title: Orthotopic liver transplantation for alpha-1-antitrypsin deficiency: an experience in 29 children and ten adults.
abstract: Thirty-nine patients (29 children and ten adults) underwent OLT for liver disease associated with A1AD from March 1980 to March 1986. Thirty of thirty-six patients (83%) with available data were homozygous phenotype PiZZ. The other six were Pi heterozygotes, being either PiMZ or PiSZ. The mean A1A activity in homozygous and heterozygous patients was 38.8 mg/dL and 114.3 mg/dL respectively. Eight patients died during the first 3 months after OLT (20%). The 5-year actuarial survival is 83% and 60% in pediatric and adult recipients respectively. Today 30 (76%) of the recipients are alive, with follow-ups of 8 to 64 months (average 27 months). The quality of life in the surviving patients is excellent.
authors.affiliation: Department of Surgery, University Health Center of Pittsburgh, University of Pittsburgh, PA.
authors.forename: C O
authors.initials: CO
authors.lastname: Esquivel
authors.forename: E
authors.initials: E
authors.lastname: Vicente
authors.forename: D
authors.initials: D
authors.lastname: Van Thiel
authors.forename: R
authors.initials: R
authors.lastname: Gordon
authors.forename: W
authors.initials: W
authors.lastname: Marsh
authors.forename: L
authors.initials: L
authors.lastname: Makowka
authors.forename: B
authors.initials: B
authors.lastname: Koneru
authors.forename: S
authors.initials: S
authors.lastname: Iwatsuki
authors.forename: M
authors.initials: M
authors.lastname: Madrigal
authors.forename: M A
authors.initials: MA
authors.lastname: Delgado Millan
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005838:Genotype; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D011183:Postoperative Complications / Q000453:epidemiology; D000515:alpha 1-Antitrypsin / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency
chemical_list: D000515:alpha 1-Antitrypsin