Results 11 to 20 of about 47,347 (252)

Genetic predisposition to porto‐sinusoidal vascular disorder: A functional genomic‐based, multigenerational family study

open access: yesHepatology, EarlyView., 2022
A deleterious variant of FCHSD1 results in mTOR pathway overactivation and may cause porto‐sinusoidal vascular disorder (PSVD). The pedigree of the family demonstrated an autosomal dominant disease with variable expressivity. Whole‐genome sequencing and Sanger sequencing both validated the existence of the FCHSD1 variant and the heterozygosity of c ...
Jingxuan Shan   +19 more
wiley   +1 more source

Inspiratory flow patterns with dry powder inhalers of low and medium flow resistance in patients with pulmonary arterial hypertension

open access: yesPulmonary Circulation, 2021
Inhalation profiles to support use of dry powder inhalers for drug delivery in patients with pulmonary arterial hypertension have not been reported. We aimed to evaluate the inspiratory flow pattern associated with low and medium flow resistance dry ...
Mariana Faria-Urbina   +4 more
doaj   +1 more source

Transcriptional profiling of lung cell populations in idiopathic pulmonary arterial hypertension

open access: yesPulmonary Circulation, 2020
Despite recent improvements in management of idiopathic pulmonary arterial hypertension, mortality remains high. Understanding the alterations in the transcriptome–phenotype of the key lung cells involved could provide insight into the drivers of ...
Didem Saygin   +7 more
doaj   +1 more source

Plasma metabolomic profile in chronic thromboembolic pulmonary hypertension

open access: yesPulmonary Circulation, 2020
We aimed to characterize the plasma metabolome of chronic thromboembolic pulmonary hypertension patients using a high-throughput unbiased omics approach.
Gustavo A. Heresi   +7 more
doaj   +1 more source

Left main bronchus compression due to main pulmonary artery dilatation in pulmonary hypertension: two case reports [PDF]

open access: yes, 2015
. Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures. Left main coronary artery and left recurrent laryngeal nerve compression have been described.
Ariff, B   +6 more
core   +1 more source

Severe thrombocytopenia in patients with idiopathic pulmonary arterial hypertension provided several strategies for lung transplantation

open access: yesPulmonary Circulation, 2020
While the prognosis of idiopathic pulmonary arterial hypertension has improved significantly due to newer medications, lung transplantation remains a critical therapeutic option for severe pulmonary arterial hypertension.
Takayuki Kobayashi   +8 more
doaj   +1 more source

Pulmonary arterial hypertension in pregnancy—a systematic review of outcomes in the modern era

open access: yesPulmonary Circulation, 2021
Pregnancy is hazardous with pulmonary arterial hypertension, but maternal mortality may have fallen in recent years. We sought to systematically evaluate pulmonary arterial hypertension and pregnancy-related outcomes in the last decade.
Ting-Ting Low   +6 more
doaj   +1 more source

Plasma proteome analysis in patients with pulmonary arterial hypertension: an observational cohort study [PDF]

open access: yes, 2017
BackgroundIdiopathic and heritable pulmonary arterial hypertension form a rare but molecularly heterogeneous disease group. We aimed to measure and validate differences in plasma concentrations of proteins that are associated with survival in patients ...
Abdul-Salam   +29 more
core   +1 more source

Glucocorticoids for treating paediatric pulmonary hypertension: A novel use for a common medication [PDF]

open access: yes, 2017
Laboratory investigations have shown the role of inflammation in the pathogenesis of pulmonary hypertension and improvement after anti-inflammatory drugs.
Aggarwal, Manish, Grady, Ronald M
core   +2 more sources

The role of endothelin-1 in pulmonary arterial hypertension. [PDF]

open access: yes, 2014
Pulmonary arterial hypertension (PAH) is a rare but debilitating disease, which if left untreated rapidly progresses to right ventricular failure and eventually death. In the quest to understand the pathogenesis of this disease differences in the profile,
Chester, AH, Yacoub, MH
core   +1 more source

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