International Urology and Nephrology

, Volume 45, Issue 5, pp 1495–1500

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

  • Chia-Hung Liu
  • Kang-Hsi Wu
  • Tze-Yi Lin
  • Chang-Ching Wei
  • Ching-Yuang Lin
  • Xian-Xiu Chen
  • Wen-I Lee
Nephrology – Case Report

DOI: 10.1007/s11255-012-0178-0

Cite this article as:
Liu, CH., Wu, KH., Lin, TY. et al. Int Urol Nephrol (2013) 45: 1495. doi:10.1007/s11255-012-0178-0

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.

Keywords

Antibiotic prophylaxis IgA nephropathy Splenectomy Wiskott–Aldrich syndrome 

Abbreviations

WAS

Wiskott–Aldrich syndrome

IgAN

IgA nephropathy

XLT

X-linked thrombocytopenia

WASP

WAS protein

MPGN

Membranoproliferative glomerulonephritis

ESRD

End-stage renal disease

Copyright information

© Springer Science+Business Media, B.V. 2012

Authors and Affiliations

  • Chia-Hung Liu
    • 1
    • 2
  • Kang-Hsi Wu
    • 2
    • 3
  • Tze-Yi Lin
    • 3
    • 4
  • Chang-Ching Wei
    • 2
    • 3
  • Ching-Yuang Lin
    • 2
    • 3
  • Xian-Xiu Chen
    • 2
    • 3
  • Wen-I Lee
    • 5
  1. 1.Department of PaediatricsTaichung Armed Forces General HospitalTaichungTaiwan, ROC
  2. 2.Division of Nephrology, Department of PaediatricsChina Medical University HospitalTaichungTaiwan, ROC
  3. 3.China Medical UniversityTaichungTaiwan, ROC
  4. 4.Department of PathologyChina Medical University HospitalTaichungTaiwan, ROC
  5. 5.Department of Paediatric Allergy, Immunology and Rheumatology, Primary Immunodeficiency Care And Research (PICAR) Institute, Chang Gung Children’s and Memorial HospitalChang Gung University College of MedicineTaoyuanTaiwan, ROC

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