Natural History of Cardiac and Respiratory Involvement, Prognosis and Predictive Factors for Long-Term Survival in Adult Patients with Limb Girdle Muscular Dystrophies Type 2C and 2D [PDF]
International audienceBackgroundType 2C and 2D limb girdle muscular dystrophies (LGMD) are a group of autosomal recessive limb girdle muscular dystrophies manifested by proximal myopathy, impaired respiratory muscle function and cardiomyopathy.
Annane, Djillali+15 more
core +9 more sources
Feasibility of non-invasive pressure support ventilation in infants with respiratory failure after extubation: a pilot study [PDF]
Objective: To evaluate the feasibility and effects of non-invasive pressure support ventilation (NIV) on the breathing pattern in infants developing respiratory failure after extubation.
Cotting, Jacques+5 more
core
Relation between respiratory variations in pulse oximetry plethysmographic waveform amplitude and arterial pulse pressure in ventilated patients. [PDF]
IntroductionRespiratory variation in arterial pulse pressure is a reliable predictor of fluid responsiveness in mechanically ventilated patients with circulatory failure.
Besnard, Cyril+4 more
core +2 more sources
ABSTRACT Background Mitofusin 2 (MFN2) is a major causative gene for axonal Charcot – Marie – Tooth disease type 2A (CMT2A), with a wide phenotypic spectrum. Comprehensive large ‐ scale genotype – phenotype association studies are essential for understanding disease pathogenesis and improved clinical management.
Masahiro Ando+13 more
wiley +1 more source
Aspects on extracorporeal life support for severe acute respiratory failure with special reference to the influenza A/H1N1 2009 pandemic [PDF]
Extracorporeal membrane oxygenation provides pulmonary and circulatory support. First abandoned from the majority of the intensive care community because of disappointing results in several trials (with questionable methods), it experienced a ...
Holzgraefe, Bernhard
core +1 more source
Scoliosis Surgery in a Patient With Advanced Friedreich's Ataxia—It Is Not Too Late
ABSTRACT Friedreich's ataxia is a multisystem disorder with scoliosis being the most common non‐neurological manifestation. While scoliosis surgery is typically performed in adolescent, ambulatory patients, few data exist on surgical outcomes in patients with advanced disease.
Kathrin Reetz+20 more
wiley +1 more source
Weaning from the ventilator in patients with respiratory failure [PDF]
Weaning from the ventilator is the gradual withdrawal of mechanical ventilatory support. Mechanical ventilation is well-accepted as rescue therapy in patients with life-threatening respiratory failure.
Berg, B.W. (Bart) van den
core
Objective Systemic sclerosis (SSc) is a rare autoimmune disease characterized by tissue fibrosis, vasculopathy, and immune dysregulation. Our objectives were to quantify the overall and site‐specific cancer risks in patients with SSc compared to the general population, examine temporal trends in cancer incidence following SSc diagnosis, and explore ...
Jihyun Na+4 more
wiley +1 more source
Background and Aim. The relative frequency of histological subtypes of lung cancer in Europe has changed dramatically during the 20th century. The aim of this study was to explore the changing epidemiology of lung cancer in Northern Greece over the last ...
T. Kontakiotis+9 more
doaj +1 more source
Objective A leading cause of death among scleroderma (SSc) patients, interstitial lung disease (ILD) remains challenging to prognosticate. The discovery of biomarkers that accurately determine which patients would benefit from close monitoring and aggressive therapy would be an essential clinical tool.
Cristina M Padilla+13 more
wiley +1 more source