Evidence for Beneficial Effect of Daily Use of Mechanical Insufflation-Exsufflation in Patients With Neuromuscular Diseases.

Background: Daily application of mechanical insufflation-exsufflation (MI-E) is used increasingly in patients with neuromuscular diseases (NMDs) to prevent pulmonary congestion and thereby respiratory tract infections, although its beneficial effect remains uncertain. We, therefore, conducted a systematic review, registered in PROSPERO (CRD42020158278), to compile available evidence for daily MI-E use in subjects with NMDs and stable respiratory condition.

Methods: We performed a systematic comprehensive search of MEDLINE, Embase, CINAHL, and Web of Science up to December 23, 2021.

Short-term effect of air stacking and mechanical insufflation-exsufflation on lung function in patients with neuromuscular diseases.

Air stacking (AS) and mechanical insufflation-exsufflation (MI-E) aim to increase cough efficacy by augmenting inspiratory lung volumes in patients with neuromuscular diseases (NMDs). We studied the short-term effect of AS and MI-E on lung function. We prospectively included NMD patients familiar with daily AS or MI-E use.

Short-term effect and effect on rate of lung function decline after surgery for neuromuscular or syndromic scoliosis.

Introduction: Understanding the impact of scoliosis surgery on lung function is important for counseling patients about risks and benefits of surgery. We prospectively compared the trends in lung function test (LFT) results before and after scoliosis surgery in children with neuromuscular diseases or dysmorphic syndromes. We hypothesized a stabilization.

Coping with paediatric illness: Child's play? Exploring the effectiveness of a play- and sports-based cognitive behavioural programme for children with chronic health conditions.

Little is known about how play affects the development of children with a chronic condition. Studying play poses major methodological challenges in measuring differences in play behaviour, which results in a relative scarcity of research on this subject. This pilot study seeks to provide novel directions for research in this area.

Effect of mechanical insufflation-exsufflation in children with neuromuscular weakness.

Introduction: Children with neuromuscular diseases develop cough impairment. Airway clearance techniques (ACTs) may help to prevent recurrent respiratory tract infections (RTIs). A commonly used ACT is mechanical insufflation-exsufflation (MI-E), but evidence for efficacy is limited.

Exercise responses in children and adults with a Fontan circulation at simulated altitude.

Background: Traveling to high altitude has become more popular. High-altitude exposure causes hypobaric hypoxia. Exposure to acute high altitude, during air travel or mountain stays, seems to be safe for most patients with congenital heart disorders (CHD).

RV adaptation to increased afterload in congenital heart disease and pulmonary hypertension.

Background: The various conditions causing a chronic increase of RV pressure greatly differ in the occurrence of RV failure, and in clinical outcome. To get a better understanding of the differences in outcome, RV remodeling, longitudinal function, and transverse function are compared between patients with pulmonary stenosis (PS), those with a systemic RV and those with pulmonary hypertension (PH).

Materials And Methods: This cross-sectional study prospectively enrolled subjects for cardiac magnetic resonance imaging (CMR), functional echocardiography and cardiopulmonary exercise testing.

Tricuspid flow and regurgitation in congenital heart disease and pulmonary hypertension: comparison of 4D flow cardiovascular magnetic resonance and echocardiography.

Background: Tricuspid valve (TV) regurgitation (TR) is a common complication of pulmonary hypertension and right-sided congenital heart disease, associated with increased morbidity and mortality. Estimation of TR severity by echocardiography and conventional cardiovasvular magnetic resonance (CMR) is not well validated and has high variability. 4D velocity-encoded (4D-flow) CMR was used to measure tricuspid flow in patients with complex right ventricular (RV) geometry and varying degrees of TR.

CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis: A Cross-sectional Study.

Rationale: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with cystic fibrosis may be an important determinant of maximal exercise capacity in cystic fibrosis. Previous studies on the relationship between CFTR genotype and maximal exercise capacity are scarce and contradictory.

Feasibility of Hypoxic Challenge Testing in Children and Adolescents with Congenital Heart and Lung Disease.

Background: This is a cross-sectional observational study to investigate the safety and feasibility of integrating changing body positions and physical activity in a hypoxic challenge test (HCT). The secondary objective was to compare oxygen saturation (Spo2) in two different locations (forehead and finger).

Methods: Included were 12 pediatric to young adult patients with congenital heart (N = 7) or lung disease (N = 5).

Response profiles of oxygen uptake efficiency during exercise in healthy children.

Background: Oxygen uptake efficiency (OUE), the relation between oxygen uptake (VO2) and minute ventilation (VE), differs between healthy children and children with heart disease. This study aimed to investigate the normal response profiles of OUE during a progressive cardiopulmonary exercise test.

Design: Cross-sectional.

Statement on Exercise Testing in Cystic Fibrosis.

This statement summarizes the information available on specific exercise test protocols and outcome parameters used in patients with cystic fibrosis (CF) and provides expert consensus recommendations for protocol and performance of exercise tests and basic interpretation of results for clinicians. The conclusions were reached employing consensus meetings and a wide-band Delphi process. Although data on utility are currently limited, standardized exercise testing provides detailed information on physiological health, allows screening for exercise-related adverse reactions and enables exercise counselling.

Physical activity assessment in cystic fibrosis: A position statement.

