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

  • Nephrology – Case Report
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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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Fig. 1
Fig. 2

Abbreviations

WAS:

Wiskott–Aldrich syndrome

IgAN:

IgA nephropathy

XLT:

X-linked thrombocytopenia

WASP:

WAS protein

MPGN:

Membranoproliferative glomerulonephritis

ESRD:

End-stage renal disease

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Acknowledgments

Grants NSC99-2314-B-182-003-MY3 provided the financial support needed to perform DNA sequencing. This study is also partly funded by grants from China Medical University Hospital (DM-100-050).

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None.

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Authors and Affiliations

  1. Department of Paediatrics, Taichung Armed Forces General Hospital, Taichung, Taiwan, ROC

    Chia-Hung Liu

  2. Division of Nephrology, Department of Paediatrics, China Medical University Hospital, #2, Yuh-Der Road, Taichung, 40402, Taiwan, ROC

    Chia-Hung Liu, Kang-Hsi Wu, Chang-Ching Wei, Ching-Yuang Lin & Xian-Xiu Chen

  3. China Medical University, Taichung, Taiwan, ROC

    Kang-Hsi Wu, Tze-Yi Lin, Chang-Ching Wei, Ching-Yuang Lin & Xian-Xiu Chen

  4. Department of Pathology, China Medical University Hospital, Taichung, Taiwan, ROC

    Tze-Yi Lin

  5. Department of Paediatric Allergy, Immunology and Rheumatology, Primary Immunodeficiency Care And Research (PICAR) Institute, Chang Gung Children’s and Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan, ROC

    Wen-I Lee

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  1. Chia-Hung Liu
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  2. Kang-Hsi Wu
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  3. Tze-Yi Lin
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  4. Chang-Ching Wei
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Corresponding author

Correspondence to Chang-Ching Wei.

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Liu, CH., Wu, KH., Lin, TY. et al. Wiskott–Aldrich syndrome with IgA nephropathy: a case report and literature review. Int Urol Nephrol 45, 1495–1500 (2013). https://doi.org/10.1007/s11255-012-0178-0

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  • DOI: https://doi.org/10.1007/s11255-012-0178-0

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