Publications by authors named "Benjamin D Fox"

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.

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Purpose: 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.

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  • - The DES-OSA score helps predict obstructive sleep apnea syndrome (OSAS) severity by using physical examination signs, and this study validated its effectiveness in the Israeli population.
  • - Researchers conducted a study with 106 patients, finding high correlation between DES-OSA scores and actual sleep apnea severity, and strong agreement between two physicians calculating the scores.
  • - The results showed that a DES-OSA score of 5 or lower could effectively rule out moderate to severe OSAS, and adding age over 66 years improved the scoring sensitivity.
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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.

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Exercise-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.

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Pulmonary 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.

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  • Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic condition that can lead to uncommon complications like pleural effusion.
  • A study analyzed 4 male patients with confirmed IgG4-RD who experienced significant pleural effusions, noting that the effusions had unique characteristics, including high protein levels and lymphocytic involvement.
  • Patients responded well to corticosteroid treatment, and the findings suggested that IgG4-RD may mimic characteristics of tuberculous effusions alongside other notable symptoms such as thoracic lymphadenopathy and elevated immunoglobulin levels.
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  • IPF patients have difficulty reaching their maximum voluntary ventilation (MVV) during exercise testing due to pulmonary vascular issues, which affects their ability to exchange gases properly.
  • A study involving two groups of IPF patients found that direct MVV significantly overestimates peak ventilation (VEpeak) during exercise, leading to potential misdiagnosis of their exercise limitations.
  • The research introduced an IPF-specific formula to predict VEpeak more accurately, showing that most patients have ventilatory limits within a reasonable range of their predicted MVV.
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  • The study investigates how cardiac output (CO) and pulmonary artery pressure (PAP) respond to exercise in humans, highlighting the mechanisms of capillary recruitment and distention during increased blood flow.
  • It involved ten subjects undergoing exercise testing, with measurements showing significant increases in CO and PAP, while a decrease in the metabolism of a specific compound (BPAP) was observed at peak exercise.
  • The results indicate that during moderate exercise, capillary recruitment is the primary response to increasing blood flow, whereas in one subject with already high CO, further increases were linked to capillary distention, providing insights into exercise physiology and pulmonary hypertension.
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  • The study investigated the impact of high variability in tacrolimus drug levels on kidney graft survival, finding that high levels of variability were linked to decreased survival rates.* -
  • Researchers analyzed data from 878 kidney transplant patients over approximately three years, discovering that a time-weighted coefficient of variability (TWCV) above 25% significantly correlated with higher risks of graft loss.* -
  • The findings suggest that reducing TWCV below 25% can improve graft survival, indicating the importance of monitoring and managing tacrolimus levels in post-transplant patients.*
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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.

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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.

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  • The study investigates the survival rates of lung transplant recipients in Israel based on donor-recipient ancestry (Jewish or Arab) and gender combinations (match or mismatch).
  • Researchers analyzed data from 345 lung transplant cases at Rabin Medical Center between 1997 and 2013, comparing survival outcomes based on these factors.
  • Results showed no significant differences in survival based on ancestry or gender, with younger donor age being the only notable factor linked to longer survival.
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Purpose: 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).

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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.

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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.

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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.

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Background: 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.

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Purpose. The aim of this study was to evaluate the sensitivity of pleural C-reactive protein (CRP) biomarker levels in identifying parapneumonic effusions. Methods.

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Background: 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.

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Article Synopsis
  • The study focused on understanding the physiological aspects and challenges faced during exercise by patients with idiopathic pulmonary fibrosis.
  • Approximately 38% of the 34 studied patients had additional health issues, though the majority showed only a moderate level of shortness of breath.
  • Despite moderately reduced aerobic capacity and some dysfunction in the lungs and circulation, the patients’ overall functional capacity, as measured by a 6-minute walking test, remained normal.
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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.

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Article Synopsis
  • Idiopathic pulmonary fibrosis (IPF) is a serious lung disease that leads to symptoms like difficulty breathing, low oxygen levels, and decreased quality of life.
  • Recent studies indicate that exercise training can significantly improve patients' physical capacity, reduce breathing issues, and enhance overall well-being.
  • The paper aims to review current research on exercise for IPF patients and highlight areas that require further investigation to better understand the long-term benefits and implications.
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