Dilated cardiomyopathy in children.

Maria Giulia Gagliardi
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Abstract

Unlabelled: Dilated cardiomyopathy (DCM) is a rare disease in the paediatric population. We analysed the epidemiology, clinical features and role of immunotherapy in the treatment of myocarditis. On the basis of experimental evidence, indicating that autoimmunity might play a role in the development of myocarditis, we treated children affected by myocarditis with immunosuppressive therapy, and we present here our series. The future availability of reliable prognostic markers should allow treatment of only those children with myocarditis who do not spontaneously recover. The possibility that DCM with myocarditis is a distinct pathological entity from the non-inflammatory form of DCM is suggested.

Conclusion: The high long-term survival rate observed in our children with myocarditis is probably due to the effect of short-term immunosuppression. This result is at odds with previously published series of conventionally treated children, whose survival probability at 1 y was approximately 0.60.

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儿童扩张型心肌病。
未标记:扩张型心肌病(DCM)是一种罕见的疾病在儿科人群。我们分析了心肌炎的流行病学、临床特点及免疫治疗在心肌炎中的作用。在实验证据的基础上,表明自身免疫可能在心肌炎的发展中起作用,我们用免疫抑制疗法治疗心肌炎患儿,并在此提出我们的系列。未来可靠的预后标记物的可用性应该允许仅治疗那些患有心肌炎但不能自行恢复的儿童。伴有心肌炎的DCM可能是一种不同于非炎症形式的DCM的病理实体。结论:小儿心肌炎长期生存率高,可能与短期免疫抑制作用有关。这一结果与先前发表的一系列常规治疗儿童的结果不一致,这些儿童在1岁时的存活率约为0.60。
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