Bozzola M, Argente J, Cisternino M, Moretta A, Valtorta A, Biscaldi I, Donnadieu M, Evain-Brion D, Severi F
Paediatric Clinic, University of Pavia, Italy.
Eur J Pediatr. 1989 Jan;148(4):300-3. doi: 10.1007/BF00444118.
The effect of long-term human chorionic gonadotropin (HCG) therapy on the linear growth and biological growth parameters was studied in six thalassaemic boys aged 14.5-15.5 years old with hypogonadotropic hypogonadism. A significant (P less than 0.001) increase in growth velocity (from 3.3 +/- 0.3 to 7.6 +/- 0.6 cm/year) was found after 6-12 months of therapy, without acceleration of bone age. A striking improvement in pubertal development was observed. The treatment significantly increased growth hormone (GH) response to L-dopa administration (P less than 0.025) as well as sleep GH secretion (P less than 0.025). Serum growth factors, evaluated as thymidine activity during deep sleep, increased (P less than 0.001), but somatomedin C (Sm-C) levels did not. Prior to treatment, baseline and peak values of plasma growth hormone releasing hormone (GH-RH) following L-dopa were low. After HCG therapy, GH-RH response to L-dopa increased significantly (from 9.2 +/- 5.6 to 20.2 +/- 6.2 pg/ml; P less than 0.05), but remained (P less than 0.001) lower than in normal prepubertal children. This study suggests that in thalassaemia major an impaired GH-RH release can be observed, in addition to the described alteration in Sm-C generation.
对6名年龄在14.5至15.5岁、患有低促性腺激素性性腺功能减退的地中海贫血男孩,研究了长期人绒毛膜促性腺激素(HCG)治疗对线性生长和生物学生长参数的影响。治疗6至12个月后,生长速度显著增加(从3.3±0.3厘米/年增至7.6±0.6厘米/年,P<0.001),且骨龄未加速。青春期发育有显著改善。治疗显著增加了生长激素(GH)对左旋多巴给药的反应(P<0.025)以及睡眠中GH分泌(P<0.025)。作为深睡眠期间胸苷活性评估的血清生长因子增加(P<0.001),但生长调节素C(Sm-C)水平未增加。治疗前,左旋多巴给药后血浆生长激素释放激素(GH-RH)的基线值和峰值较低。HCG治疗后,GH-RH对左旋多巴的反应显著增加(从9.2±5.6增至20.2±6.2皮克/毫升;P<0.05),但仍(P<0.001)低于正常青春期前儿童。这项研究表明,在地中海贫血重型中,除了所描述的Sm-C生成改变外,还可观察到GH-RH释放受损。