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长期接受生长激素治疗的软骨发育不全患者的最终成人身高

Final adult height in long-term growth hormone-treated achondroplasia patients.

作者信息

Harada Daisuke, Namba Noriyuki, Hanioka Yuki, Ueyama Kaoru, Sakamoto Natsuko, Nakano Yukako, Izui Masafumi, Nagamatsu Yuiko, Kashiwagi Hiroko, Yamamuro Miho, Ishiura Yoshihito, Ogitani Ayako, Seino Yoshiki

机构信息

Department of Pediatrics, Osaka Hospital, Japan Community Healthcare Organization (JCHO), 4-2-78, Fukushima, Fukushima-ku, Osaka, Osaka, 553-0003, Japan.

Neonatal Intensive Care Unit, Nara Prefecture General Medical Center, Nara, Japan.

出版信息

Eur J Pediatr. 2017 Jul;176(7):873-879. doi: 10.1007/s00431-017-2923-y. Epub 2017 May 13.

Abstract

UNLABELLED

The objective of this study was to evaluate the gain in final height of achondroplasia (ACH) patients with long-term growth hormone (GH) treatment. We analyzed medical data of 22 adult patients (8 males and 14 females) treated with GH at a dose of 0.05 mg/kg/day. Optionally, tibial lengthening (TL) was performed with the Ilizalov method in 15 patients and TL as well as femoral lengthening (FL) in 6 patients. Concomitant gonadal suppression therapy with buserelin acetate was applied in 13 patients. The mean treatment periods with GH were 10.7 ± 4.0 and 9.3 ± 2.5 years for males and females, respectively. GH treatment augmented the final height +0.60 ± 0.52 SD (+3.5 cm) and +0.51 ± 1.29 SD (+2.8 cm) in males and females compared to non-treated ACH patients, respectively. Final height of ACH patients that underwent GH and TL increased +1.72 ± 0.72 SD (+10.0 cm) and +1.95 ± 1.34 SD (+9.8 cm) in males and females, respectively. GH, TL, and FL increased their final height +2.97 SD (+17.2 cm) and +3.41 ± 1.63 SD (+17.3 cm) in males and females, respectively. Gonadal suppression therapy had no impact on final height.

CONCLUSIONS

Long-term GH treatment contributes to 2.6 and 2.1% of final adult height in male and female ACH patients, respectively.

摘要

未标注

本研究的目的是评估长期生长激素(GH)治疗对软骨发育不全(ACH)患者最终身高增长的影响。我们分析了22例接受GH治疗的成年患者(8例男性和14例女性)的医疗数据,GH剂量为0.05mg/kg/天。15例患者采用伊里扎洛夫方法进行了胫骨延长(TL),6例患者进行了TL以及股骨延长(FL)。13例患者同时应用醋酸布舍瑞林进行性腺抑制治疗。男性和女性接受GH治疗的平均时间分别为10.7±4.0年和9.3±2.5年。与未接受治疗的ACH患者相比,GH治疗使男性和女性的最终身高分别增加了+0.60±0.52标准差(+3.5厘米)和+0.51±1.29标准差(+2.8厘米)。接受GH和TL治疗的ACH患者,男性和女性的最终身高分别增加了+1.72±0.72标准差(+10.0厘米)和+1.95±1.34标准差(+9.8厘米)。GH、TL和FL使男性和女性的最终身高分别增加了+2.97标准差(+17.2厘米)和+3.41±1.63标准差(+17.3厘米)。性腺抑制治疗对最终身高没有影响。

结论

长期GH治疗分别使男性和女性ACH患者的最终成年身高增加了2.6%和2.1%。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac7b/5486548/f029029f719e/431_2017_2923_Fig1_HTML.jpg

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