Division of Pediatric Endocrinology and Diabetology, University Children's Hospital, Essen, Germany.
Horm Res Paediatr. 2011;75(1):56-62. doi: 10.1159/000319612. Epub 2010 Oct 1.
Growth hormone (GH) secretory status and GH therapy influence cardiac muscle mass and function. Most patients with Prader-Willi syndrome (PWS) are GH-deficient (GHD), and are at risk to develop obesity-related cardiac problems. Few data exist on the development of cardiac dimensions under GH treatment in PWS.
in a retrospective study, cardiac dimensions measured echocardiographically in 26 children with PWS before and after 22.9 ± 11.0 months of GH therapy (0.035 mg/kg/day) were compared to those of 13 GHD children treated with GH (0.025-0.035 mg/kg/day) over 13.5 ± 4.2 months.
low-normal left ventricular end-systolic [diastolic] diameter SDS increased in PWS patients (1.41, p < 0.0001 [1.23, p < 0.0001]) and in controls (0.61, p = 0.04 [0.78, p = 0.013]), becoming elevated above normal in 2/26 PWS patients. There was a trend for a higher GH effect on left ventricular end-systolic diameter in PWS (p = 0.071). Mean values of all echocardiographic dimensions remained in the normal range in both PWS patients and controls, before and under GH therapy.
the response of cardiac dimensions to GH treatment is similar in PWS patients and GHD controls, with a trend towards more elevated left ventricular parameters in PWS. Therefore, regular echocardiographic assessment may be considered in PWS children with longer treatment duration.
生长激素(GH)分泌状态和 GH 治疗会影响心肌质量和功能。大多数普拉德-威利综合征(PWS)患者存在生长激素缺乏(GHD),并且存在发生肥胖相关心脏问题的风险。关于 PWS 患者在 GH 治疗下心脏尺寸的发展,现有数据较少。
在一项回顾性研究中,比较了 26 名接受 22.9±11.0 个月(0.035mg/kg/天)GH 治疗(0.035mg/kg/天)的 PWS 儿童和 13 名接受 GH(0.025-0.035mg/kg/天)治疗 13.5±4.2 个月的 GHD 儿童治疗前后的超声心动图测量的心脏尺寸。
左心室收缩末期[舒张末期]直径 SDS 较低,正常 PWS 患者增加(1.41,p<0.0001[1.23,p<0.0001])和对照组(0.61,p=0.04[0.78,p=0.013]),26 名 PWS 患者中有 2 名升高超过正常。PWS 患者的左心室收缩末期直径对 GH 的影响有增高趋势(p=0.071)。在 GH 治疗前后,所有超声心动图参数的平均值在 PWS 患者和对照组中均保持在正常范围内。
心脏尺寸对 GH 治疗的反应在 PWS 患者和 GHD 对照组中相似,PWS 患者的左心室参数有增高趋势。因此,对于治疗时间较长的 PWS 儿童,可考虑定期进行超声心动图评估。