Cervantes C D, Lifshitz F, Levenbrown J
Department of Pediatrics, North Shore University Hospital, Manhasset, New York.
Pediatr Radiol. 1988;18(3):210-4. doi: 10.1007/BF02390397.
Fifth metacarpal bone shortening (brachymetacarpia V) was recently described to be highly prevalent in children with familial short stature (FSS). To characterize the hand bones of FSS patients with and without brachymetacarpia V, the left hand bone age radiographs of 26 FSS children were reviewed. In 16/19 patients with clinical brachymetacarpia V radiographs revealed fifth metacarpal bone shortening with a gap of 2 mm or more between the distal end of the fifth metacarpal bone and a tangential line connecting the distal ends of the third and fourth metacarpal bones. Only one of 7 patients without clinical brachymetacarpia V had a gap of 2 mm. Radiologic anthropometry revealed that FSS patients with clinically shortened fifth metacarpal bone frequently had shortened first metacarpal bones, second and third proximal phalanges, and fifth distal phalanx as well. FSS patients without clinical fifth metacarpal bone shortening had shortened third and fourth metacarpal bones, fifth proximal phalanx, and fifth distal phalanx. Fifth metacarpal bone shortening was only detected clinically if the fourth metacarpal bone was not short as well. Reduction in height correlated more with reduction in metacarpal bone length than with that of the other hand bones. These peculiar tubular bone alterations commonly seen in FSS suggest a disturbance in endochondral ossification, the process primarily involved in tubular bone elongation.
第五掌骨缩短（短掌骨V）最近被描述为在家族性矮小症（FSS）儿童中高度普遍。为了描述有和没有短掌骨V的FSS患者的手部骨骼特征，回顾了26名FSS儿童的左手骨龄X光片。在16/19例临床诊断为短掌骨V的患者中，X光片显示第五掌骨缩短，第五掌骨远端与连接第三和第四掌骨远端的切线之间有2毫米或更大的间隙。7例无临床短掌骨V的患者中只有1例间隙为2毫米。放射人类学测量显示，临床第五掌骨缩短的FSS患者通常也有第一掌骨、第二和第三近端指骨以及第五远端指骨缩短。无临床第五掌骨缩短的FSS患者有第三和第四掌骨、第五近端指骨以及第五远端指骨缩短。只有在第四掌骨也不短时，临床上才能检测到第五掌骨缩短。身高降低与掌骨长度降低的相关性比与其他手部骨骼的相关性更大。这些在FSS中常见的特殊管状骨改变提示软骨内成骨过程受到干扰，而软骨内成骨是主要参与管状骨伸长的过程。
