THE LIVER IN ADOLESCENTS WITH ALPHA(1)-ANTITRYPSIN DEFICIENCY

Of 200,000 Swedish infants screened for alpha(1)-antitrypsin deficienc y (alpha(1)ATD), 184 (127 PiZ, 2 PiZ-, 54 PiSZ, and 1 PiS-) children h ave been followed prospectively, of whom 1 PiSZ and 5 PiZ children die d in early childhood. We now report clinical and biochemical signs of liver disease in adolescence and the prognosis of neonatal liver disea se up to the age of 18 years. The alpha(1)ATD subjects were offered a clinical checkup and Liver tests at 16 and 18 years of age, 150 of 178 alpha(1)ATD subjects undergoing checkups at age 16 and 166 at age 18. Liver tests were performed in 121 adolescents at both the 16- and 18- year checkups. None of the PiZ and PiSZ subjects checked at the age of 16 and 18 years had any clinical signs of liver disease, Abnormalitie s of serum alanine aminotransferase (S-ALAT) or gamma-glutamyl transfe rase (S-GT) were found at the 18-year checkup (all PiZ and PiSZ subjec ts tested included) in 17% of PiZ and 8% of PiSZ adolescents, and at t he age of 18 years in 12% of PiZ and 15% of PiSZ subjects. In only two cases were both S-ALAT and S-GT concentrations abnormal at both the 1 6-year and 18-year follow-ups. Serum procollagen Ill peptide concentra tions were normal in all those with abnormal liver test results. Of 12 7 PiZ subjects, 22 had manifested clinical signs of liver disease in i nfancy, Of these 22, two died early in life of cirrhosis. Another two children died of other causes; however, autopsy showed histological si gns of cirrhosis in one of them, fibrosis in the other. Ah of the rema ining 18 subjects were clinically healthy at the 16- and 18-year check ups. Marginal liver test abnormalities were found in two of them. To s um up, alpha(1)ATD children followed prospectively up to 18 years of a ge continue to have favorable prognosis.