title: Living related donor liver transplantation in children.
abstract: OBJECTIVE
  The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors.
  
  
  METHODS
  We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010.
  
  
  RESULTS
  Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3). The age of the recipients ranged from 7-174 months (median, 22) and the weights ranged from 6-58 kg (median, 10). Forty-nine children (40.5%) weighed ≤10 kg. The grafts included the left lateral segment (n = 108), the left lobe (n = 12), and the right lobe (n = 1). The donors included 71 mothers, 45 fathers, 2 uncles, 1 grandmother, 1 grandfather, and 1 sister with a median age of 29 years (range, 16-53 ys) and a median weight of 68 kg (range, 47-106). Sixteen patients (12.9%) required retransplantation, most commonly due to hepatic artery thrombosis (HAT; n = 13; 10.7%). The other complications were biliary stenosis (n = 25; 20.6%), portal vein thrombosis (PVT; n = 11; 9.1%), portal vein stenosis (n = 5; 4.1%), hepatic vein stenosis (n = 6; 4.9%), and lymphoproliferative disorders (n = 8; 6.6%). The ultimate survival rate of recipients was 90.3% after 1 year and 75.8% after 3 years. Causes of early death within 1 month were HAT (n = 6), PVT (n = 2), severe graft dysfunction (n = 1), sepsis (n = 1), and intraoperative death in children with acute liver failure (n = 2). Causes of late deaths included lymphoproliferative disease (n = 3), chronic rejection (n = 2), biliary complications (n = 3), and recurrent disease (n = 3; hepatoblastoma and primary sclerosing cholangitis).
  
  
  CONCLUSIONS
  Despite the heightened possibility of complications (mainly vascular), LRDLT represented a good alternative to transplantation from cadaveric donors in pediatric populations. It was associated with a high survival ratio.
authors.affiliation: Department of Pediatrics, Pediatric Surgery and Liver Transplantation Division, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: A C A
authors.initials: AC
authors.lastname: Tannuri
authors.forename: N E M
authors.initials: NE
authors.lastname: Gibelli
authors.forename: L R S
authors.initials: LR
authors.lastname: Ricardi
authors.forename: M M
authors.initials: MM
authors.lastname: Santos
authors.forename: J G
authors.initials: JG
authors.lastname: Maksoud-Filho
authors.forename: M L
authors.initials: ML
authors.lastname: Pinho-Apezzato
authors.forename: M M
authors.initials: MM
authors.lastname: Silva
authors.forename: M C P
authors.initials: MC
authors.lastname: Velhote
authors.forename: A A R
authors.initials: AA
authors.lastname: Ayoub
authors.forename: W C
authors.initials: WC
authors.lastname: Andrade
authors.forename: A J
authors.initials: AJ
authors.lastname: Leal
authors.forename: H T
authors.initials: HT
authors.lastname: Miyatani
authors.forename: U
authors.initials: U
authors.lastname: Tannuri
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005190:Family; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
chemical_list: D007166:Immunosuppressive Agents