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脊髓脊膜膨出患者的生长与青春期发育:一项回顾性分析。

Growth and pubertal development in patients with meningomyelocele: a retrospective analysis.

作者信息

Trollmann R, Dörr H G, Strehl E, Katalinic A, Beyer R, Wenzel D

机构信息

University Hospital for Children and Adolescents, Erlangen, Germany.

出版信息

Acta Paediatr. 1996 Jan;85(1):76-80. doi: 10.1111/j.1651-2227.1996.tb13894.x.

Abstract

Our retrospective analysis of growth and pubertal development includes 109 children and adults with meningomyelocele (MMC) (52 M, 57 F) aged 3.2-21.0 years (median 8.9 years). Anthropometric data, growth-retarding factors and data on pubertal development were analysed in comparison to the normal population using standards from Prader et al. (1). The results (mean +/- SD) were as follows. Fifty patients (46.8%) had short stature (height SDS for chronological age (SDS CA) < -2). The supine length was influenced by the level of the lesion (height SDS CA: > or = L2 -3.13 +/- 1.62, < or = S2 -0.46 +/- 1.27), ambulatory status, skeletal deformities and pubertal stage. The mean adult height (n = 15, age 16.1-21.0 years) measured 141.3 cm for women (height SDS CA -3.83 +/- 1.79) and 159.2 cm for men (height SDS CA -2.27 +/- 1.81). In 82.6% of the subjects (n = 90), arm spans were within the normal range. Reduced arm spans (SDS < -2) as found in 19 patients (17.4%) with short stature (mean height SDS CA -3.29 +/- 1.29) may be caused by factors other than neurological lesions and skeletal deformities, and require further endocrinological studies. Out of 27 pubertal patients, central precocious puberty was diagnosed in five girls. The stages of puberty in MMC girls developed earlier than expected for the age-related group.

摘要

我们对生长和青春期发育的回顾性分析纳入了109例患有脊髓脊膜膨出(MMC)的儿童和成人(52例男性,57例女性),年龄在3.2至21.0岁之间(中位数8.9岁)。使用普拉德等人(1)的标准,将人体测量数据、生长迟缓因素和青春期发育数据与正常人群进行比较分析。结果(均值±标准差)如下。50例患者(46.8%)身材矮小(按实际年龄计算的身高标准差评分(SDS CA)<-2)。仰卧长度受病变水平(身高SDS CA:≥L2为-3.13±1.62,≤S2为-0.46±1.27)、行走状态、骨骼畸形和青春期阶段的影响。成年女性(n = 15,年龄16.1至21.0岁)的平均身高为141.3 cm(身高SDS CA -3.83±1.79),成年男性为159.2 cm(身高SDS CA -2.27±1.81)。82.6%的受试者(n = 90)臂展在正常范围内。19例身材矮小患者(17.4%)(平均身高SDS CA -3.29±1.29)出现臂展缩短(SDS<-2),可能是由神经病变和骨骼畸形以外的因素引起的,需要进一步进行内分泌学研究。在27例青春期患者中,5名女孩被诊断为中枢性性早熟。MMC女孩的青春期阶段比同龄组预期的要早。

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