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

