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进行性骨化性纤维发育不良患者的妊娠情况。

Pregnancy in fibrodysplasia ossificans progressiva.

作者信息

Muglu Javaid A, Garg Aditya, Pandiarajan T, Shore Eileen M, Kaplan Frederick S, Uchil Dhiraj, Dickson Malcolm J

机构信息

Department of Obstetrics & Gynaecology, Princess Alexandra Hospital , Harlow, Essex CM20 1QX , UK.

Department of Obstetrics & Gynaecology, Hope Hospital , Stott Lane, Salford M6 8HD , UK.

出版信息

Obstet Med. 2012 Mar;5(1):35-8. doi: 10.1258/om.2011.110042. Epub 2011 Dec 8.

Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare disabling genetic disorder characterized by progressive postnatal heterotopic ossification leading to cumulative disability. Heterotopic bone formation in FOP usually begins in early childhood following a series of painful, post-traumatic, inflammatory soft-tissue swellings known as flare-ups, which later undergo ossification resulting in the progressive immobilization of the chest wall, limbs and jaw by early adulthood. Pregnancy in FOP has occurred infrequently and reproductive decisions are a dilemma for an individual or couple with FOP. We present the clinical course, medical management and potential concerns of four cases of pregnancy in FOP.

摘要

进行性骨化性纤维发育不良(FOP)是一种罕见的致残性遗传病,其特征是出生后进行性异位骨化,导致累积性残疾。FOP中的异位骨形成通常始于儿童早期,在一系列称为发作的疼痛性、创伤后、炎症性软组织肿胀之后发生,这些肿胀随后会发生骨化,导致成年早期胸壁、四肢和颌骨逐渐固定。FOP患者怀孕的情况很少见,对于患有FOP的个人或夫妇来说,生育决定是一个两难的问题。我们介绍了4例FOP患者怀孕的临床过程、医疗管理及潜在问题。

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