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[先天性多发性关节挛缩症伴相关异常。一例早产三胞胎致命病程的病例报告]

[Arthrogryposis multiplex congenita with associated abnormalities. Case report of a fatal course in a premature triplet].

作者信息

Cremer R, Künzer W

机构信息

Kinderkrankenhaus der Stadt Köln.

出版信息

Monatsschr Kinderheilkd. 1988 Aug;136(8):464-6.

PMID:3221896
Abstract

A male premature triplet with healthy siblings was born with characteristic symptoms of a Rossi syndrome: pterygiums at neck, axillae, elbows and in a moderate way at hips and knees with inhibition of extension of the affected joints, dislocation of the right hip, club-feet, cutis laxa et hyperelastica, facial dysmorphism, double pelvis renalis with ureter duplex on the right side, kryptorchidism, hypoplastic genital. The boy died after 38 days because of a haemorrhage of the lungs. An intrauterine immobilisation is discussed as cause of the syndrome.

摘要

一名男童为早产三胞胎之一,其兄弟姐妹均健康。该男童出生时具有罗西综合征的典型症状:颈部、腋窝、肘部有翼状胬肉,髋部和膝部有中度翼状胬肉,受累关节伸展受限,右髋关节脱位,马蹄内翻足,皮肤松弛且弹性过强,面部畸形,双侧肾盂,右侧输尿管重复,隐睾,生殖器发育不全。该男童在38天后因肺部出血死亡。文中讨论了子宫内固定作为该综合征病因的可能性。

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