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Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 3...

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Autores principales: Kwiatkowska, Joanna, Zuk, Malgorzata, Migdal, Anna, Kusa, Jacek, Skiba, Elzbieta, Zygielo, Karolina, Przetocka, Kinga, Werynski, Piotr, Banaszak, Pawel, Rzeznik-Bieniaszewska, Alina, Surmacz, Rafal, Bobkowski, Waldemar, Wojcicka-Urbanska, Barbara, Werner, Bozena, Pluzanska, Joanna, Ostrowska, Katarzyna, Waldoch, Anna, Kopec, Grzegorz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7356296/
https://www.ncbi.nlm.nih.gov/pubmed/32503164
http://dx.doi.org/10.3390/jcm9061717
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author Kwiatkowska, Joanna
Zuk, Malgorzata
Migdal, Anna
Kusa, Jacek
Skiba, Elzbieta
Zygielo, Karolina
Przetocka, Kinga
Werynski, Piotr
Banaszak, Pawel
Rzeznik-Bieniaszewska, Alina
Surmacz, Rafal
Bobkowski, Waldemar
Wojcicka-Urbanska, Barbara
Werner, Bozena
Pluzanska, Joanna
Ostrowska, Katarzyna
Waldoch, Anna
Kopec, Grzegorz
author_facet Kwiatkowska, Joanna
Zuk, Malgorzata
Migdal, Anna
Kusa, Jacek
Skiba, Elzbieta
Zygielo, Karolina
Przetocka, Kinga
Werynski, Piotr
Banaszak, Pawel
Rzeznik-Bieniaszewska, Alina
Surmacz, Rafal
Bobkowski, Waldemar
Wojcicka-Urbanska, Barbara
Werner, Bozena
Pluzanska, Joanna
Ostrowska, Katarzyna
Waldoch, Anna
Kopec, Grzegorz
author_sort Kwiatkowska, Joanna
collection PubMed
description We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was 11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%) had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth (n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome (n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of them with double combination therapy, which improved after changing the reimbursement policy. The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a multidisciplinary approach to the management of PAH children.
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spelling pubmed-73562962020-07-31 Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL) Kwiatkowska, Joanna Zuk, Malgorzata Migdal, Anna Kusa, Jacek Skiba, Elzbieta Zygielo, Karolina Przetocka, Kinga Werynski, Piotr Banaszak, Pawel Rzeznik-Bieniaszewska, Alina Surmacz, Rafal Bobkowski, Waldemar Wojcicka-Urbanska, Barbara Werner, Bozena Pluzanska, Joanna Ostrowska, Katarzyna Waldoch, Anna Kopec, Grzegorz J Clin Med Article We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was 11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%) had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth (n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome (n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of them with double combination therapy, which improved after changing the reimbursement policy. The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a multidisciplinary approach to the management of PAH children. MDPI 2020-06-03 /pmc/articles/PMC7356296/ /pubmed/32503164 http://dx.doi.org/10.3390/jcm9061717 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Kwiatkowska, Joanna
Zuk, Malgorzata
Migdal, Anna
Kusa, Jacek
Skiba, Elzbieta
Zygielo, Karolina
Przetocka, Kinga
Werynski, Piotr
Banaszak, Pawel
Rzeznik-Bieniaszewska, Alina
Surmacz, Rafal
Bobkowski, Waldemar
Wojcicka-Urbanska, Barbara
Werner, Bozena
Pluzanska, Joanna
Ostrowska, Katarzyna
Waldoch, Anna
Kopec, Grzegorz
Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)
title Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)
title_full Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)
title_fullStr Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)
title_full_unstemmed Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)
title_short Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)
title_sort children and adolescents with pulmonary arterial hypertension: baseline and follow-up data from the polish registry of pulmonary hypertension (bnp-pl)
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7356296/
https://www.ncbi.nlm.nih.gov/pubmed/32503164
http://dx.doi.org/10.3390/jcm9061717
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