HEPATIC MORPHOLOGY AND IRON QUANTITATION IN PERINATAL HEMOCHROMATOSIS- COMPARISON WITH A LARGE PERINATAL CONTROL POPULATION, INCLUDING CASES WITH CHRONIC LIVER-DISEASE

We compared hepatic morphology, hepatocellular siderosis, extrahepatic parenchymal siderosis, and (by chemical assay of liver and spleen) th e amount of elemental iron and copper in 12 cases of perinatal hemochr omatosis (PH) with 119 perinatal controls. Controls were subgrouped ac cording to diagnoses based on clinical and autopsy findings; 37 bad ch ronic liver disease, either hepatic fibrosis (17) or cirrhosis (20). G raded semiquantitatively, hepatocellular siderosis varied widely among controls, and some showed more than PH cases. By chemical assay, tota l hepatic iron in PH cases was not significantly greater than in any c ontrol group except the preterm. Therefore, our findings do not suppor t an etiological role for iron in PH. Its distinctive hepatic morpholo gy seems related to onset Of liver disease during fetal life, when per iportal hepatocytes normally contain hemosiderin (as in 71 of 82 contr ols without chronic liver disease). Environmental agents (such as hypo xia, virus, drug) that could damage afetal liver would usually damage other fetal organs as well. They would be unlikely to recur in a subse quent pregnancy and thereby account for PH occurring in siblings. In i nitiating PH, therefore, putative environmental agents may need to int eract with a factor or factors intrinsic to the developing fetal liver .