Orthotopic liver transplantation for alpha-1-antitrypsin deficiency: an experience in 29 children and ten adults. Transplantation proceedings Esquivel, C. O., Vicente, E., Van Thiel, D., Gordon, R., Marsh, W., Makowka, L., Koneru, B., Iwatsuki, S., Madrigal, M., Delgado Millan, M. A. 1987; 19 (5): 3798-3802


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.

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