Registries for paediatric pulmonary hypertension
نویسندگان
چکیده
منابع مشابه
Registries for paediatric pulmonary hypertension.
Pulmonary arterial hypertension (PAH) is a progressive, angio-obliterative disease leading to increased pulmonary vascular resistance, right heart failure, and death in ,25–60% of PAH patients 5 years after diagnosis [1–3]. The estimated prevalence of PAH is 15–50 cases per million adults [4–6] and 2–16 cases per million children [7–9]. In certain at-risk groups, however, the occurrence of PAH ...
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In July 2009, the patient’s condition worsened with PFT results comparable to those at first presentation (fig. 1). Treatment with azathioprine (150 mg?day) was then started as hydroxychloroquine was withdrawn. This treatment allowed a significant gradual clinical, radiological and functional improvement (fig. 2), with almost normalised PFTs by December 2011 (fig 1). SACE level also normalised....
متن کامل[Pulmonary arterial hypertension: epidemiology and registries].
Registries of patients with pulmonary arterial hypertension (PAH) have been instrumental in characterizing the presentation and natural history of the disease and provide a basis for prognostication. Since the initial accumulation of data conducted in the 1980s, subsequent registry databases have yielded information about the demographic factors, treatment, and survival of patients and have per...
متن کاملTreatment options for paediatric pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is a serious, progressive condition, which can present idiopathically or secondary to conditions such as systemic sclerosis or congenital heart disease. The condition exists in both adult and paediatric forms, which possess several similar characteristics. Adult and paediatric PAH can, however, be distinguished based on underlying pathology and the presence...
متن کاملThe challenges in paediatric pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting both adults and children. While overall survival has improved in recent years, the need for improved therapeutic approaches remains. Treatments for paediatric PAH have not yet been sufficiently examined, particularly regarding potential toxicities and optimal dosing, and there is a lack of appropriate clinical trial ...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2013
ISSN: 0903-1936,1399-3003
DOI: 10.1183/09031936.00065713