title: Successful Long-term Graft Survival of a Renal Transplantation Patient with Wiskott-Aldrich Syndrome.
abstract: Wiskott-Aldrich syndrome, a rare X-linked hereditary syndrome, is characterized by immunodeficiency, thrombocytopenia, and eczema. The underlying T-cell defect renders renal transplantation and immunosuppressive treatments uncertain. The present case exhibited the mild clinical manifestation, regarded as X-linked thrombocytopenia. He successfully underwent a living-donor ABO-compatible renal transplantation and splenectomy in 2002, and thereafter experiencing no severe rejection, serious infection, or malignancy for more than 10 years. Though IgA nephropathy was detected 8 months after transplantation, the patient's renal function and proteinuria were stable without any treatment. The present case showed a successful long-term graft survival and the importance of splenectomy added to renal transplantation.
authors.affiliation: Department of Surgery, Kidney Center, Tokyo Women's Medical University, Japan.
authors.forename: Kotaro
authors.initials: K
authors.lastname: Kai
authors.forename: Maki
authors.initials: M
authors.lastname: Sumida
authors.forename: Yaeko
authors.initials: Y
authors.lastname: Motoyoshi
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Ogawa
authors.forename: Katsuyuki
authors.initials: K
authors.lastname: Miki
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Iwadoh
authors.forename: Akihito
authors.initials: A
authors.lastname: Sannomiya
authors.forename: Toru
authors.initials: T
authors.lastname: Murakami
authors.forename: Ichiro
authors.initials: I
authors.lastname: Koyama
authors.forename: Kumiko
authors.initials: K
authors.lastname: Kitajima
authors.forename: Ichiro
authors.initials: I
authors.lastname: Nakajima
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Morio
authors.forename: Shohei
authors.initials: S
authors.lastname: Fuchinoue
mesh_terms: D000328:Adult; D005922:Glomerulonephritis, IGA / Q000503:physiopathology; D006085:Graft Survival; D006801:Humans; D007677:Kidney Function Tests; D016030:Kidney Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D013156:Splenectomy; D014923:Wiskott-Aldrich Syndrome / Q000453:epidemiology*