title: Wiskott-Aldrich syndrome with IgA nephropathy: a case report and literature review.
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.
authors.affiliation: Department of Paediatrics, Taichung Armed Forces General Hospital, Taichung, Taiwan, ROC.
authors.forename: Chia-Hung
authors.initials: CH
authors.lastname: Liu
authors.forename: Kang-Hsi
authors.initials: KH
authors.lastname: Wu
authors.forename: Tze-Yi
authors.initials: TY
authors.lastname: Lin
authors.forename: Chang-Ching
authors.initials: CC
authors.lastname: Wei
authors.forename: Ching-Yuang
authors.initials: CY
authors.lastname: Lin
authors.forename: Xian-Xiu
authors.initials: XX
authors.lastname: Chen
authors.forename: Wen-I
authors.initials: WI
authors.lastname: Lee
mesh_terms: D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D002648:Child; D004656:Enalapril / Q000627:therapeutic use; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D006417:Hematuria / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011507:Proteinuria / Q000209:etiology; D013156:Splenectomy; D013921:Thrombocytopenia / Q000209:etiology / Q000601:surgery; D014923:Wiskott-Aldrich Syndrome / Q000150:complications*
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D007166:Immunosuppressive Agents; D004656:Enalapril