Management of a pregnancy with Crigler-Najjar syndrome type 2: a case report

IF 0.6 Q3 EDUCATION & EDUCATIONAL RESEARCH Journal of Perinatal Education Pub Date : 2023-04-01 DOI:10.2399/prn.23.0311007
E. Çağlıyan, S. Özmen, Ezgi Bilicen, Aslı Akdöner, O. Yavuz, M. Soytürk
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Abstract

Objective: To report a case with Crigler-Najjar syndrome type 2 of elevated bilirubin levels who was treated with triple therapy. Case(s): Crigler-Najjar syndrome is a rare congenital disorder that causes non-obstructive non-hemolytic unconjugated jaundice. The syndrome is divided into two groups according to the severity and the clinical presentation of the disease. In these cases, there is an elevated risk of antenatal death or permanent neurological impairment of the fetus due to fetal kernicterus caused by excessively increased unconjugated bilirubin levels. Phototherapy, phenobarbital and plasmapheresis can be useful in reducing serum total bilirubin concentrations, thus adverse maternal and neonatal risks. Conclusion: At her 37 weeks of gestation, the patient delivered a healthy girl. No pathological neurological findings were found and the baby had normal growth with intact neurological development.
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2型克里格勒-纳贾尔综合征妊娠的处理:1例报告
目的:报告1例胆红素水平升高的Crigler-Najjar综合征2型患者采用三联疗法治疗。病例:Crigler-Najjar综合征是一种罕见的先天性疾病,引起非梗阻性非溶血性非共轭黄疸。根据病情的严重程度和临床表现可分为两组。在这些病例中,由于未结合胆红素水平过度升高而引起的胎儿核黄疸会增加产前死亡或胎儿永久性神经损伤的风险。光疗、苯巴比妥和血浆置换可有效降低血清总胆红素浓度,从而降低孕产妇和新生儿的不良风险。结论:患者在妊娠37周时产下一名健康的女婴。未发现病理神经学发现,婴儿生长正常,神经发育完整。
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来源期刊
Journal of Perinatal Education
Journal of Perinatal Education EDUCATION & EDUCATIONAL RESEARCH-
CiteScore
0.70
自引率
16.70%
发文量
51
期刊介绍: The Journal of Perinatal Education (JPE) is the leading peer-reviewed journal specifically for childbirth educators. Through evidence-based articles, the JPE advances the knowledge of aspiring and seasoned educators in any setting-independent or private practice, community, hospital, nursing or midwifery school-and informs educators and other health care professionals on research that will improve their practice and their efforts to support natural, safe, and healthy birth. The JPE also publishes features that provide practical resources and advice health care professionals can use to enhance the quality and effectiveness of their care or teaching to prepare expectant parents for birth. The journal''s content focuses on pregnancy, childbirth, the postpartum period, breastfeeding, neonatal care, early parenting, and young family development. In addition to childbirth educators, the JPE''s readers include nurses, midwives, physicians, and other professionals involved with perinatal education and maternal-child health care.
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