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TAFRO综合征中的肾脏受累:综述

Renal involvement in TAFRO syndrome: a review.

作者信息

Ubara Yoshifumi, Sawa Naoki

机构信息

Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, Japan.

出版信息

Clin Exp Nephrol. 2025 Jan;29(1):21-28. doi: 10.1007/s10157-024-02573-9. Epub 2024 Nov 3.

Abstract

Renal involvement in TAFRO syndrome is characterized clinically by general edema with ascites and pleural effusions and a rapidly progressive decline in renal function, with urinary protein levels of usually less than 1 g/day. The histologic features of the kidneys can be described as glomerular microangiopathy characterized by mesangiolysis or mesangial loosening, endothelial cell proliferation, edematous opening in the subendothelial space, and glomerular basement membrane (GBM) doubling due to newly formed basement membrane. Findings such as rupture of the GBM, foot-process effacement or fusion, and epithelial cell loss are rare, and thrombus formation is difficult to identify in the glomerulus. Furthermore, immunodeposits are not seen on immunofluorescence staining or electron microscopy. Unlike adults, in addition to the glomerular lesions described above, adolescents appear to show intimal proliferation of the arterioles and interlobular arteries to the vascular poles and occlusion of the vascular lumen.

摘要

TAFRO综合征的肾脏受累在临床上表现为全身性水肿伴腹水和胸腔积液,以及肾功能迅速进行性下降,尿蛋白水平通常低于1g/天。肾脏的组织学特征可描述为肾小球微血管病,其特征为系膜溶解或系膜疏松、内皮细胞增殖、内皮下间隙水肿性开放以及由于新形成的基底膜导致肾小球基底膜(GBM)增厚。GBM破裂、足突消失或融合以及上皮细胞丢失等表现罕见,肾小球内血栓形成难以识别。此外,免疫荧光染色或电子显微镜检查未见免疫沉积物。与成人不同,除上述肾小球病变外,青少年似乎还表现出小动脉和小叶间动脉向血管极的内膜增殖以及血管腔闭塞。

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