Orthotopic liver transplantation for alpha-1-antitrypsin deficiency: an experience in 29 children and ten adults.
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.
Esquivel
C O
CO
Department of Surgery, University Health Center of Pittsburgh, University of Pittsburgh, PA.
Vicente
E
E
Van Thiel
D
D
Gordon
R
R
Marsh
W
W
Makowka
L
L
Koneru
B
B
Iwatsuki
S
S
Madrigal
M
M
Delgado Millan
M A
MA
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
D000515:alpha 1-Antitrypsin