Iron and pediatric liver disease.
As a rule, stainable iron can normally be present in the liver during the perinatal period and is normally absent from the liver during childhood and adolescence. Liver disease can be expected to develop when repeated transfusions are administered and neither phlebotomy nor chelation therapy is undertaken; in the setting of prolonged transfusional support, the source of iron overload is no mystery, and appropriate steps can be taken prophylactically to minimize the risk of iron-related liver disease. If elevated iron stores are discovered in a pediatric patient without a history of transfusion, the diagnosis of HH should be considered. Symptomatic HH is rare in childhood or adolescence, and most patients with HH in these age groups will come to medical attention because a relative with HH has been identified and family members are being screened for iron overload. It is important to initiate phlebotomy therapy in patients with HH before iron toxicity develops. To find iron in the liver of a newborn infant with liver disease is not necessarily abnormal. It is possible that iron, as an oxidant, may potentiate damage initiated by other agents, so that depletion of even physiologically normal iron stores may be of value in treatment. Most important is to remember that criteria for assessing iron overload in adults are not suited for assessing iron overload in newborn infants, and to hold back from ascribing to iron overload too large a role in whatever disease process is underway.
Knisely
A S
AS
Department of Pathology, Children's Hospital of Pittsburgh, Pennsylvania 15213.
D002648:Child; D006432:Hemochromatosis / Q000235:genetics*; D006801:Humans; D007501:Iron / Q000009:adverse effects / Q000378:metabolism*; D008099:Liver / Q000378:metabolism; D008107:Liver Diseases; D012806:Siderosis
D007501:Iron