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Diagnostic, preventive, medical and surgical management of alpha 1 ‐antitrypsin deficiency in childhood
Author(s) -
Burdelski M
Publication year - 1994
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1994.tb13205.x
Subject(s) - medicine , alpha 1 antitrypsin deficiency , liver transplantation , disease , transplantation , intensive care medicine , pathophysiology , liver disease , metabolic disorder , medical therapy , chronic liver disease , pediatrics , cirrhosis
Only 10 years ago the management of alpha 1 ‐antitrypsin deficiency (AATD) as one of the most frequent hepatic‐based metabolic disorders in children had been restricted to diagnostic and palliative therapeutic aspects (1). After liver transplantation became an optional curative therapy in patients suffering from end‐stage liver disease (2), the transplantation of chronic end‐stage liver disease caused by AATD has extended to one of the most frequent indications in hepatic‐based metabolic disorders. Recently, new pathophysiological concepts of the development of liver damage in AATD have enabled us to examine optimized prevention therapy in this disorder (3, 4). This paper attempts to show how optimized therapy of affected children may be achieved.