2013
DOI: 10.1183/09031936.00065713
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Registries for paediatric pulmonary hypertension

Abstract: @ERSpublications Paediatric pulmonary arterial hypertension should be treated specifically and differently from adult pulmonary arterial hypertension http://ow.ly/mzPDT 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][2][3]. The estimated prevalence of PAH is 15-50 cases per million adults [4-6] and 2-16 cases per million children [7][8][9].… Show more

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Cited by 26 publications
(27 citation statements)
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“…Similar numbers have been observed in the UK, with annual cases of IPAH of 0.48 per million and a prevalence of 2.1 per million [286]. National and large-scale registries either including children [284] or exclusively dedicated to paediatrics [287,288] have described the different aetiologies of PH, with IPAH, HPAH and CHD-PAH as the most common. However, PH associated with respiratory disease is also noted to be important and may be underreported [287].…”
Section: Specific Pulmonary (Arterial) Hypertension Subsetssupporting
confidence: 50%
“…Similar numbers have been observed in the UK, with annual cases of IPAH of 0.48 per million and a prevalence of 2.1 per million [286]. National and large-scale registries either including children [284] or exclusively dedicated to paediatrics [287,288] have described the different aetiologies of PH, with IPAH, HPAH and CHD-PAH as the most common. However, PH associated with respiratory disease is also noted to be important and may be underreported [287].…”
Section: Specific Pulmonary (Arterial) Hypertension Subsetssupporting
confidence: 50%
“…Podobne wartości zaobserwowano w Wielkiej Brytanii z roczną zapadalnością na IPAH rzędu 0,48 na milion i chorobowością 2,1 na milion [286]. W rejestrach krajowych i innych dużych bazach danych obejmujących dzieci [284] lub wyłącznie poświęconych pediatrii [287,288] opisano różne etiologie PH, przy czym IPAH, HPAH i CHD-PAH występowały najczęściej. Również PH związane z chorobami układu oddechowego występuje często, jednak może nie być zgłaszane do rejestrów [287].…”
Section: Tętnicze Nadciśnienie Płucne U Dzieciunclassified
“…The estimated prevalence of PAH is 2-16 cases per million children (49). In the past, studies in adults with PH have been used to make assumptions regarding the disease in children, but this approach has been recognized as inadequate.…”
Section: Age-related Effects Of Estrogen On the Pulmonary Vasculaturementioning
confidence: 99%
“…In the past, studies in adults with PH have been used to make assumptions regarding the disease in children, but this approach has been recognized as inadequate. A recent, comprehensive analysis of pediatric registries has outlined epidemiology, clinical practice, and outcome of PAH in children (11,49). It appears important to recognize the fetal origins and developmental and adaptive processes of pulmonary vascular disease in children (see below), along with the role of congenital heart disease.…”
Section: Age-related Effects Of Estrogen On the Pulmonary Vasculaturementioning
confidence: 99%