scholarly journals Pulmonary arterial hypertension in the USA: an epidemiological study in a large insured pediatric population

2017 ◽  
Vol 7 (1) ◽  
pp. 126-136 ◽  
Author(s):  
Lin Li ◽  
Susan Jick ◽  
Stefanie Breitenstein ◽  
Gemzel Hernandez ◽  
Alexander Michel ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Epidemiological Study ◽  
Pediatric Population ◽  
The Usa ◽  
Pulmonary Arterial

Healthcare resource utilization and costs in pediatric pulmonary arterial hypertension in a third-level hospital in Mexico

2021 ◽  
Author(s):  
Humberto García-Aguilar ◽  
Silvia Guzmán Vázquez ◽  
Karen Sánchez Trejo ◽  
Yazmin Escobar Juárez ◽  
Katiria Itzel Juárez Vásquez ◽  
...  
Keyword(s):  
Standard Deviation ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Resource Utilization ◽  
Pharmacological Treatment ◽  
Pediatric Population ◽  
Physician Visits ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Level Hospital

Aim: The objective of this study was to evaluate the healthcare costs and resource utilization of pediatric pulmonary arterial hypertension management at a third-level hospital in Mexico. Methods: A retrospective cohort study was conducted in a pediatric population with pulmonary arterial hypertension. Only direct medical costs, derived from pharmacological treatment, laboratory tests, physician visits and hospitalizations, were considered. From an institutional perspective, all costs were accounted for in 2019 US dollars. Results: A total of 82 patients were included. Of these, 55% were female and the mean age was 6.9 (standard deviation ± 4) years. The mean annual cost was $17,452.14 (standard deviation ± $38,944.10), with a median cost of $8,832.75. Conclusion: Pulmonary arterial hypertension is a costly disease, with hospitalization and pharmacological treatment being areas with a higher economic burden. Functional class IV has greater resource utilization and costs.

scholarly journals A Case of Pulmonary Arterial Hypertension Associated with Adult Hemophagocytic Lymphohistiocytosis

2016 ◽  
Vol 6 (4) ◽  
pp. 614-615 ◽  
Author(s):  
Julius Koifman ◽  
John Granton ◽  
John Thenganatt
Keyword(s):  
Differential Diagnosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Respiratory Symptoms ◽  
Cell Lymphoma ◽  
Pediatric Population ◽  
Mantle Cell ◽  
Life Threatening ◽  
Pulmonary Arterial

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH. To our knowledge, PAH associated with adult HLH has only been described in the literature once before. PAH may now be a potential differential diagnosis for patients with HLH who present with respiratory symptoms.

Pulmonary arterial hypertension in Spanish pediatric registry age: clinical characterization, management and survival

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Playan Escribano ◽  
T Segura De La Cal ◽  
P Escribano Subias ◽  
C Labrandero ◽  
A Rodriguez Ogando ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Population ◽  
Functional Class ◽  
Funding Source ◽  
First Line Therapy ◽  
Significant Difference ◽  
Pulmonary Arterial ◽  
Large Groups

Abstract Background Pulmonary arterial hypertension (PAH) includes idiopathic PAH and congenital heart disease (CHD) related PAH. A wide variety of CHD can develop PAH, but their clinical characteristics define four large groups: Eisenmenger, PAH associated with non-restrictive shunt, severe PAH associated with restrictive shunt and postoperative PAH. Our aim was to study the clinical and prognostic differences between these groups and idiopathic PAH. Methods The REHIPED registry is a Spanish, multicenter, observational and prospective registry on pulmonary hypertension in the pediatric population (<18 years of age) that began in 2008. 183 patients were analyzed: baseline characteristics, functional class, right catheterization data, treatment and survival were compared Results 183 patients were analyzed, characteristics are detailed in the table. In patients with idiopathic PAH, treatment with prostanoids was more frequently used as first line therapy and during follow up. The average follow-up time was 9 years. There was not a statistically significant difference in survival among the 4 clinical groups of CHD related PAH. There was a borderline significant difference (logrank p 0.05) in survival between the group of CHD related PAH and idiopathic PAH. Conclusion Idiopathic PAH patients have worse outcome than CHD related PAH patients although they have less comorbidities, less severe hemodynamics and are treated more aggressively. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Janssen, Ferrer

scholarly journals Ask the Expert: A Regulatory Perspective on Clinical Trials for Pulmonary Arterial Hypertension

