| Literature DB >> 34304384 |
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.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