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威尔逊氏病患者的妊娠情况

Pregnancy in Wilson's disease.

作者信息

Walshe J M

出版信息

Q J Med. 1977 Jan;46(181):73-83.

PMID:866569
Abstract

The effect of pregnancy has been studied in 10 mothers with Wilson's disease. Three were presymptomatic but had the typical biochemical lesion, two of these were receiving penicillamine treatment at the time of conception, the third had yet to be diagnosed. The remaining seven mothers had had symptoms of Wilson's disease and had been receiving treatment for periods ranging from two and a half to 19 years. These mothers had 15 pregnancies between them, 13 went to full term but two ended prematurely at 26 and 30 weeks. In only one did pregnancy have an unfavourable effect on the Wilson's disease; this mother had been on penicillamine for only two and a half years in a suboptimal dose because of drug induced thrombocytopoenia. In addition she had extensive oesophageal varices and pregnancy was complicated by toxaemia. The other nine patients remained well and two had three pregnancies each. On six occasions penicillamine was taken throughout pregnancy, but in seven it was discontinued from the sixth to the twelfth week. All 15 babies were normal but one died of extreme prematurity (26 weeks gestation). Pregnancy does not appear to be contraindicated in well treated Wilson's disease and penicillamine does not seem to pose an undue risk to the foetus.

摘要

对10位患有威尔逊氏病的母亲的妊娠影响进行了研究。其中3位处于症状前期,但有典型的生化损伤,其中2位在受孕时正在接受青霉胺治疗,第3位尚未被诊断。其余7位母亲有威尔逊氏病症状,接受治疗的时间从两年半到19年不等。这些母亲共有15次妊娠,13次足月分娩,但有2次分别在26周和30周时早产。只有1例妊娠对威尔逊氏病产生了不利影响;这位母亲因药物性血小板减少症,仅以次优剂量服用青霉胺两年半。此外,她有广泛的食管静脉曲张,妊娠并发了毒血症。其他9例患者情况良好,其中2位各有3次妊娠。有6次整个孕期都服用青霉胺,但有7次在孕6至12周时停药。所有15名婴儿均正常,但有1例因极度早产(妊娠26周)死亡。对于经过良好治疗的威尔逊氏病患者,妊娠似乎并非禁忌,而且青霉胺似乎不会对胎儿构成不当风险。

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