NATURAL HISTORY OF CHILDREN WITH ALPHA1‐ANTITRYPSIN DEFICIENCY AND NEONATAL CHOLESTASIS

Publisher Website
Google Scholar
Abstract
Ten children with .alpha.1-antitrypsin deficiency, phenotype PiZ and neonatal cholestasis were followed to the ages of 4-20 yr. Standard liver function tests, urinary bile acid excretion and liver morphology were investigated within 1 wk and the results were compared. Three patients had morphological signs of liver cirrhosis and 1 of these had clinical signs as well. Compared to healthy children of the same age, urinary bile acid excretion was increased only in those with morphologically confirmed liver cirrhosis. Although most of the other patients had some minor histological changes in the liver biopsies, the normal urinary bile acid excretion was in accordance with their excellent general clinical state and is interpreted as a short-term favorable prognostic sign.