[alpha1-antitrypsin deficiency. Clinical and morphological aspects during childhood (author's transl)]

In many cases so called neonatal hepatitis of unknown origin nowadays is recognized as a manifestation of alpha1-antitrypsin deficiency. Out of 12 patients with Pi-type ZZ, 5 were diagnosed because of cholestatic jaundice, 2 because of hepato-splenomegaly in the first trimenon, and 3 by family examination. We believe that the affection may be due to a perinataly acquired cytomegalic inclusion disease in one case, in another to a congenital rubella infection. The latter child died at the age of one year because of an esophageal hemorrhage. Over a mean observation time of 3 years the other patients are doing well and show no signs of portal hypertension. The very different course of the hepatopathy is demonstrated. Common bacteria or toxins which do not usually lead to an illness may be realisation factors just as "classical" causes of hepatitis. Up to now it is not known how these factors influence the course of alpha-antitrypsin deficiency.