Department of Pediatrics, Dokkyo Medical University, Koshigaya Hospital, Saitama, Japan.
Am J Med Genet A. 2014 Mar;164A(3):671-5. doi: 10.1002/ajmg.a.36355. Epub 2014 Jan 17.
Long-term treatment with growth hormone (GH) in patients with Prader-Willi syndrome (PWS) improves not only height velocity, height standard deviation score, and final height, but also the degree of obesity and body composition abnormalities. Anecdotally, PWS patients tend to suffer from severe obesity and its complications after cessation of GH therapy. However, there have been no studies to investigate changes in body mass index (BMI) and adipose tissue distribution after cessation of GH therapy in young PWS patients. Therefore, we investigated changes in the BMI-standard deviation score (SDS) and adipose tissue distribution after cessation of GH therapy in PWS patients. We evaluated 14 PWS patients. BMI-SDS was calculated at 0, 6, 12, 18, and 24 months before and after cessation of GH treatment. We also evaluated subcutaneous adipose tissue (SAT) (cm(2)) and visceral adipose tissue (VAT) (cm(2)) area in 8 of the 14 study patients with single slice abdominal computed tomography at the level of the umbilicus. The BMI-SDS significantly increased at 6, 12, 18, and 24 months after cessation of GH therapy (P = 0.039, P = 0.008, P = 0.003, P = 0.003, respectively). There was a tendency toward increases in VAT at 12 and 24 months after cessation of GH therapy, but the increases did not reach statistical significance (P = 0.062, P = 0.125, respectively). Therefore, cessation of GH therapy in PWS patients worsened BMI. To maintain good body composition and prevent complications of obesity, long-term use of GH in adult PWS patients may be advisable.
长期给予生长激素（GH）治疗可改善 Prader-Willi 综合征（PWS）患者的身高增长速度、身高标准差评分和最终身高，还可改善肥胖程度和身体成分异常。据报道，PWS 患者在停止 GH 治疗后往往会遭受严重肥胖及其并发症的困扰。然而，目前尚无研究调查年轻 PWS 患者停止 GH 治疗后体重指数（BMI）和脂肪组织分布的变化。因此，我们调查了 PWS 患者停止 GH 治疗后 BMI 标准差评分（SDS）和脂肪组织分布的变化。我们评估了 14 例 PWS 患者。在 GH 治疗停止前和停止后 0、6、12、18 和 24 个月，计算 BMI-SDS。我们还对 14 例研究患者中的 8 例在脐水平进行单次腹部 CT 评估时，评估了皮下脂肪组织（SAT）（cm²）和内脏脂肪组织（VAT）（cm²）面积。GH 治疗停止后 6、12、18 和 24 个月时，BMI-SDS 显著增加（P = 0.039，P = 0.008，P = 0.003，P = 0.003）。停止 GH 治疗后 12 个月和 24 个月时，VAT 有增加的趋势，但差异无统计学意义（P = 0.062，P = 0.125）。因此，PWS 患者停止 GH 治疗会使 BMI 恶化。为了维持良好的身体成分并预防肥胖相关并发症，PWS 成年患者长期使用 GH 可能是合理的。
