title: Living donor liver transplantation in children with cholestatic liver disease: a single-center experience.
abstract: OBJECTIVE
  Cholestatic liver disease (CLD) is the main indication for liver transplantation in children. This retrospective study evaluated the outcomes of living donor liver transplantation (LDLT) in children with CLD.
  
  
  METHODS
  One hundred fifty-nine children with CLD who underwent 164 LDLT between May 2001 and May 2011 were evaluated. Their original diseases were biliary atresia (n=145, 91%), Alagille syndrome (n=8, 5%), primary sclerosing cholangitis (n=2), and the others (n=4). The mean age and body weight of the recipients at LDLT was 42±53 months and 14.0±11.0 kg, respectively.
  
  
  RESULTS
  Parents were living donors in 98%. The left lateral segment was the most common type of graft (77%). There were no reoperations and no mortality in any living donor. Recipients' postoperative surgical complications consisted mainly of hepatic arterial problems (7%), hepatic vein stenosis (5%), portal vein stenosis (13%), biliary stricture (18%), intestinal perforation (3%). The overall rejection rate was 31%. Cytomegalovirus infection and Epstein-Barr virus disease were observed in 26% and 5%, respectively. Retransplantation was performed five times in four patients; the main cause was hepatic vein stenosis (n=3). Four patients died; the main cause was gastrointestinal perforation (n=2). The body height of Alagille syndrome patients less than 2 years old significantly improved compared with older patients after LDLT. The 1-, 5-, and 10-year patient survival rates were 98%, 97%, and 97%, respectively.
  
  
  CONCLUSIONS
  LDLT for CLD is an effective treatment with excellent long-term outcomes.
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Oyama Municipal Hospital, Tochigi, Japan. Koimizu@jichi.ac.jp
authors.forename: K
authors.initials: K
authors.lastname: Mizuta
authors.forename: T
authors.initials: T
authors.lastname: Urahashi
authors.forename: Y
authors.initials: Y
authors.lastname: Ihara
authors.forename: Y
authors.initials: Y
authors.lastname: Sanada
authors.forename: T
authors.initials: T
authors.lastname: Wakiya
authors.forename: N
authors.initials: N
authors.lastname: Yamada
authors.forename: N
authors.initials: N
authors.lastname: Okada
authors.forename: S
authors.initials: S
authors.lastname: Egami
authors.forename: S
authors.initials: S
authors.lastname: Hishikawa
authors.forename: M
authors.initials: M
authors.lastname: Hyodo
authors.forename: Y
authors.initials: Y
authors.lastname: Sakuma
authors.forename: T
authors.initials: T
authors.lastname: Fujiwara
authors.forename: H
authors.initials: H
authors.lastname: Kawarasaki
authors.forename: Y
authors.initials: Y
authors.lastname: Yasuda
mesh_terms: D000367:Age Factors; D016738:Alagille Syndrome / Q000401:mortality / Q000601:surgery*; D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000401:mortality / Q000601:surgery*; D005260:Female; D006084:Graft Rejection / Q000209:etiology / Q000601:surgery; D006498:Hepatectomy / Q000009:adverse effects / Q000401:mortality; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D019520:Living Donors; D008297:Male; D011183:Postoperative Complications / Q000209:etiology / Q000601:surgery; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome