title: Sequential liver-kidney transplantation in a boy with congenital hepatic fibrosis and nephronophthisis from a living donor.
abstract: A five-yr-old boy developed chronic liver failure and ESKD because of CHF and juvenile NPHP. He underwent sequential liver and kidney transplantation with a compatible blood type from his father, at five yr, seven months and five yr, 11 months old, respectively. Because the patient was not in ESKD, we initially performed LDLT because of significant portal hypertension. Even after LDLT, his ascites was not ameliorated, and he needed continuous drainage of ascites and daily albumin and gamma globulin infusion. Thereafter, he progressed to ESKD and needed hemodialysis for one month before LDKT. CDC crossmatch for donor B cells in the warm test, FCXM for B cell IgG, and flow PRA for donor class II were positive before LDKT. After pretreatment of three courses of plasma exchange and intravenous gamma globulin, LDKT was performed. Two weeks after LDKT, AIHA concomitant with autoimmune thrombocytopenia, also called Evans syndrome, occurred because of passenger lymphocytes from the donor; however, the patient was successfully treated with intravenous methylprednisolone. Eighteen months have passed since LDKT, and liver and kidney function in both the recipient and donor are normal.
authors: Division of Nephrology, National Center for Child Health and Development, Tokyo, Japan. tudagawa.ped@tmd.ac.jp
authors: Tomohiro
authors: T
authors: Udagawa
authors: Koichi
authors: K
authors: Kamei
authors: Masao
authors: M
authors: Ogura
authors: Akiko
authors: A
authors: Tsutsumi
authors: Shunsuke
authors: S
authors: Noda
authors: Mureo
authors: M
authors: Kasahara
authors: Akinari
authors: A
authors: Fukuda
authors: Seisuke
authors: S
authors: Sakamoto
authors: Shigenobu
authors: S
authors: Shigeta
authors: Hideaki
authors: H
authors: Tanaka
authors: Tatsuo
authors: T
authors: Kuroda
authors: Kentarou
authors: K
authors: Matsuoka
authors: Atuko
authors: A
authors: Nakazawa
authors: Takuto
authors: T
authors: Nagai
authors: Osamu
authors: O
authors: Uemura
authors: Shuichi
authors: S
authors: Ito
mesh_terms: D002675:Child, Preschool; D006801:Humans; D006975:Hypertension, Portal / Q000503:physiopathology; D007074:Immunoglobulin G / Q000378:metabolism; D052177:Kidney Diseases, Cystic / Q000473:pathology*; D016030:Kidney Transplantation / Q000379:methods*; D008103:Liver Cirrhosis / Q000151:congenital / Q000473:pathology*; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008214:Lymphocytes / Q000166:cytology; D008297:Male; D010951:Plasma Exchange; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology / Q000628:therapy; D006435:Renal Dialysis; D016896:Treatment Outcome
chemical_list: D007074:Immunoglobulin G