title: ABO-incompatible renal transplantation in Epstein syndrome.
abstract: Epstein syndrome (ES) is an autosomal dominant hereditary disease characterized by hereditary nephritis, sensory deafness, and thrombocytopenia. We herein report the case of a 20-yr-old man with ES who underwent ABO blood type-incompatible living-donor kidney transplantation from his mother. He was given platelet transfusion, and his pre-operative number of platelets were 108 x 10(3)/microL. After transplantation, urine output and the decrease in serum creatinine (sCr) were within the acceptable ranges. On the seventh post-operative day (POD), sCr had risen and urine output decreased. Anti-type A antibody rapidly elevated from <2 times (x2) just before transplantation to 64 times (x64), and the patient required hemodialysis again. Resistance index (RI) by ultrasound increased from an average of 0.5 approximately 0.6 on POD 1 to an average of 0.7 approximately 0.8 on POD 7. However, several biopsies (POD 4, 7, and 10) showed no obvious findings of acute rejection except for intense C4d deposition. Because acute antibody-mediated rejection was not completely ruled out, he was treated with methyl-prednisolone pulse therapy, plasma exchange, cyclophosphamide, and immunoglobulin. Regardless, his titer of anti-type A antibody was still high, and he still presented oliguria. We performed an emergent splenectomy. Consequently, the levels of anti-type A antibody decreased, the RI also dropped to an average of 0.6. However, on POD 19 and 25 (platelets were 27 x 10(3)/microL and 36 x 10(3)/microL), he developed a massive intraperitoneal hematoma around the graft and region of the removed spleen, which pushed the graft out and caused acute tubular necrosis, resulting in anuria. The RI rose to an average of 0.8 approximately 1.0 after these episodes. He also experienced bleeding from a duodenal ulcer on POD 21. However, his renal function has fully recovered after acute hemodialysis for 35 d. The latest sCr was 1.5 mg/dL with a recovery in RI to 0.6. Although his platelet count was maintained at a minimum of 50 x 10(3)/microL, he had several severe bleeding episodes, concluding that sufficient platelets are necessary after transplantation in ES.
authors.affiliation: Department of Pediatric Nephrology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
authors.forename: Masao
authors.initials: M
authors.lastname: Ogura
authors.forename: Eriko
authors.initials: E
authors.lastname: Kikuchi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kaito
authors.forename: Koichi
authors.initials: K
authors.lastname: Kamei
authors.forename: Kentaro
authors.initials: K
authors.lastname: Matsuoka
authors.forename: Hideaki
authors.initials: H
authors.lastname: Tanaka
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.forename: Takashi
authors.initials: T
authors.lastname: Sekine
authors.forename: Shuichi
authors.initials: S
authors.lastname: Ito
mesh_terms: D000017:ABO Blood-Group System / Q000276:immunology*; D000328:Adult; D001787:Blood Group Incompatibility / Q000276:immunology*; D006319:Hearing Loss, Sensorineural / Q000276:immunology* / Q000628:therapy; D006801:Humans; D016030:Kidney Transplantation / Q000276:immunology* / Q000473:pathology; D019520:Living Donors; D008297:Male; D009394:Nephritis, Hereditary / Q000276:immunology* / Q000601:surgery; D010956:Plasmapheresis; D013577:Syndrome; D013921:Thrombocytopenia / Q000276:immunology* / Q000628:therapy; D055815:Young Adult
chemical_list: D000017:ABO Blood-Group System