Pulmonary arterial hypertension (PAH) frequently is associated with an imbalance in antiproliferative bone morphogenic protein-2 receptor signaling and proproliferative type-II activin receptor signaling, favoring the latter. Sotatercept is an activin ligand trap that reduces the dominant detrimental activin signaling and provides clinical benefit. We report a patient with heritable PAH in whom sotatercept had neither positive nor negative effects; we relate that fact to his PAH being caused by a previously unreported variant of unknown significance (c.
View Article and Find Full Text PDFPurpose: This study aimed to validate the new DormoTech Vlab device's performance, usability, and validity as a sleep test and physiological data recorder. The novel device has been designed for patient comfort, ease of use, and home-based assessment of sleep disordered breathing and other sleep-related measurements.
Methods: Forty-seven adults (mean age = 52 years, 42% female, body mass index 29.
BMJ Open Respir Res
November 2022
Background: Non-invasive ventilation (NIV) is effective in a variety of acute respiratory illnesses in hospitalised patients. Home NIV is effective for stable patients with hypercapnia due to neuromuscular or chronic pulmonary disease. However, there are little data to guide which patients may benefit from NIV immediately following hospitalisation with hypercapnia.
View Article and Find Full Text PDFExercise-induced increases in pulmonary blood flow normally increase pulmonary arterial pressure only minimally, largely due to a reserve of pulmonary capillaries that are available for recruitment to carry the flow. In pulmonary arterial hypertension, due to precapillary arteriolar obstruction, such recruitment is greatly reduced. In exercising pulmonary arterial hypertension patients, pulmonary arterial pressure remains high and may even increase further.
View Article and Find Full Text PDFPulmonary arterial pressure rises minimally during exercise. The pulmonary microcirculation accommodates increasing blood flow recruitment of pulmonary capillaries and, at higher flows, by distention of already perfused capillaries. The flow transition range between recruitment and distention has not been studied or compared across mammalian species, including humans.
View Article and Find Full Text PDFSarcoidosis Vasc Diffuse Lung Dis
November 2020
J Vasc Interv Radiol
October 2019
Am J Physiol Lung Cell Mol Physiol
September 2019
Introduction: Step climbing is a potentially useful modality for testing exercise capacity. However, there are significant variations between test protocols and lack of consistent validation against gold standard cycle ergometry cardiopulmonary exercise testing (CPET). The purpose of the study was to validate a novel technique of exercise testing using a dedicated device.
View Article and Find Full Text PDFAnn Transplant
November 2017
BACKGROUND Lung transplant (LTx) recipients suffer from high rates of malignancy. Exposure to immunosuppressive medication such as tacrolimus has been proposed as a risk factor for tumorigenesis. We hypothesized that chronically high levels of tacrolimus would be associated with risk of malignancy.
View Article and Find Full Text PDFPurpose: Immunosuppressive therapy plays a major role in the development of post-transplant cancer. In this nested case-control study of kidney transplant recipients (KTRs), we investigated whether the incidence of post-transplant cancer is associated with the level of tacrolimus exposure over time.
Methods: We screened the Rabin Medical Center database for adults who received kidney transplants between 2001 and 2014 and developed post-transplant cancer (excluding basal and squamous cell skin cancers).
Background: Silicosis is a progressive lung disease resulting from the inhalation of respirable crystalline silica. Lung transplantation is the only treatment for end-stage silicosis. The aim of this study was to analyze the survival experience following lung transplantation among patients with silicosis.
View Article and Find Full Text PDFEur J Phys Rehabil Med
April 2017
Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal interstitial lung disease associated with cardiovascular impairments which compromise exercise tolerance and worsen prognosis.
Aim: To examine the effect of participating in supervised exercise training (ET) program on cardiovascular function in patients with IPF.
Design: A randomized controlled study.
Pulm Circ
September 2016
It is unclear whether pregnancy is a trigger or accelerant for idiopathic pulmonary arterial hypertension (PAH). Alternatively, its frequency close to the onset of symptoms and diagnosis in the idiopathic PAH population may represent a coincidence in a disease that predominates in young women. We describe a carrier of a BMPR2 gene mutation who had an uneventful first pregnancy but had aggressive PAH during her second pregnancy and now has ongoing heritable PAH.
View Article and Find Full Text PDFBackground: Combination therapy (CT) for patients with pulmonary arterial hypertension (PAH) has been recommended for many years, despite weak evidence of efficacy over monotherapy (MT). A previous meta-analysis comparing CT vs MT with pulmonary vasodilators failed to demonstrate a clear reduction in clinical worsening events.
Methods: We searched for relevant articles in PubMed, EMBASE, the Cochrane Database, and clinicaltrials.
Purpose. The aim of this study was to evaluate the sensitivity of pleural C-reactive protein (CRP) biomarker levels in identifying parapneumonic effusions. Methods.
View Article and Find Full Text PDFBackground: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fatal interstitial lung disease associated with poor prognosis and limited effective treatment options. Reliable predictors of outcome in daily clinical practice are needed to determine high-risk patients for urgent lung transplantation referral. This study aimed to identify practical prognostic predictors of mortality using cardiopulmonary exercise testing (CPET) in IPF subjects.
View Article and Find Full Text PDFJ Cardiopulm Rehabil Prev
December 2017
Objective: To examine the effect of participating in a 12-week supervised exercise training (ET) program on physical activity and body composition in patients with idiopathic pulmonary fibrosis (IPF).
Design: Randomized controlled trial assessing physical activity and body composition at baseline, after 12-week intervention, and at 11 months follow-up.
Setting: Outpatient hospital.