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Review
. 2013 Oct;45(5):1495-500.
doi: 10.1007/s11255-012-0178-0. Epub 2012 May 1.

Wiskott-Aldrich syndrome with IgA nephropathy: a case report and literature review

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Review

Wiskott-Aldrich syndrome with IgA nephropathy: a case report and literature review

Chia-Hung Liu et al. Int Urol Nephrol. 2013 Oct.

Abstract

The pathogenesis of renal involvement in Wiskott-Aldrich syndrome (WAS) is unclear and renal outcome is generally poor in such situations. Here we present the case of an 8-year-old boy with WAS who developed hematuria, proteinuria, and declining renal function that did not improve with the combined use of immunosuppressive agents and angiotensin-converting-enzyme inhibitor. Renal pathology revealed IgA nephropathy (IgAN). The patient underwent splenectomy for refractory thrombocytopenia. The proteinuria remitted and renal function improved after splenectomy, long-term antibiotic prophylaxis, and tapering of immunosuppressive agents.

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