Knisely A S
Department of Pathology, Children's Hospital of Pittsburgh, Pennsylvania 15213.
Semin Liver Dis. 1994 Aug;14(3):229-35. doi: 10.1055/s-2007-1007314.
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.
通常情况下，围产期肝脏中可出现可染色铁，而儿童期和青春期肝脏中通常不存在可染色铁。当反复输血且未进行放血或螯合治疗时，预计会发生肝脏疾病；在长期输血支持的情况下，铁过载的来源并不神秘，可以采取适当的预防措施将铁相关肝脏疾病的风险降至最低。如果在无输血史的儿科患者中发现铁储存升高，应考虑遗传性血色素沉着症（HH）的诊断。症状性HH在儿童期或青春期很少见，这些年龄组的大多数HH患者就医是因为已确定有HH亲属并对家庭成员进行铁过载筛查。在HH患者发生铁中毒之前启动放血治疗很重要。在患有肝病的新生儿肝脏中发现铁不一定异常。铁作为一种氧化剂，可能会增强其他因素引发的损伤，因此即使是生理上正常的铁储存耗竭在治疗中也可能有价值。最重要的是要记住，评估成人铁过载的标准不适用于评估新生儿铁过载，并且在任何正在进行的疾病过程中，都不要将铁过载的作用归因过大。
