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特发性半身肥大的临床表现与自然病程:11例病例回顾

Manifestations and natural history of idiopathic hemihypertrophy: a review of eleven cases.

作者信息

Viljoen D, Pearn J, Beighton P

出版信息

Clin Genet. 1984 Aug;26(2):81-6. doi: 10.1111/j.1399-0004.1984.tb00797.x.

DOI:10.1111/j.1399-0004.1984.tb00797.x
PMID:6467671
Abstract

Idiopathic hemihypertrophy is a specific entity which is distinct from the numerous other causes of limb overgrowth. In our investigation of eleven new cases in South Africa and Australia, no genetic or other aetiological factors could be recognised. There were no differences in bone age between the normal and hypertrophied limbs and the pattern of growth through life showed that relative body proportions remained the same. Idiopathic hemihypertrophy is associated with mild mental retardation, genito-urinary anomalies and an oncogenic potential. Regular clinical surveillance for abdominal tumours is recommended.

摘要

特发性半身肥大是一种特定的病症,有别于导致肢体过度生长的众多其他病因。在我们对南非和澳大利亚的11例新病例进行的调查中,未发现遗传或其他病因因素。正常肢体和肥大肢体的骨龄没有差异,终生生长模式表明相对身体比例保持不变。特发性半身肥大与轻度智力发育迟缓、泌尿生殖系统异常以及致癌潜能有关。建议对腹部肿瘤进行定期临床监测。

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