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Immunoglobulin-Negative DNAJB9-Associated Fibrillary... » Isaúde
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American Journal of Kidney Diseases
2020-07-22 00:00:00

Immunoglobulin-Negative DNAJB9-Associated Fibrillary Glomerulonephritis: A Report of 9 Cases

Description: Fibrillary glomerulonephritis (FGN) was previously defined by glomerular deposition of haphazardly oriented fibrils that stain with antisera to immunoglobulins but do not stain with Congo red. We report what is to our knowledge the first series of immunoglobulin-negative FGN, consisting of 9 adults (7 women and 2 men) with a mean age at diagnosis of 66 years. Patients presented with proteinuria (100%; mean protein excretion, 3g/d), hematuria (100%), and elevated serum creatinine level (100%). Comorbid conditions included carcinoma in 3 and hepatitis C virus infection in 2; no patient had hypocomplementemia or monoclonal gammopathy.

Identifier: 10.1053/j.ajkd.2020.04.015
Section: Case Reports
Volume: 0
Author: Samar M. Said, Alejandro Best Rocha, Virginie Royal, Anthony M. Valeri, Christopher P. Larsen, Jason D. Theis, Julie A. Vrana, Ellen D. McPhail, Lalitha Bandi, Saied Safabakhsh, Chadwick Barnes, Lynn D. Cornell, Mary E. Fidler, Mariam Priya Alexander

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