Introduction and objective Left-heart dysfunction and pulmonary vasculopathy are increasingly recognized as contributing factors of exercise capacity limitation in interstitial fibrosing lung disease (IFLD).
E. Panagiotidou +9 more
doaj +1 more source
Onasemnogene Abeparvovec in Patients With SMA: Interim Results of the RESTORE Registry in Japan
ABSTRACT Objective There are limited real‐world data regarding the safety and effectiveness of onasemnogene abeparvovec (OA; Zolgensma) infusion, a one‐time gene replacement therapy, for Japanese patients with spinal muscular atrophy (SMA). We aimed to improve understanding of the real‐world outcomes for OA in Japan.
Kayoko Saito +8 more
wiley +1 more source
Beta Agonist Lung Injury TrIal-2 (BALTI-2) trial protocol : a randomised, double-blind, placebo-controlled of intravenous infusion of salbutamol in the acute respiratory distress syndrome [PDF]
Background: The Acute Respiratory Distress Syndrome (ARDS) is a common cause of respiratory failure in critically ill patients. Experimental studies suggest that treatment with beta agonists may be helpful in ARDS.
McCabe Chris +28 more
core +2 more sources
Cognitive and Neuroimaging Divergence Between Juvenile and Adult FUS Amyotrophic Lateral Sclerosis
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis.
Alexandra V. Jürs +7 more
wiley +1 more source
Soluble IL-2R Levels Predict in-Hospital Mortality in COVID-19 Patients with Respiratory Failure
Acute respiratory distress syndrome is the primary cause of death in patients with coronavirus disease 2019 (COVID-19) pneumonia. Our study aims to determine the association between serum markers and mortality in COVID-19 patients with respiratory ...
Su Hwan Lee +8 more
core +1 more source
ABSTRACT Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an ultra‐rare, ACTA2‐related disorder characterized by severe cerebrovascular disease, aortic aneurysms, and smooth muscle dysfunction. Using molecular dynamics simulations and in silico drug screening, we identified that sapropterin dihydrochloride (Kuvan) is a candidate capable of ...
Moran Hausman‐Kedem +9 more
wiley +1 more source
The successful treatment of hypercapnic respiratory failure with oral modafinil
Helen Parnell,1 Ginny Quirke,1 Sally Farmer,1 Sumbo Adeyemo,2 Veronica Varney11Respiratory Department, 2Pharmacy Department, St Helier Hospital, Carshalton, Surrey, UKAbstract: Hypercapnic respiratory failure is common in advanced chronic obstructive ...
Farmer S +4 more
core
Role of Respiratory Viruses in Severe Acute Respiratory Failure
Respiratory viruses are widespread in the community, affecting both the upper and lower respiratory tract. This review provides an updated synthesis of the epidemiology, pathophysiology, clinical impact, and management of severe respiratory viral ...
Mokrani, David +3 more
core +1 more source
Oro-nasal mask versus helmet in acute hypercapnic respiratory failure
The choice of the interface for noninvasive ventilation (NIV) is a key factor in NIV success. We hypothesised that a new helmet specifically design to improve performance in hypercapnic patients would be clinically equivalent to a standard oronasal mask.
Musti M. +57 more
core +1 more source
Respiratory health and endotoxin: associations and modification by CD14/-260 genotype [PDF]
Exposure to endotoxin has been associated with increased respiratory symptoms and decrements in lung function in occupational settings but little is known about the health effects of domestic exposure in adults.
Jarvis, D. +77 more
core +1 more source

