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NELL-1相关性膜性肾病——来自印度的病例系列

NELL-1 Associated Membranous Nephropathy - A Case Series from India.

作者信息

Sharma Yogita, Jain Akash, Gupta Pallav, Tiwari Vaibhav, Gupta Anurag, Bhargava Vinant, Malik Manish, Gupta Ashwani, Bhalla Anil Kumar, Rana Devinder Singh

机构信息

Department of Nephrology, Sir Ganga Ram Hospital, New Delhi, India.

Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India.

出版信息

Indian J Nephrol. 2024 Nov-Dec;34(6):654-656. doi: 10.25259/ijn_326_23. Epub 2024 Jun 4.

Abstract

Neural epidermal growth factor-like 1 (NELL-1) is responsible for a substantial proportion of cases of PLA2R-negative primary membranous nephropathy (MN). To investigate the occurrence, association, and treatment responses, we carried out this ambispective study. In the study, nine (10%) of all patients with MN at our center were found to be NELL-1 positive. On follow up, all patients recieved immunosuppression (steroids, cyclophosphamide or rituximab). Five patients were in complete remission, while one patient had partial remission, two patients had a progressive decline in kidney function, and one patient was lost to follow-up early. No link was found with malignancies, traditional Indian medicines or infection.

摘要

神经表皮生长因子样1(NELL-1)是导致相当一部分磷脂酶A2受体(PLA2R)阴性原发性膜性肾病(MN)病例的原因。为了研究其发生情况、相关性及治疗反应,我们开展了这项回顾性和前瞻性相结合的研究。在该研究中,我们中心所有MN患者中有9例(10%)被发现NELL-1呈阳性。在随访过程中,所有患者均接受了免疫抑制治疗(类固醇、环磷酰胺或利妥昔单抗)。5例患者完全缓解,1例部分缓解,2例患者肾功能进行性下降,1例患者早期失访。未发现与恶性肿瘤、传统印度药物或感染存在关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8631/11618949/6a4641c02684/IJN-34-6-654-g1.jpg

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