Sequential liver-kidney transplantation in a boy with congenital hepatic fibrosis and nephronophthisis from a living donor

Tomohiro Udagawa, Koichi Kamei, Masao Ogura, Akiko Tsutsumi, Shunsuke Noda, Mureo Kasahara, Akinari Fukuda, Seisuke Sakamoto, Shigenobu Shigeta, Hideaki Tanaka, Tatsuo Kuroda, Kentarou Matsuoka, Atuko Nakazawa, Takuto Nagai, Osamu Uemura, Shuichi Ito

Research output: Contribution to journalArticlepeer-review

11 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)E275-E280
JournalPediatric Transplantation
Issue number7
Publication statusPublished - 2012 Nov
Externally publishedYes


  • Evans syndrome
  • congenital hepatic fibrosis
  • living donor kidney transplant
  • living donor liver transplantation
  • nephronophthisis
  • sequential liver-kidney transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation


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