妊娠期Wilson病的孕产妇和围产期结局:三级保健中心的5年经验

Q4 Medicine

Journal of SAFOG Pub Date : 2017-01-01 DOI:10.5005/JP-JOURNALS-10006-1521

K. Vishnupriya, C. N. Sheela, Mahalakshmi Thayumanasundaram

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引用次数: 1

摘要

Wilson病是一种非常罕见的遗传性常染色体隐性遗传病，发病率为1 / 30,000活产婴儿，与铜代谢受损导致胆道排泄减少和铜在肝脏和大脑中的积累有关。患者可能无症状，也可能伴有暴发性肝病或神经精神疾病。未经治疗的肝豆状核变性与不孕症/低生育能力/复发性妊娠丢失有关。目的:探讨妊娠期肝豆状核病的母婴结局。材料与方法:回顾性观察性研究于2010年11月至2015年11月在印度班加罗尔圣约翰医学院附属医院妇产科进行。在研究期间确定了5例Wilson病患者。这五名接受常规治疗的妇女的妊娠结局都很好。结论:肝豆状核变性患者接受常规治疗，无症状且妊娠正常，妊娠结局良好。他们需要在更高的中心进行定期的监测和积极的管理，包括胃肠病学家、产科医生、神经科医生和重症监护医生。

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Maternal and Perinatal Outcome of Wilson Disease in Pregnancy: A 5-year Experience at a Tertiary Care Center

318 ABSTRACT Introduction: Wilson disease is a very rare inherited autosomal recessive disorder, with an incidence of 1 in 30,000 live births, associated with impaired copper metabolism leading to decreased biliary excretion and accumulation of copper in the liver and brain. Patients may be asymptomatic or might present with fulminant liver disease or neuropsychiatric illness. Untreated Wilson disease is related to infertility/subfertility/ recurrent pregnancy loss. Aim: To study the maternal and perinatal outcomes of Wilson disease in pregnancy. Materials and methods: It is a retrospective observational study carried out in the Department of Obstetrics and Gynecology, St. John’s Medical College Hospital, Bengaluru, India, between November 2010 and November 2015. Five patients with Wilson disease were identified during the study period. Pregnancy outcome was good in all these five women who were on regular treatment. Conclusion: Patients with Wilson disease who receive regular treatment and who remain asymptomatic and conceive normally have favorable pregnancy outcomes. They merit regular surveillance and active management in higher centers with multidisciplinary approach involving gastroenterologists, obstetricians, neurologists, and intensivists.

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来源期刊

Journal of SAFOG Medicine-Obstetrics and Gynecology

CiteScore

0.40

自引率

0.00%

发文量

135

期刊介绍： Journal of South Asian Federation of Obstetrics and Gynaecology is the aim of this journal to provide platform for members to have access to scientific and peer-reviewed clinically oriented guidelines for practice and professional updating of subject of obstetrics and gynecology. The scope of South Asian Federation of Obstetrics and Gynaecology (SAFOG) journal is to cover the broad subject of obstetrics and gynecology and give out articles, information, and practice guidelines. The journal has fixed format of review articles, presentations as original studies by members and readers, operative skill reviews, case reports, and midwifery peer-reviewed practice guidelines. In addition, call upon experts and clinical authorities among member countries of SAFOG region to give out editorials on policy formulating topics such as maternal mortality, abortions, contraceptives, reproductive health preservation of women, etc. As well as to tabulate reproductive health country specific data to create awareness regarding the enormity of maternal and neonatal problems. To give out reader specific information for forthcoming professional events in the region. It remains a platform for better communication and exchange of reproductive indices among member countries. Also to amalgamate with world bodies, recommendations for practice guidelines for members. Brief of the journal: The SAFOG journal is the official organ and publication of South Asia Federation of Obstetrics and Gynecology. This is a scientific and peer-reviewed platform of exchange of professional activities, clinical practice guidelines, operative skill updates, and nursing midwifery practices among member countries.