title: The optimal immunosuppressive protocol for the portal vein infusion of PGE1 and methylprednisolone in pediatric liver transplantation for fulminant hepatic failure of unknown etiology.
abstract: The outcome of LTx in pediatric patients with FHF of unknown etiology remains inferior to that of LTx in pediatric patients with cholestatic diseases. A higher incidence of steroid-resistant severe rejection has been increasingly recognized among the responsible factors. We assessed the efficacy of the administration of steroids and PGE1 via PVI in the management of LTx for FHF in pediatric patients. In our early cohort (1995-2007), seven patients who underwent LTx for FHF of unknown etiology were treated with conventional immunosuppressive therapy (calcineurin inhibitor and a steroid). Seven of eight grafts (one patient underwent re-LTx) sustained CV and/or CPV associated with ACR, and four patients died of a graft failure or infectious complications that were associated with the treatment for rejection. Of note, the pathological incidence of CV/CPV was significantly higher in recipients with FHF of unknown etiology than in recipients with biliary cholestatic disease during the same study period (87.5% vs. 13.7%, p < 0.00001). From 2008, three patients underwent LTx for cryptogenic FHF with PVI and conventional IS. PVI was well tolerated, and no relevant severe complications were observed. More strikingly, the patients who received PVI overcame biopsy-proven immunological events and are all currently doing well with excellent graft function after more than five yr. We conclude that PVI is technically safe and effective for preventing severe rejection in pediatric patients who undergo LTx for FHF of unknown etiology and that it does not increase the risk of fatal infectious complications.
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Yamada
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Ken
authors.initials: K
authors.lastname: Hoshino
authors.affiliation: Department of Pathology, Kawasaki Municipal Hospital, Kanagawa, Japan.
authors.forename: Rie
authors.initials: R
authors.lastname: Irie
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Hirofumi
authors.identifier: 0000-0002-0283-8240
authors.initials: H
authors.lastname: Tomita
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Mototoshi
authors.initials: M
authors.lastname: Kato
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Shimojima
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Fujino
authors.affiliation: Department of General Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Taizo
authors.initials: T
authors.lastname: Hibi
authors.affiliation: Department of General Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Shinoda
authors.affiliation: Department of General Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Obara
authors.affiliation: Department of General Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Osamu
authors.initials: O
authors.lastname: Itano
authors.affiliation: Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, Tokyo, Japan.
authors.forename: Shigeyuki
authors.initials: S
authors.lastname: Kawachi
authors.affiliation: Department of Hepatobiliary and Pancreatic Surgery, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Minoru
authors.initials: M
authors.lastname: Tanabe
authors.affiliation: Department of Pathology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Michiie
authors.initials: M
authors.lastname: Sakamoto
authors.affiliation: Department of General Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Kitagawa
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
mesh_terms: D000293:Adolescent; D000527:Alprostadil / Q000008:administration & dosage* / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006084:Graft Rejection / Q000517:prevention & control*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D007223:Infant; D007262:Infusions, Intravenous; D017114:Liver Failure, Acute / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage* / Q000627:therapeutic use; D011169:Portal Vein; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D000527:Alprostadil; D008775:Methylprednisolone
keywords: central perivenulitis; central venulitis; fulminant hepatic failure; pediatric liver transplantation; portal vein infusion