部分脂肪营养不良合并膜增殖性肾小球肾炎一例

Mesangiocapillary glomerulonephritis associated with partial lipodystrophy: a case report

目的对１例以水肿、蛋白尿为主诉入院的疑难病例进行确诊。方法根据患儿的症状、体征、特殊检查（血补体Ｃ３、皮肤、肾活组织病理检查等）及文献资料进行分析、确诊。结果患儿以间断水肿、蛋白尿为主要表现，面容呈尸灰色，双面颊皮下脂肪消失，左肩部皮下脂肪欠丰满；血补体Ｃ３明显下降，左肩部皮肤活组织病理检查显示皮下脂肪部分萎缩，肾活组织病理检查显示膜增殖性肾小球肾炎Ｉ型改变。结论确诊患儿为部分脂肪营养不良合并膜增殖性肾小球肾炎（Ｉ型）。此病少见，应提高认识，以防漏诊。

Objective To diagnose a difficult and complicated case suffering from edema and proteinuria for 4 months. Methods Diagnosis was confirmed according to the symptoms, signs, special laboratory examinations( serum complement 3 and skin and renal biopsies, etc.) and previously published data. Results The patient manifested as edema, proteinuria, special “carcass” appearance, depression of the cheek due to loss of subcutaneous fat tissue, slight depression of the shoulder, low serum complement 3. A biopsy sample taken from his shoulder showed partial atrophy, and renal biopsy showed the typical mesangiocapillary glomerulonephritis, type Ⅱ ( dense deposit disease). Conclusion The diagnosis of partial lipodystrophy associated with mesangiocapillary glomerulonephritis of the dense deposit type was established. Great attention should be paid to this rare disease.