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普拉德-威利综合征患者脂肪分布特征:与脂肪细胞因子的关系及其对生长激素治疗的影响。

Characterization of fat distribution in Prader-Willi syndrome: relationships with adipocytokines and influence of growth hormone treatment.

机构信息

Department of Pediatrics, Dokkyo Medical University Koshigaya Hospital, Saitama, Japan.

出版信息

Am J Med Genet A. 2013 Jan;161A(1):27-33. doi: 10.1002/ajmg.a.35653. Epub 2012 Dec 13.

Abstract

Marked anthropometric changes are seen in Prader-Willi syndrome (PWS). Emaciation is observed during infancy, whereas severe obesity is found in older children and adults. Growth hormone (GH) treatment modifies the anthropometric changes in PWS patients. In this study, we examined changes in the body composition of 51 PWS patients (age range, 6-54 years; median, 16.5 years), with a focus on the amount of abdominal visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT), VAT/SAT ratio, and serum levels of adipocytokines (adiponectin, leptin, and resistin). The relationships between VAT, SAT, and adipocytokines, and lipid abnormalities and type 2 diabetes in 24 patients with obese PWS were also evaluated. With increasing age, SAT and VAT both increased markedly, but in 18 patients receiving GH treatment, VAT remained low at ≤30 cm(2) . In the GH-completed patients (n = 19), VAT and SAT increased with age to levels similar to those in non-GH-treated patients (n = 14). In the obese group, adiponectin decreased as VAT increased (r = -0.35, P = 0.11). Leptin (r = 0.67, P < 0.001) and resistin (r = 0.45, P = 0.04) showed positive correlations with SAT. Total cholesterol, low-density lipoprotein, and triglyceride levels correlated negatively with adiponectin (r = -0.59, r = -0.56, r = -0.56, respectively, P < 0.05) and hemoglobin A1c (r = -0.42, P = 0.08). To maintain lower VAT and prevent cardiovascular disease risk factors, GH treatment may be advisable even in adult patients with PWS.

摘要

普拉德-威利综合征(PWS)患者会出现明显的人体测量学变化。婴儿期会出现消瘦,而在较大的儿童和成人中则会出现严重肥胖。生长激素(GH)治疗可改变 PWS 患者的人体测量学变化。在这项研究中,我们检查了 51 名 PWS 患者(年龄范围 6-54 岁;中位数 16.5 岁)的身体成分变化,重点关注腹部内脏脂肪组织(VAT)和皮下脂肪组织(SAT)的量、VAT/SAT 比值以及血清脂联素、瘦素和抵抗素等脂肪细胞因子的水平。还评估了 24 名肥胖 PWS 患者中 VAT、SAT 和脂肪细胞因子与血脂异常和 2 型糖尿病之间的关系。随着年龄的增长,SAT 和 VAT 均显著增加,但在 18 名接受 GH 治疗的患者中,VAT 仍保持在≤30cm2以下。在完成 GH 治疗的患者(n=19)中,VAT 和 SAT 随年龄增加至与未接受 GH 治疗的患者(n=14)相似的水平。在肥胖组中,随着 VAT 的增加,脂联素减少(r=-0.35,P=0.11)。瘦素(r=0.67,P<0.001)和抵抗素(r=0.45,P=0.04)与 SAT 呈正相关。总胆固醇、低密度脂蛋白和甘油三酯水平与脂联素呈负相关(r=-0.59,r=-0.56,r=-0.56,P<0.05)和糖化血红蛋白(r=-0.42,P=0.08)。为了维持较低的 VAT 和预防心血管疾病的危险因素,即使对于成年 PWS 患者,GH 治疗也可能是明智的。

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