Gagliardi Maria Giulia
Department of Cardiology, Ospedale Pediatrico Bambino Gesù, Scientific Institute, (IRCCS), Rome, Italy.
Acta Paediatr Suppl. 2006 Jul;95(452):14-6. doi: 10.1111/j.1651-2227.2006.tb02408.x.
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.
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.
扩张型心肌病(DCM)在儿科人群中是一种罕见疾病。我们分析了心肌炎治疗中免疫疗法的流行病学、临床特征及作用。基于实验证据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患心肌炎的儿童,并在此展示我们的病例系列。未来可靠预后标志物的出现应能使仅对那些不能自发恢复的心肌炎患儿进行治疗。提示伴有心肌炎的DCM与非炎症形式的DCM可能是不同的病理实体。
我们观察到患心肌炎儿童的高长期生存率可能归因于短期免疫抑制的作用。这一结果与先前发表的常规治疗儿童系列不同,那些儿童1年时的生存概率约为0.60。