Results 11 to 20 of about 27,650 (185)
Pulmonary hypertension associated with neurofibromatosis type 2
Pulmonary Circulation, 2021 Although precapillary pulmonary hypertension is a rare but severe complication of patients with neurofibromatosis type 1 (NF1), its association with NF2 remains unknown.
Hirohisa Taniguchi +9 more
doaj +1 more source
Pulmonary Circulation, 2020 Platelet-derived growth factor is one of the major growth factors found in human and mammalian serum and tissues. Abnormal activation of platelet-derived growth factor signaling pathway through platelet-derived growth factor receptors may contribute to ...
Kang Wu +21 more
doaj +1 more source
Outcomes in Children With Idiopathic Pulmonary Arterial Hypertension [PDF]
Circulation, 2004 Background— Treatment for idiopathic pulmonary arterial hypertension in children includes calcium channel blockade (CCB) for acute responders with vasodilator testing and chronic epoprostenol for nonresponders. We sought to determine parameters associated with survival and treatment success.
Delphine, Yung +5 more
openaire +2 more sources
Hepatology, 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
Pulmonary 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
Nature Communications, 2021 Idiopathic pulmonary arterial hypertension is a rare and fatal disease with a heterogeneous treatment response. Here the authors show that unsupervised machine learning of whole blood transcriptomes from 359 patients with idiopathic pulmonary arterial ...
Sokratis Kariotis +34 more
doaj +1 more source
Transcriptional profiling of lung cell populations in idiopathic pulmonary arterial hypertension
Pulmonary 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
Pulmonary 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
Pulmonary 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
Pulmonary 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