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患有普拉德-威利综合征(PWS)的儿童和青少年中的非酒精性脂肪性肝病(NAFLD)。

Non-Alcoholic Fatty Liver Disease (NAFLD) in children and adolescents with Prader-Willi Syndrome (PWS).

作者信息

Fintini D, Inzaghi E, Colajacomo M, Bocchini S, Grugni G, Brufani C, Cappa M, Nobili V, Cianfarani S, Crinò A

机构信息

Autoimmune Endocrine Diseases Unit, Bambino Gesù Children's Hospital, Research Institute, Palidoro, Rome, Italy.

Endocrinology and Diabetic Unit, Bambino Gesù Children's Hospital, Research Institute, Palidoro, Rome, Italy.

出版信息

Pediatr Obes. 2016 Jun;11(3):235-8. doi: 10.1111/ijpo.12052. Epub 2015 Jul 1.

Abstract

We tested the hypothesis that patients with Prader-Willi syndrome (PWS) may be at lower risk of developing non-alcoholic fatty liver disease (NAFLD) because of a higher insulin sensitivity. Twenty-one PWS patients and 42 control subjects closely similar for age, gender, pubertal stage and body mass index (CNT), were studied. Metabolic profile and body composition were assessed. NAFLD was established by a validated method of US grading (range from G0 to G3). PWS patients showed a significantly better metabolic profile (lower waist circumference, fasting glucose levels, HOMA-IR, cholesterol, transaminase levels and trunk fat mass/fat mass ratio). Furthermore, NAFLD G1stage was significantly more frequent in PWS subjects (P < 0.05), whereas G2 stage was significantly more frequent in control patients (P < 0.05). NAFLD grading seems to correlate with body composition in PWS, also after adjustment for sex and GH treatment. To our knowledge, this is the first report suggesting a reduced risk of NAFLD in PWS children.

摘要

我们检验了以下假设

普拉德-威利综合征(PWS)患者由于胰岛素敏感性较高,发生非酒精性脂肪性肝病(NAFLD)的风险可能较低。我们研究了21例PWS患者和42例在年龄、性别、青春期阶段和体重指数方面与之非常相似的对照受试者(CNT)。评估了代谢概况和身体组成。通过一种经过验证的超声分级方法(范围从G0到G3)确定NAFLD。PWS患者表现出明显更好的代谢概况(腰围、空腹血糖水平、稳态模型评估的胰岛素抵抗指数、胆固醇、转氨酶水平以及躯干脂肪量/脂肪量比率更低)。此外,NAFLD G1期在PWS受试者中显著更常见(P < 0.05),而G2期在对照患者中显著更常见(P < 0.05)。在对性别和生长激素治疗进行调整后,NAFLD分级似乎与PWS患者的身体组成相关。据我们所知,这是首份提示PWS儿童发生NAFLD风险降低的报告。

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