42例儿童期起病的成人生长激素缺乏症的临床分析

Clinical analysis of 42 adults with childhood-onset adult growth hormone deficiency

目的探讨儿童期起病的成人生长激素缺乏症(Co-AGHD)的病因分布、临床特点及治疗情况。方法回顾性分析2012年7月至2014年12月北京协和医院内分泌矮小门诊定期随诊的42例Co-AGHD患者出生情况、病因、临床特点、影像检查及治疗情况。根据是否接受重组人生长激素(rh GH)替代治疗,分为治疗组(n=12例)和未治疗组(n=30例),评价治疗效果。结果男性患者的比例高于女性。先天性病因39例,其中垂体发育不良20例,合并空泡蝶鞍3例,部分空泡蝶鞍1例,其他病因不详。获得性病因3例,均为颅咽管瘤术后患者。两组rhGH替代治疗在改善肝功能异常和血脂异常方面无明显差异(P>0.05)。结论 Co-AGHD病因多是先天性,其中大多患者出生史异常并伴有垂体发育不良。AGHD易合并多种代谢相关疾病。rh GH替代治疗在改善肝功能异常和血脂异常方面的有效性仍需进一步证实。

Objective To investigate the causes,clinical features and therapeutic effects of childhood-onset adult growth hormone deficiency（ Co-AGHD）. Methods Clinical data of 42 Co-AGHD patients（ 36 males and 6 females）treated in Nanism Clinic of Endocrinology Department of Peking Union Medical College Hospital during July 2012 and Dec. 2014 were retrospectively analyzed,including etiology,clinical features and therapeutic responses. According to whether they received recombinant human growth hormone（ rh GH） therapy,the patients were divided into treatment group（ n = 12） and control group（ n = 30）. The therapeutic effects were assessed. Results Among the 42 patients,the proportion of male was much higher than that of female. Congenital Co-AGHD was found in 39 cases： 20 with pituitary hypoplasia,3 with empty sella syndrome,1 with partially empty sella and the others with undefined causes. Acquired Co-AGHD was found in 3 cases which was induced by craniopharyngioma surgery. There was no significant difference between rh GH treatment group and control group in indexes of lipid and glucose（ P〉0. 05）. Conclusions Congenital etiology is the major cause of Co-AGHD,especially pituitary hypoplasia and empty sella syndrome. AGHD is linked to dyslipidemia,obesity and other metabolic diseases. Further studies and are needed to investigate the effects of rh GH replacement therapy on improvement of liver and lipid metabolism.