Pediatr Res 67:656–659

    Article  PubMed  CAS  Google Scholar 

  • Passuello V, Puhl AG, Wirth S et al (2009) Pregnancy outcome in maternal Crigler-Najjar syndrome type II: a case report and systematic review of the literature. Fetal Diagn Ther 26:121–126

    Article  PubMed  CAS  Google Scholar 

  • Robertson A, Karp W, Brodersen R (1991) Bilirubin displacing effect of drugs used in neonatology. Acta Paediatr Scand 80:1119–1127

    Article  PubMed  CAS  Google Scholar 

  • Roszkowski I, Pizarek-Miedzinska D (1968) Jaundice in pregnancy. II. Clinical course of pregnancy and delivery and condition of neonate. Am J Obstet Gynecol 101:500–503

    PubMed  CAS  Google Scholar 

  • Saxena P, Arora R, Minocha B (2005) Crigler-Najjar syndrome with pregnancy. J Obstet Gynecol India 55:270–271

    Google Scholar 

  • Smith JF Jr, Baker JM (1994) Crigler-Najjar disease in pregnancy. Obstet Gynecol 84:670–672

    PubMed  Google Scholar 

  • Strauss KA, Robinson DI, Vreman HK, Puffenberger EG, Hart G, Morton DH (2006) Management of hyperbilirubinemia and prevention of kernicterus in 20 patients with Crigler-Najjar disease. Eur J Pediatr 165(5):306–319

    Article  PubMed  Google Scholar 

  • Taylor WG, Walkinshaw SA, Farquharson RG, Fisken RA, Gilmore IT (1991) Pregnancy in Crigler-Najjar syndrome. Br J Obstet Gynaecol 98:1290–1291

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Janneke G. Langendonk .

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Communicated by: Robin Lachmann

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© 2012 SSIEM and Springer-Verlag Berlin Heidelberg

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Wilson, J.H.P., Sinaasappel, M., Lotgering, F.K., Langendonk, J.G. (2012). Recommendations for Pregnancies in Patients with Crigler-Najjar Syndrome. In: Brown, G., Morava, E., Peters, V., Gibson, K., Zschocke, J. (eds) JIMD Reports - Case and Research Reports, 2012/4. JIMD Reports, vol 7. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2012_142

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  • DOI: https://doi.org/10.1007/8904_2012_142

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-32441-3

  • Online ISBN: 978-3-642-32442-0

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