Okabe T, Nishikawa K, Miyamori C, Sato T
Department of Pediatrics, School of Medicine, Kanazawa University, Japan.
Acta Paediatr Jpn. 1991 Jun;33(3):357-62. doi: 10.1111/j.1442-200x.1991.tb01566.x.
To evaluate the effect of human growth hormone (hGH) on the growth of children with achondroplasia, three patients with achondroplasia and one patient with hypochondroplasia were treated with 0.5 IU/kg/W of pituitary-extracted hGH for 6-12 months. Mean height velocity was significantly increased from 4.0 +/- 0.4 to 7.5 +/- 0.7 cm/year (P less than 0.05) by hGH. The tibial index, defined as the length/width ratio of left tibia, did not change during the treatment, indicating that hGH promotes growth without exaggeration of tubular bone deformity. Case 2, who had atlantoaxial dislocation, developed sleep apnea and neurological deficits during the second hGH treatment, but these were cured by operation. Thus, hGH therapy is effective in promoting growth in patients with achondroplasia, but the complication of atlantoaxial dislocation should be explored and corrected before the treatment.
为评估人生长激素(hGH)对软骨发育不全患儿生长的影响,对3例软骨发育不全患者和1例低软骨发育不全患者采用垂体提取的hGH,剂量为0.5 IU/kg/周,治疗6至12个月。hGH治疗后,平均身高增长速度从4.0±0.4显著增加至7.5±0.7厘米/年(P<0.05)。胫骨指数定义为左胫骨长度与宽度之比,在治疗期间未发生变化,表明hGH促进生长但不加重管状骨畸形。病例2患有寰枢椎脱位,在第二次hGH治疗期间出现睡眠呼吸暂停和神经功能缺损,但通过手术治愈。因此,hGH治疗对促进软骨发育不全患者的生长有效,但在治疗前应排查并纠正寰枢椎脱位并发症。