Lazar Liora, Dan Sivan, Phillip Moshe
Institute for Endocrinology and Diabetes, Schneider Children's Medical Center of Israel, Petah Tikva and Faculty of Medicine, Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Clin Endocrinol (Oxf). 2003 Jul;59(1):82-8. doi: 10.1046/j.1365-2265.2003.01805.x.
Growth without GH has been reported in patients with organic combined pituitary hormone deficiency (CPHD) after resection of craniopharyngiomas and hypothalamic tumours or in septo-optic dysplasia.
This study describes the growth pattern and final height of five children (four boys, one girl) with idiopathic CPHD (GH, TSH, ACTH, LH and FSH) who maintained normal growth despite persistent GH deficiency throughout the growth period.
Presenting findings were borderline small penis in two children diagnosed at ages 3 and 9 years, and absence of pubertal signs in three adolescents diagnosed at age 12.8-13.7 years. The latter three patients also exhibited acromegaloid features. The height of all patients was within the 10-25th percentiles, and weight at the 25-50th percentiles. Although they were moderately overweight, accelerated weight gain was not observed. Prepubertal growth rate was 4-5 cm/year. The pubertal growth period, starting after initiation of sex hormone therapy (chronological age 15.9-16.3 years and bone age 12.5-14.5 years) continued for 4-5.5 years. Total pubertal growth was 6-11.7 cm with reduced growth spurt. Final height, which was attained at an advanced age (19-22 years), was 170-179 cm in the boys and 164 cm in the girl, equal to or exceeding the target height range. Repeated hormonal evaluations revealed undetectable GH and IGF-I levels, and no evidence of hyperprolactinaemia or hyperinsulinism.
Final height attainment within or above target height range may occur in patients with idiopathic CPHD despite persistent GHD. As this was not mediated by GH, IGF-I, insulin or prolactin, some other growth factors probably played a growth-promoting role.
据报道,颅咽管瘤和下丘脑肿瘤切除术后的器质性联合垂体激素缺乏症(CPHD)患者或视隔发育不良患者在无生长激素(GH)的情况下仍可生长。
本研究描述了5例特发性CPHD(生长激素、促甲状腺激素、促肾上腺皮质激素、黄体生成素和促卵泡激素缺乏)儿童(4名男孩,1名女孩)的生长模式和最终身高,这些儿童在整个生长期间尽管持续存在生长激素缺乏,但仍保持正常生长。
两名分别在3岁和9岁时确诊的儿童表现为阴茎短小临界状态,三名在12.8 - 13.7岁时确诊的青少年无青春期体征。后三名患者还表现出肢端肥大样特征。所有患者的身高处于第10 - 25百分位数之间,体重处于第25 - 50百分位数之间。尽管他们有中度超重,但未观察到体重加速增加。青春期前生长速率为每年4 - 5厘米。青春期生长阶段始于性激素治疗开始后(实际年龄15.9 - 16.3岁,骨龄12.5 - 14.5岁),持续4 - 5.5年。青春期总生长量为6 - 11.7厘米,生长突增减少。最终身高在较高年龄(19 - 岁)达到,男孩为170 - 179厘米,女孩为164厘米,等于或超过目标身高范围。多次激素评估显示生长激素和胰岛素样生长因子 - I水平检测不到,且无高催乳素血症或高胰岛素血症的证据。
特发性CPHD患者尽管持续存在生长激素缺乏,但仍可能达到或超过目标身高范围的最终身高。由于这并非由生长激素、胰岛素样生长因子 - I、胰岛素或催乳素介导,一些其他生长因子可能发挥了促进生长的作用。