马凡氏综合征与心脏
Marfan's syndrome and the heart.
作者信息
Stuart Alan Graham, Williams Andrew
机构信息
Congenital Heart Centre, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, UK.
出版信息
Arch Dis Child. 2007 Apr;92(4):351-6. doi: 10.1136/adc.2006.097469.
In recent years, there have been many advances in the treatment of cardiac disease in children with Marfan's syndrome. Early diagnosis, meticulous echocardiographic follow-up and multidisciplinary assessment are essential. Medical treatment with beta-blockers is probably helpful in most children with aortic root dilatation. Research on TGFbeta signalling and the potential treatment role of TGFbeta antagonists may lead to exciting new treatments, but the results of clinical trials are awaited. In managing the cardiovascular complications of Marfan's syndrome, the paediatrician has to walk a difficult path. On the one hand, restrictive lifestyle advice and drugs may need to be prescribed, often in the context of a family history of major surgery or even sudden death. On the other hand, it is essential to encourage the often asymptomatic child to develop and mature as normally as possible.
近年来，马凡氏综合征患儿的心脏病治疗取得了许多进展。早期诊断、细致的超声心动图随访以及多学科评估至关重要。对于大多数主动脉根部扩张的儿童，使用β受体阻滞剂进行药物治疗可能有益。对转化生长因子β（TGFβ）信号传导及TGFβ拮抗剂潜在治疗作用的研究可能会带来令人兴奋的新疗法，但仍有待临床试验结果。在处理马凡氏综合征的心血管并发症时，儿科医生面临着一条艰难的道路。一方面，通常在有重大手术甚至猝死家族史的情况下，可能需要开出限制性的生活方式建议和药物。另一方面，鼓励通常无症状的儿童尽可能正常地成长和成熟至关重要。
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