To the Editor.—The optimism expressed by Talamo and Feingold1 regarding the prognosis in children with hepatocellular disease and Pi ZZ α1-antitrypsin must be tempered with a note of caution. As Alper and I reported previously,2 many of these children do improve clinically after the initial, neonatal illness. In fact, over half of the patients we have observed have had "remissions" of months to years, although most have had minimal biochemical abnormalities, slight hepatosplenomegaly, or both during much if not all of these quiescent periods. The patients described by Talamo and Feingold would appear to be similar.
The few liver biopsies performed during the asymptomatic stage have shown varying degrees of hepatitic activity,3 with progressive portal cirrhosis. None of our posthepatitic patients with the Pi ZZ phenotype has escaped severe and progressive cirrhosis later in childhood or in early adulthood. The quiescent period would appear
JOHNSON AM. Alpha1-Antitrypsin Deficiency. Am J Dis Child. 1973;126(6):861. doi:10.1001/archpedi.1973.02110190703030