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首页> 外文期刊>Journal of the Endocrine Society. >SUN-598 Phenotypic Study of Meso-Somatous (Roch-Leri) Lipomatosis
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SUN-598 Phenotypic Study of Meso-Somatous (Roch-Leri) Lipomatosis

机译：Sun-598中索 - 躯体（Roch-Leri）脂质病的表型研究

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Background: Lipomatosis is a condition in which multiple lipomas are present on the body. Different entities which are accompanied by multiple lipomas include Proteus syndrome, Cowden syndrome and related disorders due to PTEN gene mutations, MEN1, benign symmetric lipomatosis (Madelung or Launois-Bensaude disease), Dercum’s Disease, familial lipodystrophy, hibernomas, epidural lipomatosis, familial angiolipomatosis, and meso-somatous lipomatosis (LMS) still called Roch-Leri lipomatosis. LMS is characterized by the presence of many discrete, encapsulated lipomas of 2 to 5 cm, painless, at the level of the trunk and forearms. The aim of the present study was to determine the clinico-biological phenotype of LMS, as compared to controls. Patients and methods: In this single-center study (NCT01784289), 18 healthy controls (C) and 11 LMS were included after examining the cohort of 76 patients referred for suspected lipodystrophic syndrome between 2009 and 2019 in the Endocrinology Department of a University Hospital. Clinical (sex, age, weight, BMI, blood pressure (BP), alcohol consumption), metabolic (fasting blood glucose (FBG) and insulin levels, lipid balance, ASAT, ALAT, GammaGT, leptin), immuno-inflammatory (CBC, lymphocyte immunophenotyping), and anthropometric (% of body fat in DEXA, steatosis and intra-abdominal / total abdominal fat ratio (IAF / TAF) in MRI) were evaluated. Results: The following parameters, expressed as % or median, differed significantly between LMS vs. C groups, respectively: weight 100 vs. 69kg (p0.01), BMI 30.8 vs. 22.7 (p0.01), systolic BP 140 vs. 115 (p0.01) and diastolic BP 80 vs. 70 mmHg (p0.05), gammaGT 74 vs. 18 IU / L (p0.01), fasting insulin levels 7.3 vs. 4.7 microIU / mL (p0.05), leptin 28 vs. 5 ng / mL (p0.01), CD3 (867 vs. 1444 / mm3 (p0.01), CD4 499 vs 866 / mm3 (p0.05), CD8 227 vs. 546 (p0.05), fat mass 41 vs. 22 (p 5 in 82% of LMS patients and lipomas were localized first to the forearms (82%), then the thighs (73%) and the abdomen (55%). At diagnosis, the age of LMS patients was 20 years old; 55% of the LMS patients had a BMI above 30 and 45% above 25. No patient had excessive alcohol consumption. Five had a history of auto-immuneor inflammatory disease: 1 hyperthyroidism, 1 hypothyroidism, 1 multiple sclerosis, 1 vitiligo, 1 Raynaud syndrome. Conclusion : LMS mainly affects overweight men and is associated with hypertension, hyperinsulinism, increased gammaGT and a decrease in CD3, CD4 and CD8 lymphocytes, suggesting an immune dysregulation, all the more so that 45% had an associated auto-immune/inflammatory disease.
机译：背景：脂质病是在身体上存在多个Lipomas的病症。伴随多种Lipomas的不同实体包括PTEN基因突变，MEN1，良性对称脂质病（Madelung或Launois-Bensaude疾病），Dercum疾病，家族性脂肪疗法，冬眠，硬膜外病变，家族性血管症，中躯体脂质瘤（LMS）仍称为罗氏脂质瘤。 LMS的特征在于存在许多离散，包封的Lipomas 2至5cm，在躯干和前臂的水平下疼痛。与对照相比，本研究的目的是确定LMS的临床生物表型。患者和方法：在该单一的研究（NCT01784289）中，在检查大学医院内分泌部门的疑似脂肪竞技综合征的76名患者队列后，包括18例健康对照（C）和11 LMS。临床（性，年龄，体重，BMI，血压（BP），醇消耗），代谢（空腹血糖（FBG）和胰岛素水平，脂肪平衡，Asat，Alat，γ，瘦素），免疫炎症（CBC，评价淋巴细胞免疫蛋白型，并评估了人体测定量（DEXA中体脂肪的百分比，脂肪变性和腹部/腹部/总腹部脂肪比（IAF / TAF）中的含量）。结果：以下参数表示为％或中值，分别在LMS与C组之间显着不同：重量100与69kg（p <0.01），BMI 30.8与22.7（P <0.01），收缩压BP 140与。 115（P <0.01）和舒张压BP 80对70 mmHg（P <0.05），γ74对18 IU / L（P <0.01），禁食胰岛素水平7.3与4.7微碘/ mL（P <0.05），瘦素28与5 ng / ml（p <0.01），CD3（867 vs.1444 / mm3（P <0.01），CD4 499 Vs 866 / mm3（P <0.05），CD8 227和546（P <0.05） ，脂肪质量41对22（82％的LMS患者和Lipomas的P 5首先定位于前臂（82％），然后大腿（73％）和腹部（55％）。在诊断中，年龄LMS患者20岁; 55％的LMS患者的BMI高于30％和45％以上。没有患者饮用过多的酒精消费。五有炎症炎症症的历史：1甲状腺功能亢进，1个甲状腺功能亢进，1个硬化症，1个白癜风，1 raynaud综合征。结论：LMS主要影响超重男性并相关随着高血压，高胰岛素，γ含量增加和CD3，CD4和CD8淋巴细胞的减少，表明免疫失调，更为改善，因此45％具有相关的自身免疫/炎性疾病。

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