Literature DB >> 34304384

A case of early recurrent immunoglobulin A nephropathy and T-cell-mediated rejection in a transplant patient with Wiskott-Aldrich syndrome.

Kosei Yamaguchi1,2, Mineaki Kitamura3, Yuki Kawaguchi2, Kanako Hayashi2, Kumiko Muta2, Masayuki Nakazawa2, Tsuyoshi Matsuda4,5, Toru Onita4, Masaharu Nishikido4, Hideki Sakai5, Hiroshi Mukae6, Tomoya Nishino2.   

Abstract

Wiskott-Aldrich syndrome (WAS) is an X-chromosome recessive immunodeficiency disease characterized by the triad of thrombocytopenia, eczema, and susceptibility to infection owing to WAS protein gene abnormalities. Kidney transplantation is rarely offered to WAS patients with end-stage renal disease because of concerns that thrombocytopenia and immune disorders may affect the clinical outcome. Here, we report the case of a 20-year-old kidney transplant patient who developed end-stage renal disease owing to immunoglobulin (Ig)A nephropathy caused by WAS. Despite recurrent IgA nephropathy and T-cell-mediated rejection 7 months after transplantation, two rounds of steroid pulse therapy attenuated his renal function and urinary abnormality. His serum creatinine level was maintained at approximately 1.5 mg/dL 1 year after transplantation. No other WAS-related complications were observed throughout the clinical course. Although WAS can cause poor prognosis in kidney transplant patients, careful follow-up may allow kidney transplantation to be performed.
© 2021. Japanese Society of Nephrology.

Entities:  

Keywords:  IgA nephropathy; Kidney transplantation; T-cell-mediated rejection; Wiskott–Aldrich syndrome

Mesh:

Year:  2021        PMID: 34304384      PMCID: PMC8810969          DOI: 10.1007/s13730-021-00631-9

Source DB:  PubMed          Journal:  CEN Case Rep        ISSN: 2192-4449


  2 in total

1.  Renal transplantation in Wiskott-Aldrich syndrome.

Authors:  M C Webb; P A Andrews; C G Koffman; J S Cameron
Journal:  Transplantation       Date:  1993-12       Impact factor: 4.939

2.  IgA Nephropathy Complicated with X-linked Thrombocytopenia.

Authors:  Yuki Kakio; Haruhito Adam Uchida; Masashi Kitagawa; Yuka Arata; Ayako Kato; Akiko Inoue-Torii; Norikazu Hinamoto; Ayu Ogawa-Akiyama; Hitoshi Sugiyama; Jun Wada
Journal:  Acta Med Okayama       Date:  2018-06       Impact factor: 0.892

  2 in total

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