儿童原发性生长激素缺乏症远期随访：附80例临床分析  

Long-term follow up of childhood-onset primary growth hormone deficiency ： clinical analysis of 80 cases

作　　者：常国营[1] 董治亚[1] 王伟[1] 陈凤生[1] 倪继红[1] 孙文鑫[1] 王秀民[1] 肖园[1] 陆文丽[1] 王德芬[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院儿内科,200025

出　　处：《中华内分泌代谢杂志》2010年第7期560-563,共4页Chinese Journal of Endocrinology and Metabolism

摘　　要：目的 观察原发性儿童生长激素缺乏症（CO-GHD）过渡至成人期终身高（FAH）、脂代谢变化、性发育及生活质量等问题,再评估进入成年期后生长激素（GH）-胰岛素样生长因子I（IGF-I）轴功能.探讨不同类型GHD各时期的有效管理.方法 原发性CO-GHD随访至成人期80例,男62例,年龄均≥18岁;女18例,年龄均≥16岁.其中单纯性GHD（IGHD）22例、多垂体激素缺乏（MPHD）58例.随访身高、体重、血压、生长速率（GV）、性发育及婚育状况;空腹血脂、血糖、胰岛素、IGF-I及胰岛素样生长因子结合蛋白3（IGFBP3）;骨龄、B超（肝、盆腔）、学历职业、既往rhGH及其他激素治疗等.结果 rhGH治疗组较未治组FAH有明显改善;两组血脂、血糖和胰岛素水平无统计学差异,血脂异常检出率分别为39.0%、47.4%;脂肪肝检出率26.8%、31.6%,均无统计学差异（P〉0.05）,尚无代谢综合征发现.IGHD与MPHD患者IGF-I SDS分别为-1.43＋0.31、-3.01±0.66,IGFBP3 SDS为-2.10±0.33、-3.17±0.19,差异均有统计学意义（P〈0.05）.IGHD患者性发育正常,MPHD性功能低下者占79.7%,婚育状况较IGHD者差.结论 CO-GHD经rhGH治疗可改善FAH;转换期后再评估GH-IGF轴是必要的;成年后有血脂代谢异常的风险;IGHD育龄妇女可正常生育,MPHD虽存在垂体低促性腺激素,但亦有程度差异.Objectives To evaluate final adult height（FAH）, lipid profile, sexual development, and quality of life in individuals with childhood-onset growth hormone deficiency （CO-GHD） during the transition from childhood to adulthood, to reassess the function of GH-IGF-I axis, and to explore effective managements for different types of GHD in each period. Methods Totally 80 CO-GHD patients were divided into 2 groups; 22 patients with isolated growth hormone deficiency （ IGHD） and 58 patients with multiple pituitary hormone deficiencies （MPHD）; 62 male （age ≥18 years） and 18 female （ age ≥ 16 years） patients. The clinical and biochemical parameters, education and occupation, rhGH, and other hormones therapy in the past were followed up. Results rhGH replacement improved FAH of patients with GHD. The incidences of either hyperlipidemia （39.0% , 47.4%） or fatty liver disease （26.8%, 31.6%） showed no statistically significant changes between 2 groups with and without rhGH replacement. Mean value of IGF-I SDS was significantly higher in IGHD group than that in MPHD group （-1.43±0. 31,-3. 01 ±0. 66） ,and also IGFBP3（-2. 10±0. 33,-3. 17±0. 19,all P〈 0.05 ）. Patients with IGHD had normal sexual development, but the incidence of sexual dysfunction accounted for 79.7% in MPHD group. Conclusions rhGH improves FAH of individuals with CO-GHD. Patients with CO-GHD should be followed during the transition period; GHD patients carry a high risk of metabolic abnormalities in the adulthood; IGHD female can give birth to offsprings; patients with MPHD have gonadotrophin deficiency of varying degrees.

关 键 词：儿童生长激素缺乏症 原发性 成人终身高 脂代谢 性发育 

分 类 号：R725.8[医药卫生—儿科]