Fujita Keinosuke, Yokoya Susumu, Fujieda Kenji, Shimatsu Akira, Tachibana Katsuhiko, Tanaka Hiroaki, Tanizawa Takakuni, Teramoto Akira, Nishi Yoshikazu, Hasegawa Yukihiro, Hanew Kunihiko, Horikawa Reiko, Nagai Toshiro, Tanaka Toshiaki
GH Treatment Study Committee, Foundation for Growth Science, Tokyo, Japan ; Department of Pediatrics Children's Medical Center, Osaka City General Hospital, Osaka, Japan.
Clin Pediatr Endocrinol. 2010 Jul;19(3):63-8. doi: 10.1297/cpe.19.63. Epub 2010 Aug 31.
Growth hormone (GH) therapy was approved in 1999 for only GH-deficient Turner syndrome (TS) in Japan. It was subsequently approved for all cases of TS regardless of GH secretory status since 1999. The dose of GH is 1.0 u (0.35 mg)/kg/wk at present, but it was 0.5 u (0.175 mg)/kg/wk before 1999. The adult height in patients with TS on the dose of 0.5 u/kg/wk was studied from the report on of Foundation for Growth Science in 2000. GH therapy was registered for 920 cases, and 258 cases reached adult height. The mean adult height was 145.7 cm. The adult height in patients with TS without GH therapy was reported to be 138 cm in Japan. Thus, the height gain by GH treatment was 7.7 cm. The mean age at the start of GH therapy was 12.0 yr old. The mean duration of GH therapy was 5.6 yr. The mean age at the start of estrogen therapy was 17.0 yr old. Patients in Japan were older at the start of GH and estrogen therapy than in the US and Europe at that time. The adult height and gain of height SD were not correlated with age at the start GH therapy in this study. This may be the result of the older age at the start of GH therapy and the low dose of the GH therapy. Patients are beginning to start GH therapy at a much earlier age and the dose has been doubled in Japan. We expect that the recent data concerning adult height in the patients with TS after GH therapy will improve better than this report.
1999年,生长激素(GH)疗法在日本仅被批准用于生长激素缺乏的特纳综合征(TS)患者。自1999年起,该疗法随后被批准用于所有TS病例,无论其生长激素分泌状态如何。目前GH的剂量为1.0单位(0.35毫克)/千克/周,但在1999年之前为0.5单位(0.175毫克)/千克/周。根据生长科学基金会2000年的报告,对接受0.5单位/千克/周剂量GH治疗的TS患者的成人身高进行了研究。登记接受GH治疗的有920例,其中258例达到成人身高。平均成人身高为145.7厘米。据报道,在日本,未经GH治疗的TS患者的成人身高为138厘米。因此,GH治疗使身高增加了7.7厘米。开始GH治疗的平均年龄为12.0岁。GH治疗的平均持续时间为5.6年。开始雌激素治疗的平均年龄为17.0岁。当时,日本患者开始GH和雌激素治疗的年龄比美国和欧洲的患者大。在本研究中,成人身高和身高增加标准差与开始GH治疗时的年龄无关。这可能是由于开始GH治疗的年龄较大以及GH治疗剂量较低所致。在日本,患者开始GH治疗的年龄要早得多,而且剂量已经翻倍。我们预计,关于GH治疗后TS患者成人身高的最新数据将比本报告中的数据有所改善。