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一名儿童疑似拉森综合征:随访12年的病例

Presumed Larsen syndrome in a child: a case with a 12-year follow-up.

作者信息

Mei Haibo, He Rongguo, Liu Kun, Wu Jiangyan, Tang Jin, Yan An

机构信息

Department of Pediatric Orthopaedics, Hunan Children's Hospital, The Pediatric Academy of University of South China, Changsha City, China.

出版信息

J Pediatr Orthop B. 2015 May;24(3):268-73. doi: 10.1097/BPB.0000000000000126.

Abstract

Larsen syndrome (OMIM 150250) was first described in 1950 as an entity characterized by distinct facial features and dislocations of the multiple large joint, and cleft palate, hearing loss, and spinal abnormalities were occasionally observed. The prevalence of Larsen syndrome is estimated to be one in 100,000 live births . Management of multiple large-joint dislocations often proves difficult with a tendency toward recurrence, particularly if a patient has complete dislocation of the knee .We treated a boy with the clinical phenotype of Larsen syndrome using 10 orthopedic procedures, but failed to achieve a satisfactory outcome.The aim of this report is to review the surgical course and report results of surgical treatments for this patient with 12 years of follow-up.

摘要

拉森综合征(OMIM 150250)于1950年首次被描述为一种具有独特面部特征、多个大关节脱位的疾病,偶尔还会观察到腭裂、听力损失和脊柱异常。据估计,拉森综合征在活产婴儿中的患病率为十万分之一。多个大关节脱位的治疗往往很困难,有复发倾向,尤其是当患者膝关节完全脱位时。我们对一名具有拉森综合征临床表型的男孩进行了10次骨科手术治疗,但未取得满意效果。本报告的目的是回顾手术过程,并报告对该患者进行12年随访的手术治疗结果。

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