Alpha 1antitrypsin deficiency causes
Refer Robbins page no 851 Clinical Features. Neonatal hepatitis with cholestatic jaundice appears in 10% to 20% of newborns with the defi- ciency. In adolescence, presenting symptoms may be related to hepatitis, cirrhosis or pulmonary disease. Attacks of hepatitis may subside with apparent complete recovery, or they may become chronic and lead progressively to cir- rhosis. Alternatively, the disease may remain silent until cirrhosis appears in middle to later life. Hepatocellular carcinoma develops in 2% to 3% of PiZZ adults, usually, but not always, in the setting of cirrhosis. The treatment, indeed the cure, for severe hepatic disease is ohotopic liver transplantation. In patients with pulmonary disease the single most impoant preventive measure is avoidance of cigarette smoking, because smoking markedly acceler- ates emphysema and the destructive lung disease associ- ated with a1AT deficiency
Congenital cystic fibrosis
Neonatal hepatitis
Pulmonary fibrosis
All of the above
miscellaneous