Renal complications of lipodystrophy: A closer look at the natural history of kidney disease.
Renal complications of lipodystrophy: A closer look at the natural history of kidney disease.
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DOI：
10.1111/cen.13732
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发表时间：
2018-07
影响因子：
3.2
通讯作者：
Oral EA
中科院分区：
文献类型：
--
作者：
Akinci B;Unlu SM;Celik A;Simsir IY;Sen S;Nur B;Keskin FE;Ozgen Saydam B;Kutbay Ozdemir N;Sarer Yurekli B;Ergur BU;Sonmez M;Atik T;Arslan A;Demir T;Altay C;Tunc UA;Arkan T;Gen R;Eren E;Akinci G;Yilmaz AA;Bilen H;Ozen S;Celtik A;Savas Erdeve S;Cetinkaya S;Onay H;Sarioglu S;Oral EA
脂肪营养不良综合征是一组以脂肪组织丢失为特征的异质性疾病。在以前的报告中，蛋白尿是一个引人注目的发现。在这项多中心研究中，从土耳其脂肪营养不良研究组数据库中登记的103名非HIV相关性脂肪营养不良患者中收集了前瞻性随访数据，以研究治疗未治疗的脂肪营养不良患者的肾脏并发症。主要观察指标包括通过研究蛋白尿水平和估计的肾小球滤过率(EGFR)来确定慢性肾脏疾病(CKD)。测量肾脏体积。9例患者行经皮肾活检。37例全身性脂肪营养不良患者中有17例以蛋白尿为特征的CKD，66例部分性脂肪营养不良患者中有29例有CKD表现，其中12例进展为肾功能衰竭。泛发性脂营养不良患者出现肾脏并发症的时间明显较早。CKD患者年龄较大，胰岛素抵抗较多，代谢控制较差。肾脏体积的增加与代谢控制不良和瘦素水平受抑有关。肾活检可见肾小球基底膜增厚、系膜基质异常、足细胞损伤、局灶节段性硬化、缺血性改变及不同程度的肾小管异常。电子显微镜图像上可见脂质空泡。CKD在发病较早的脂肪营养不良患者中非常常见。肾脏受累似乎是多因素的。虽然在某些情况下，严重的胰岛素抵抗导致的糖尿病控制不佳可能会导致疾病的发生，但内在的潜在遗传缺陷也可能导致导致肾脏疾病发病的细胞自主机制。
Lipodystrophy syndromes are a group of heterogeneous disorders characterized by adipose tissue loss. Proteinuria is a remarkable finding in previous reports. In this multi-center study, prospective follow-up data were collected from 103 subjects with non-HIV associated lipodystrophy registered in the Turkish Lipodystrophy Study Group database to study renal complications in treatment naïve patients with lipodystrophy. Main outcome measures included ascertainment of chronic kidney disease (CKD) by studying the level of proteinuria and the estimated glomerular filtration rate (eGFR). Kidney volume was measured. Percutaneous renal biopsies were performed in 9 patients. Seventeen of 37 patients with generalized and 29 of 66 patients with partial lipodystrophy had CKD characterized by proteinuria, of those 12 progressed to renal failure subsequently. The onset of renal complications was significantly earlier in patients with generalized lipodystrophy. Patients with CKD were older and more insulin resistant and had worse metabolic control. Increased kidney volume was associated with poor metabolic control and suppressed leptin levels. Renal biopsies revealed thickening of glomerular basal membranes, mesangial matrix abnormalities, podocyte injury, focal segmental sclerosis, ischemic changes and tubular abnormalities at various levels. Lipid vacuoles were visualized in electron microscopy images. CKD is conspicuously frequent in patients with lipodystrophy which has an early onset. Renal involvement appears multifactorial. While poorly controlled diabetes caused by severe insulin resistance may drive the disease in some cases, inherent underlying genetic defects may also lead to cell-autonomous mechanisms contributory to the pathogenesis of kidney disease.
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影响因子：
3.5
作者：
Haque, WA;Vuitch, F;Garg, A
通讯作者：
Garg, A
影响因子：
8
作者：
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影响因子：
5.8
作者：
Javor, ED;Moran, SA;Gorden, P
通讯作者：
Gorden, P
影响因子：
8
作者：
Hogg, RJ;Portman, RJ;Ingelfinger, J
通讯作者：
Ingelfinger, J
影响因子：
19.6
作者：
Kambham, N;Markowitz, GS;D'Agati, VD
通讯作者：
D'Agati, VD