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.

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 Schistosomiasis-associated pulmonary arterial hypertension: a systematic review

2020 ◽  
Vol 29 (155) ◽  
pp. 190089 ◽  
Author(s):  
Daniela Knafl ◽  
Christian Gerges ◽  
Charles H. King ◽  
Marc Humbert ◽  
Amaya L. Bustinduy
Keyword(s):  
Systematic Review ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Transforming Growth Factor ◽  
Meta Analysis ◽  
Signalling Pathway ◽  
Pathophysiological Mechanisms ◽  
Clinical Registries ◽  
Life Threatening ◽  
Pulmonary Arterial

Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is a life-threatening complication of chronic hepatosplenic schistosomiasis. It is suggested to be the leading cause of pulmonary arterial hypertension (PAH) worldwide. However, pathophysiological data on Sch-PAH are scarce. We examined the hypothesis that there are pronounced similarities in pathophysiology, haemodynamics, and survival of Sch-PAH and idiopathic PAH (iPAH).This systematic review and meta-analysis was registered in the PROSPERO database (identifier CRD42018104066). A systematic search and review of the literature was performed according to PRISMA guidelines for studies published between 01 January 1990 and 29 June 2018.For Sch-PAH, 18 studies evaluating pathophysiological mechanisms, eight studies on haemodynamics (n=277), and three studies on survival (n=191) were identified. 16 clinical registries reporting data on haemodynamics and survival including a total of 5792 patients with iPAH were included for comparison. Proinflammatory molecular pathways are involved in both Sch-PAH and iPAH. The transforming growth factor (TGF)-β signalling pathway is upregulated in Sch-PAH and iPAH. While there was no difference in mean pulmonary artery pressure (54±17 mmHg versus 55±15 mmHg, p=0.29), cardiac output (4.4±1.3 L·min−1versus 4.1±1.4 L·min−1, p=0.046), and cardiac index (2.6±0.7 L·min−1·m−2versus 2.3±0.8 L·min−1·m−2, p<0.001) were significantly higher in Sch-PAH compared to iPAH, resulting in a lower pulmonary vascular resistance in Sch-PAH (10±6 Woods units versus 13±7 Woods units, p<0.001). 1- and 3-year survival were significantly better in the Sch-PAH group (p<0.001).Sch-PAH and iPAH share common pathophysiological mechanisms related to inflammation and the TGF-β signalling pathway. Patients with Sch-PAH show a significantly better haemodynamic profile and survival than patients with iPAH.

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

scholarly journals Survival of patients with pulmonary arterial hypertension during therapy PAH-specific drugs

2017 ◽  
Vol 14 (3) ◽  
pp. 58-64
Author(s):  
D Yu Platonov ◽  
N A Tsareva ◽  
S N Avdeev
Keyword(s):  
Clinical Trials ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Patient Survival ◽  
Morphological Changes ◽  
Pulmonary Arteries ◽  
Progressive Increase ◽  
Functional Disorders ◽  
Life Threatening ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a life threatening syndrome, which is characterized by a progressive increase in pulmonary vascular resistance (PVR), medium pulmonary artery pressure and functional disorders and morphological changes of pulmonary arteries. Despite the relative abundance of PAH-specific drugs, the choice of a suitable agent for the treatment of a particular patient remains challenging. One of the most important parameters measured in clinical studies is patient survival or, in some cases, mortality. Survival or mortality as secondary or components of the primary endpoint one way or another has been studied in clinical trials of drugs for the treatment of PAH. For tablets drugs for the treatment of some forms of PAH (sildenafil, riociguat, bosentan, macitentan, ambrisentan) currently available data studies, which studied the survival.

scholarly journals Intravascular Large B-cell Lymphoma Presenting Pulmonary Arterial Hypertension as an Initial Manifestation

2010 ◽  
Vol 49 (1) ◽  
pp. 51-54 ◽  
Author(s):  
Takeshi Kotake ◽  
Satoru Kosugi ◽  
Takayuki Takimoto ◽  
Soichi Nakata ◽  
Junko Shiga ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Initial Manifestation ◽  
Large B Cell Lymphoma ◽  
Pulmonary Arterial ◽  
Large B Cell

scholarly journals Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Kofi A. Mensah ◽  
Rajwardhan Yadav ◽  
Terence K. Trow ◽  
Cristina M. Brunet ◽  
Wassim H. Fares
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Treatment Approach ◽  
Long Term Outcomes ◽  
Care Level ◽  
Life Threatening ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

We describe a critically ill young woman with systemic lupus erythematosus (SLE) presenting with circulatory shock, multiorgan dysfunction, and elevated right-sided heart pressures. She was found to have recurrent acute severe pulmonary arterial hypertension (PAH) in the setting of an SLE flare. Our report highlights the variable course that SLE-associated PAH can take in the same patient and the implications of this for instituting the most effective treatment approach with each episode. This report also highlights the potential for SLE-associated PAH to present with life-threatening symptoms requiring critical care level interventions. We also describe evidence-based therapies, which can result in significant improvement in symptoms, function, and long-term outcomes.

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