Wiskott–Aldrich syndrome with IgA nephropathy: a case report and literature review
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- Liu, CH., Wu, KH., Lin, TY. et al. Int Urol Nephrol (2013) 45: 1495. doi:10.1007/s11255-012-0178-0
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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.
KeywordsAntibiotic prophylaxis IgA nephropathy Splenectomy Wiskott–Aldrich syndrome
End-stage renal disease