Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare hereditary, metabolic disease characterized by serum levels of alpha-1-antitrypsin (AAT) that are well below the normal range. In the most severe form, the disease can clinically manifest with chronic liver disorders (cirrhosis, fibrosis), respiratory disorders (emphysema, bronchiectasis), and rarely panniculitis or vasculitis. Disease data Classification Disease Synonyms Alpha-1-proteinase inhibitor deficiency Niedobór inhibitora Alfa-1-proteinazy Alpha1-antitrypsin deficiency ORPHA code 60 OMIM code 613490 ICD10 code E88.0 ICD11 code 5C5A *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl