title: Feasibility of auxiliary partial orthotopic liver transplantation from living donors for patients with adult-onset type II citrullinemia.
abstract: More than 20 patients with adult-onset type II citrullinemia have undergone liver transplantation, showing dramatic therapeutic effects. In Japan, living donor liver transplantation is the standard technique of liver transplantation because of the rare availability of cadaveric donors. The feasibility of auxiliary partial orthotopic liver transplantation (APOLT) for adult-onset type II citrullinemia to overcome the problem of a small-for-size graft in living donor liver transplantation has not been defined. We recently performed APOLT for patients with type II citrullinemia. Here, we present 2 patients: patient 1 was a 32-year-old man and patient 2 was a 43-year-old woman. Both patients suffered from hepatic encephalopathy, and laboratory data showed highly elevated plasma levels of ammonia and citrulline. In patient 1, the liver graft was obtained from a patient with familial amyloid polyneuropathy as a domino liver transplant. In patient 2, APOLT was performed after graft donation from her husband. The postoperative clinical courses of both patients were uneventful, and the neurological symptoms were completely resolved. The plasma concentrations of ammonia and citrulline normalized rapidly in both patients. APOLT can provide an adequate hepatocyte mass to correct the underlying enzyme deficiency in adult patients with type II citrullinemia. In addition, APOLT can be carried out safely to overcome the limitation of graft volume in living donor liver transplantation.
authors.affiliation: Third Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Masahide
authors.initials: M
authors.lastname: Yazaki
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Hashikura
authors.forename: Yo-ichi
authors.initials: Y
authors.lastname: Takei
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Ikegami
authors.forename: Shin-ichi
authors.initials: S
authors.lastname: Miyagawa
authors.forename: Kanji
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Takahiko
authors.initials: T
authors.lastname: Tokuda
authors.forename: Keiko
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Takeyori
authors.initials: T
authors.lastname: Saheki
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000328:Adult; D020159:Citrullinemia / Q000601:surgery*; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D016896:Treatment Outcome