2020 ◽  
Vol 19 (2) ◽  
pp. 62-65 ◽  
Author(s):  
Christine Garnett ◽  
Norman Stockbridge
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Population ◽  
Composite Endpoint ◽  
The United States ◽  
New Drugs ◽  
Trial Duration ◽  
Clinical Endpoints ◽  
Number Of Patients ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a rare, progressive disease. There are 11 drugs available in the United States to treat adult PAH patients; however, all drugs primarily act through vasodilation and have modest effects on clinical endpoints. None of these drugs can claim survival benefit in their product labels. New drugs are needed that target other mechanisms in the disease to have durable benefits for patients. To demonstrate clinical benefit, new drugs are now tested in large, randomized, placebo-controlled trials evaluating their effect to delay clinical worsening, a composite endpoint of morbidity events and death. Efficient clinical trial designs, such as the use of enrichment strategies, that reduce the number of patients and trial duration would be valuable for this disease. It would also be desirable to have new clinical endpoints that measure improvement in quality of life and allow the use of extrapolation strategies to the pediatric population. Academic, industry, and regulatory partnerships are key to advancing therapies for this disease.

scholarly journals An epidemiological study of pulmonary arterial hypertension

2007 ◽  
Vol 30 (1) ◽  
pp. 104-109 ◽  
Author(s):  
A. J. Peacock ◽  
N. F. Murphy ◽  
J. J. V. McMurray ◽  
L. Caballero ◽  
S. Stewart
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Epidemiological Study ◽  
Pulmonary Arterial

scholarly journals Pulmonary arterial hypertension: Specialists’ knowledge, practices, and attitudes of genetic counseling and genetic testing in the USA

2017 ◽  
Vol 7 (2) ◽  
pp. 372-383 ◽  
Author(s):  
Joseph E. Jacher ◽  
Lisa J. Martin ◽  
Wendy K. Chung ◽  
James E. Loyd ◽  
William C. Nichols
Keyword(s):  
Genetic Counseling ◽  
Genetic Testing ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arteries ◽  
Knowledge Practices ◽  
Wilcoxon Rank Sum Test ◽  
The Usa ◽  
Education And Awareness ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is characterized by obstruction of pre-capillary pulmonary arteries, which leads to sustained elevation of pulmonary arterial pressure. Identifying those at risk through early interventions, such as genetic testing, may mitigate disease course. Current practice guidelines recommend genetic counseling and offering genetic testing to individuals with heritable PAH, idiopathic PAH, and their family members. However, it is unclear if PAH specialists follow these recommendations. Thus, our research objective was to determine PAH specialists’ knowledge, utilization, and perceptions about genetic counseling and genetic testing. A survey was designed and distributed to PAH specialists who primarily work in the USA to assess their knowledge, practices, and attitudes about the genetics of PAH. Participants’ responses were analyzed using parametric and non-parametric statistics and groups were compared using the Wilcoxon rank sum test. PAH specialists had low perceived and actual knowledge of the genetics of PAH, with 13.2% perceiving themselves as knowledgeable and 27% actually being knowledgeable. Although these specialists had positive or ambivalent attitudes about genetic testing and genetic counseling, they had poor utilization of these genetic services, with almost 80% of participants never or rarely ordering genetic testing or referring their patients with PAH for genetic counseling. Physicians were more knowledgeable, but had lower perceptions of the value of genetic testing and genetic counseling compared to non-physicians ( P < 0.05). The results suggest that increased education and awareness is needed about the genetics of PAH as well as the benefits of genetic testing and genetic counseling for individuals who treat patients with PAH.

Sign in / Sign up

Export Citation Format

Share Document