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Iron and pediatric liver disease
1Department of Pathology, Children's Hospital of Pittsburgh, Pennsylvania 15213.
Insights
Iron overload in the liver is normal in newborns but absent in children. Early phlebotomy is crucial for hereditary hemochromatosis (HH) patients to prevent liver disease and iron toxicity.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Iron Metabolism
Background:
- Stainable liver iron is normal in the perinatal period but absent in childhood/adolescence.
- Liver disease risk increases with transfusions if iron overload is untreated.
- Elevated iron stores in non-transfused children suggest hereditary hemochromatosis (HH).
Purpose of the Study:
- To clarify the role of iron in liver disease across different age groups.
- To differentiate normal perinatal iron from pathological iron overload.
- To guide diagnosis and management of iron overload in pediatric patients.
Main Methods:
- Review of physiological iron presence and pathological overload.
- Clinical presentation of hereditary hemochromatosis (HH) in pediatric populations.
- Comparison of adult vs. infant criteria for assessing liver iron overload.
Main Results:
- Liver iron is normal in newborns but pathological in older children without transfusions (suggesting HH).
- Untreated iron overload from transfusions leads to liver disease.
- HH is rare symptomatically in children; diagnosis often follows family screening.
Conclusions:
- Liver iron assessment criteria differ significantly between neonates and adults.
- Early phlebotomy is vital for HH patients to prevent iron toxicity and liver damage.
- Iron's role as an oxidant may contribute to liver injury, even at normal levels.
Abstract:
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.