ALPHA-1-ANTITRYPSIN DEFICIENCY IN CHILDREN

Hereditary deficiency of the alpha-1-antitrypsin occupies a leading position among the causes of chronic nonspecific lung diseases with emphysema formation. Deficiency of alpha-1-antitrypsin is a potentially fatal hereditary disease, under-diagnosed by physicians of various specialities. The authors familiarize pediatricians with this hereditary disease, difficulties of its diagnosis and treatment. There are presented own author`s data on the diagnosis and the experience of the enzyme replacement therapy of alpha-1-antitrypsin deficiency with the drug INN «Alpha-1 antitrypsin human» (Respikam)