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软骨发育不全性侏儒的骨骼生长与发育

Skeletal growth and development of the achondroplastic dwarf.

作者信息

Nehme A M, Riseborough E J, Tredwell S J

出版信息

Clin Orthop Relat Res. 1976 May(116):8-23.

PMID:1277655
Abstract

Certain patterns in growth and development can be seen in the typical achondroplastic dwarf. Height is approximatley 5 standard deviations below average size for age through the age of 14. Sitting height is within normal limits at all ages. On the average the femur is 8 standard deviations and the tibia 7 standard deviations below normal size. Compared to normal, both femur and tibia exhibit approximately a 50 per cent inhibition in growth. A slight decrease in the deviance from the norm is observed for all parameters during growth until the time of the adolescent growth spurt when an increased deviance is seen. Related to normal children of the same sex and age, females are more severely affected than male achondroplastic dwarfs. The skeletal development of the hand is irregular, often having extra metacarpal epiphyses and a wide variance in skeletal maturation assessment between the carpals and the rest of the hand. The skeletal age is generally below chronological age until the adolescent growth spurt when a rapid maturation pattern occurs. Standard skeletal age assessment techniques cannot be applied to the achondroplastic individual.

摘要

典型的软骨发育不全性侏儒在生长发育方面存在某些特定模式。在14岁之前,身高大约比同年龄平均身高低5个标准差。各年龄段的坐高均在正常范围内。平均而言,股骨比正常尺寸低8个标准差,胫骨比正常尺寸低7个标准差。与正常情况相比,股骨和胫骨的生长均受到约50%的抑制。在生长过程中,直到青春期生长突增之前,所有参数与正常标准的偏差都有轻微下降,而在青春期生长突增时偏差会增大。与同龄同性别的正常儿童相比,女性软骨发育不全性侏儒受到的影响比男性更严重。手部骨骼发育不规则,常出现额外的掌骨骨骺,腕骨与手部其他部位的骨骼成熟度评估差异很大。骨骼年龄通常低于实际年龄,直到青春期生长突增时会出现快速成熟模式。标准的骨骼年龄评估技术不适用于软骨发育不全个体。

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