ALPHA-1-ANTITRYPSIN DEFICIENCY ASSOCIATED WITH HEPATIC CIRRHOSIS AND IGA NEPHRITIS

The association between alpha(1)-antitrypsin (A1AT) deficiency and glo merulonephritis has only sporadically been reported, and mostly based upon autopsy findings, as opposed to the more frequent linkage between A1AT deficiency and lung emphysema with or without hepatic cirrhosis. The present case report describes a 30-year-old man with A1AT deficie ncy, without evidence of lung disease, who developed hepatic cirrhosis in early childhood and IgA glomerulonephritis and hypertension in adu lt life. The IgA nephritis followed an unusual course, with a sudden d eterioration of the renal function, possibly induced by uncontrolled h ypertension or the possible occurrence of vasculitis. After 6 months o f hemodialysis, the patient successfully underwent living-related-dono r kidney transplantation.