Liver Transplantation for Advanced Liver Disease with Alpha-1antitrypsin Deficiency

ALPHA-1-antitrypsin deficiency associated with chronic obstructive airway disease was recognized in 1963 by Laurell and Ericksson. 1 In 1969, Sharp 2 described the first cases of alpha-1-antitrypsin-deficiency disease in children with cirrhosis. Since then, this inborn error has been recognized as o...

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Bibliographic Details
Published inThe New England journal of medicine Vol. 302; no. 5; pp. 272 - 275
Main Authors Hood, John M, Koep, Lawrence J, Peters, Robert L, Schröter, Gerhard P. J, Weil, Richard, Redeker, Allan G, Starzl, Thomas E
Format Journal Article
LanguageEnglish
Published Boston Massachusetts Medical Society 31.01.1980
Subjects
Case reports
Ehlers-Danlos syndrome
Genotype & phenotype
Health sciences
Hepatitis
Liver cirrhosis
Liver diseases
Liver transplantation
Liver transplants
Lung diseases
Patients
Pediatrics
Transplantation
Transplants & implants
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Summary:ALPHA-1-antitrypsin deficiency associated with chronic obstructive airway disease was recognized in 1963 by Laurell and Ericksson. 1 In 1969, Sharp 2 described the first cases of alpha-1-antitrypsin-deficiency disease in children with cirrhosis. Since then, this inborn error has been recognized as one of the more common factors in cirrhosis of infancy and childhood, 3 including "neonatal hepatitis." 4 Alpha-1-antitrypsin is a glycoprotein that accounts for a major portion of the alpha-1 globulin fraction of the serum. 5 It is responsible for approximately 90 per cent of the antitrypsin activity 6 of the serum, and it also inhibits several other plasma enzymes, including plasmin, 7 elastase, 8 collagenase, 9 and . . .
ISSN:0028-4793
1533-4406
DOI:10.1056/NEJM198001313020505