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普拉德-威利综合征成年患者停止生长激素治疗后不久,内脏脂肪组织会增加。

Visceral adipose tissue increases shortly after the cessation of GH therapy in adults with Prader-Willi syndrome.

作者信息

Koizumi Mikiko, Ida Shinobu, Shoji Yasuko, Nishimoto Yukiko, Etani Yuri, Kawai Masanobu

机构信息

Department of Gastroenterology and Endocrinology, Osaka Women's and Children's Hospital, Osaka 594-1101, Japan.

Department of Pediatrics, Yodogawa Christian Hospital, Osaka 533-0024, Japan.

出版信息

Endocr J. 2018 Nov 29;65(11):1127-1137. doi: 10.1507/endocrj.EJ18-0107. Epub 2018 Sep 4.

Abstract

GH therapy in pediatric patients with Prader-Willi syndrome (PWS) improves body composition, but discontinuation of GH after achieving adult height has been implicated in its deterioration. Although there is evidence for the deleterious effects of visceral adipose tissue (VAT) rather than subcutaneous adipose tissue (SAT) on the development of obesity-related complications, the effects of GH discontinuation on fat distribution in adults with PWS has not been fully investigated. Therefore, we utilized dual-energy X-ray absorptiometry (DEXA) and abdominal computed tomography (CT) to compare the fat distribution between before and 6 months or 12 months after the cessation of GH therapy in 7 adult PWS patients. GH therapy was initiated at a mean age of 4.1 ± 1.4 years and discontinued at a mean age of 18.9 ± 1.8 years. Serum IGF-1 levels were decreased by discontinuation of GH therapy. Fat mass was significantly increased 6 and 12 months after GH cessation, whereas muscle mass and bone mineral density were unchanged during both study periods. Abdominal CT analysis revealed that elevations in fat mass were due to increases in VAT rather than SAT. Circulating low-density lipoprotein (LDL) cholesterol levels were significantly elevated 6 months after GH cessation. In conclusion, discontinuation of GH therapy caused rapid increases in visceral adipose tissue and LDL cholesterol levels. These findings indicate that continuation of GH therapy may be a therapeutic option to maintain body composition; however, further studies regarding the long-term benefits and adverse effects of GH therapy in adults with PWS are required.

摘要

生长激素(GH)治疗普拉德-威利综合征(PWS)患儿可改善身体成分,但成年身高达成后停用GH与身体成分恶化有关。尽管有证据表明内脏脂肪组织(VAT)而非皮下脂肪组织(SAT)对肥胖相关并发症的发生有有害影响,但GH停用对成年PWS患者脂肪分布的影响尚未得到充分研究。因此,我们利用双能X线吸收法(DEXA)和腹部计算机断层扫描(CT)比较了7例成年PWS患者在GH治疗停止前、停止后6个月或12个月时的脂肪分布情况。GH治疗起始的平均年龄为4.1±1.4岁,停用的平均年龄为18.9±1.8岁。停用GH治疗后血清胰岛素样生长因子-1(IGF-1)水平下降。GH停止后6个月和12个月时脂肪量显著增加,而在两个研究期间肌肉量和骨矿物质密度均无变化。腹部CT分析显示,脂肪量增加是由于VAT增加而非SAT增加。GH停止后6个月时循环低密度脂蛋白(LDL)胆固醇水平显著升高。总之,停用GH治疗导致内脏脂肪组织和LDL胆固醇水平迅速升高。这些发现表明,继续进行GH治疗可能是维持身体成分的一种治疗选择;然而,需要进一步研究GH治疗对成年PWS患者的长期益处和不良反应。

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