Background: The aim of this position statement was to inform the choice of physical activity tools for use within CF research and clinical settings.

Methods: A systematic review of physical activity tools to explore evidence for reliability, validity, and responsiveness. Narrative answers to "four key questions" on motion sensors, questionnaires and diaries were drafted by the core writing team and then discussed at the Exercise Working Group in ECFS Lisbon 2013.

Ventilatory response to exercise in adolescents with cystic fibrosis and mild-to-moderate airway obstruction.

Data regarding the ventilatory response to exercise in adolescents with mild-to-moderate cystic fibrosis (CF) are equivocal. This study aimed to describe the ventilatory response during a progressive cardiopulmonary exercise test (CPET) up to maximal exertion, as well as to assess the adequacy of the ventilatory response for carbon dioxide (CO2) exhalation. Twenty-two adolescents with CF (12 boys and 10 girls; mean ± SD age: 14.

Comparing four non-invasive methods to determine the ventilatory anaerobic threshold during cardiopulmonary exercise testing in children with congenital heart or lung disease.

Background: The ventilatory anaerobic threshold (VAT) is an important method to assess the aerobic fitness in patients with cardiopulmonary disease. Several methods exist to determine the VAT; however, there is no consensus which of these methods is the most accurate.

Objective: To compare four different non-invasive methods for the determination of the VAT via respiratory gas exchange analysis during a cardiopulmonary exercise test (CPET).

Prediction of mortality in adolescents with cystic fibrosis.

Introduction: Lung function, nutritional status, and parameters of exercise capacity are known predictors of mortality in patients with cystic fibrosis (CF). The aim of the current study was to use these important parameters to develop a multivariate model to predict mortality in adolescent patients with CF.

Methods: A total of 127 adolescents with CF (57 girls) with a mean age of 12.

Merits of exercise therapy before and after major surgery.

Purpose Of Review: Advances in medical care have led to an increasing elderly population. Elderly individuals should be able to participate in society as long as possible. However, with an increasing age their adaptive capacity gradually decreases, specially before and after major life events (like hospitalization and surgery) making them vulnerable to reduced functioning and societal participation.

Estimating peak oxygen uptake in adolescents with cystic fibrosis.

Objectives: To predict peak oxygen uptake (VO2 peak) from the peak work rate (W peak) obtained during a cycle ergometry test using the Godfrey protocol in adolescents with cystic fibrosis (CF), and assess the accuracy of the model for prognostication clustering.

Methods: Out of our database of anthropometric, spirometric and maximal exercise data from adolescents with CF (N=363; 140 girls and 223 boys; age 14.77 ± 1.

Effect of inspiratory muscle training before cardiac surgery in routine care.

Background: Inspiratory muscle training (IMT) before cardiac surgery has proved to be a promising intervention to reduce postoperative pneumonia in a randomized controlled trial setting. Effects of IMT in routine care have not been reported.

Objective: The purpose of this study was to investigate the effect of IMT before cardiac surgery on postoperative pneumonia in routine care at a Dutch university medical center using propensity scoring.

Preoperative physical therapy for elective cardiac surgery patients.

Background: After cardiac surgery, physical therapy is a routine procedure delivered with the aim of preventing postoperative pulmonary complications.

Objectives: To determine if preoperative physical therapy with an exercise component can prevent postoperative pulmonary complications in cardiac surgery patients, and to evaluate which type of patient benefits and which type of physical therapy is most effective.

Search Methods: Searches were run on the Cochrane Central Register of Controlled Trials (CENTRAL) on the Cochrane Library (2011, Issue 12 ); MEDLINE (1966 to 12 December 2011); EMBASE (1980 to week 49, 2011); the Physical Therapy Evidence Database (PEDro) (to 12 December 2011) and CINAHL (1982 to 12 December 2011).

Validity of the oxygen uptake efficiency slope in children with cystic fibrosis and mild-to-moderate airflow obstruction.

Purpose: The oxygen uptake efficiency slope (OUES) has been proposed as an 'effort-independent' measure of cardiopulmonary exercise capacity, which could be used as an alternative measurement for peak oxygen uptake (VO(2peak)) in populations unable or unwilling to perform maximal exercise. The aim of the current study was to investigate the validity of the OUES in children with cystic fibrosis (CF).

Methods: Exercise data of 22 children with CF and mild to moderate airflow obstruction were analyzed and compared with exercise data of 22 healthy children.

Oxygen uptake efficiency slope in healthy children.

The objective of this study was to investigate the characteristics of the submaximal Oxygen Uptake Efficiency Slope (OUES) in a healthy pediatric population. Bicycle ergometry exercise tests with gas-analyses were performed in 46 healthy children aged 7-17 years. Maximal OUES, submaximal OUES, VO(2)peak, V(E)peak, and ventilatory threshold (VT) were determined.

Exercise stress testing in children with metabolic or neuromuscular disorders.

The role of exercise as a diagnostic or therapeutic tool in patients with a metabolic disease (MD) or neuromuscular disorder (NMD) is relatively underresearched. In this paper we describe the metabolic profiles during exercise in 13 children (9 boys, 4 girls, age 5-15 yrs) with a diagnosed MD or NMD. Graded cardiopulmonary exercise tests and/or a 90-min prolonged submaximal exercise test were performed.