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沙特阿拉伯肺动脉高压诊断和治疗指南:儿童肺动脉高压。

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension in children.

机构信息

Department of Pediatric, King Fahd Armed Forces Hospital, Jeddah, Saudi Arabia.

Department of Pediatric, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

出版信息

Ann Thorac Med. 2014 Jul;9(Suppl 1):S113-20. doi: 10.4103/1817-1737.134053.

Abstract

Pulmonary hypertension (PH) is relatively uncommon in children. Pulmonary arterial hypertension (PAH) in pediatric comprises a wide spectrum of diseases, from a transient neonatal condition to a progressive disease associated with morbidity and mortality. Most common PAH in pediatric are idiopathic (IPAH) or PAH associated with congenital heart disease (PAH-CHD), while other associated conditions, such as connective tissue disease (CTD), are less common in pediatrics. Despite better understanding of PH and the availability of new medications during recent decades; the diagnosis, investigation and choice of therapy remain a challenge in children, as evidence-based recommendations depend mainly on adult studies. In this review, we provide a detailed discussion about the distinctive features of PAH in pediatric, mainly emphacysing on classification and diagnostic algorithm.

摘要

肺动脉高压(PH)在儿童中相对少见。儿科肺动脉高压(PAH)包括广泛的疾病谱,从短暂的新生儿期疾病到与发病率和死亡率相关的进行性疾病。儿科最常见的 PAH 是特发性(IPAH)或与先天性心脏病(PAH-CHD)相关的 PAH,而其他相关疾病,如结缔组织疾病(CTD),在儿科中则较少见。尽管近年来对 PH 的认识有所提高,并且出现了新的药物,但儿童的诊断、检查和治疗选择仍然是一个挑战,因为基于证据的建议主要依赖于成人研究。在这篇综述中,我们详细讨论了儿科 PAH 的独特特征,主要强调了分类和诊断算法。

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