Douwes Johannes M, van Loon Rosa L E, Roothooft Marcus T R, Berger Rolf M F
Universitair Medisch Centrum Groningen, Centrum voor Congenitale Hartafwijkingen, Beatrix Kinderziekenhuis, Groningen, the Netherlands.
Ned Tijdschr Geneeskd. 2011;155(49):A3901.
Progressive pulmonary arterial hypertension (PAH) is a rare condition with high morbidity and mortality. Paediatric PAH distinguishes itself from PAH in adults, but is still poorly characterized. Paediatric PAH presents itself with non-specific symptoms which often results in later diagnosis. Determination of the correct underlying diagnosis in paediatric PAH is complex, and must therefore take place at specialized centres. Paediatric progressive PAH is usually either idiopathic or associated with congenital heart disease. Pediatric PAH frequently co-occurs with dysmorphic abnormalities, which may point towards aetiological mechanisms. Recent reports suggest an improved survival and exercise tolerance due to treatment with new second-generation drugs for paediatric PAH. In the Netherlands, the care for children with PAH is centralised to guarantee the optimization of diagnosis and treatment in accordance with the most recent scientific insights.
进行性肺动脉高压（PAH）是一种发病率和死亡率都很高的罕见疾病。儿童PAH有别于成人PAH，但其特征仍不明确。儿童PAH表现为非特异性症状，这常常导致诊断延迟。确定儿童PAH正确的潜在诊断很复杂，因此必须在专业中心进行。儿童进行性PAH通常要么是特发性的，要么与先天性心脏病有关。儿童PAH常与畸形异常同时出现，这可能指向病因机制。最近的报告表明，使用治疗儿童PAH的新型第二代药物可提高生存率和运动耐量。在荷兰，对PAH患儿的护理是集中进行的，以确保根据最新科学见解优化诊断和治疗。
