Mims R B
J Natl Med Assoc. 1978 Feb;70(2):125-8.
Endocrine function was evaluated in a 38-year-old man who had patchy asymmetrical acral hypertrophy and giantism. The history and clinical manifestations were consistent with previously described cases of the Klippel-Trenaunay syndrome. Pituitary and peripheral hormone concentrations were generally elevated, but his endocrine status appeared normal by clinical evaluation. Of particular interest were elevated growth hormone and somatomedin-A concentrations and responses to provocative tests. These findings suggest that this patient had abnormal cell receptor pathophysiology as the cause of the asymmetrical acral hypertrophy and giantism, which often occurred in the same anatomical site.
对一名38岁患有斑片状不对称性肢端肥大和巨人症的男性进行了内分泌功能评估。病史和临床表现与先前描述的Klippel-Trenaunay综合征病例一致。垂体和外周激素浓度普遍升高，但通过临床评估其内分泌状态似乎正常。特别令人感兴趣的是生长激素和生长调节素A浓度升高以及对激发试验的反应。这些发现表明，该患者存在异常的细胞受体病理生理学，这是不对称性肢端肥大和巨人症的原因，且这些症状常发生在同一解剖部位。
