enalapril and Wiskott-Aldrich-Syndrome

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.

Topics: Angiotensin-Converting Enzyme Inhibitors; Child; Enalapril; Glomerulonephritis, IGA; Hematuria; Humans; Immunosuppressive Agents; Male; Proteinuria; Splenectomy; Thrombocytopenia; Wiskott-Aldrich Syndrome