scholarly journals A review of respiratory manifestations and their management in Ehlers-Danlos syndromes and hypermobility spectrum disorders

2021 ◽  
Vol 18 ◽  
pp. 147997312110253
Author(s):  
Karan Chohan ◽  
Nimish Mittal ◽  
Laura McGillis ◽  
Laura Lopez-Hernandez ◽  
Encarna Camacho ◽  
...  
Keyword(s):  
Sleep Apnea ◽  
Muscle Function ◽  
Respiratory Muscle ◽  
Respiratory Symptoms ◽  
Management Strategies ◽  
Narrative Review ◽  
Respiratory Muscle Function ◽  
Respiratory Management ◽  
Spectrum Disorders ◽  
Continuous Positive Pressure

Background: Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD) are a heterogeneous group of heritable genetic connective tissue disorders with multiple characteristics including joint hypermobility, tissue fragility, and multiple organ dysfunction. Respiratory manifestations have been described in EDS patients, but have not been systematically characterized. A narrative review was undertaken to describe the respiratory presentations and management strategies of individuals with EDS and HSD. Methods: A broad literature search of Medline, Embase, Cochrane Database of Systematic Reviews, and Cochrane CENTRAL was undertaken from inception to November 2020 of all study types, evaluating EDS/ HSD and pulmonary conditions. This narrative review was limited to adult patients and publications in English. Results: Respiratory manifestations have generally been described in hypermobile EDS (hEDS), classical and vascular EDS subtypes. Depending on EDS subtype, they may include but are not limited to dyspnea, dysphonia, asthma, sleep apnea, and reduced respiratory muscle function, with hemothorax and pneumothorax often observed with vascular EDS. Respiratory manifestations in HSD have been less frequently characterized in the literature, but exertional dyspnea is the more common symptom described. Respiratory symptoms in EDS can have an adverse impact on quality of life. The respiratory management of EDS patients has followed standard approaches with thoracotomy tubes and pleurodesis for pleural manifestations, vocal cord strengthening exercises, continuous positive pressure support for sleep apnea, and exercise training. Reduced respiratory muscle function in hEDS patients responds to inspiratory muscle training. Conclusion: Respiratory symptoms and manifestations are described in EDS and HSD, and have generally been managed using conservative non-surgical strategies. Research into the prevalence, incidence and specific respiratory management strategies in EDS and HSD is needed to mitigate some of the associated morbidity.

Respiratory Muscle Function during Obstructive Sleep Apnea

1990 ◽  
Vol 142 (3) ◽  
pp. 533-539 ◽  
Author(s):  
Pearce G. Wilcox ◽  
Peter D. Paré ◽  
Jeremy D. Road ◽  
John A. Fleetham
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Muscle Function ◽  
Respiratory Muscle ◽  
Respiratory Muscle Function ◽  
Obstructive Sleep

scholarly journals Respiratory muscle function in the newborn: a narrative review

2021 ◽  
Author(s):  
Theodore Dassios ◽  
Aggeliki Vervenioti ◽  
Gabriel Dimitriou
Keyword(s):  
Congenital Anomalies ◽  
Muscle Function ◽  
Respiratory Muscle ◽  
Work Of Breathing ◽  
Respiratory Muscles ◽  
Current Evidence ◽  
Muscle Fibres ◽  
List Type ◽  
Respiratory Muscle Function ◽  
Neurally Adjusted Ventilator Assist

Abstract Our aim was to summarise the current evidence and methods used to assess respiratory muscle function in the newborn, focusing on current and future potential clinical applications. The respiratory muscles undertake the work of breathing and consist mainly of the diaphragm, which in the newborn is prone to dysfunction due to lower muscle mass, flattened shape and decreased content of fatigue-resistant muscle fibres. Premature infants are prone to diaphragmatic dysfunction due to limited reserves and limited capacity to generate force and avoid fatigue. Methods to assess the respiratory muscles in the newborn include electromyography, maximal respiratory pressures, assessment for thoraco-abdominal asynchrony and composite indices, such as the pressure–time product and the tension time index. Recently, there has been significant interest and a growing body of research in assessing respiratory muscle function using bedside ultrasonography. Neurally adjusted ventilator assist is a novel ventilation mode, where the level of the respiratory support is determined by the diaphragmatic electrical activity. Prolonged mechanical ventilation, hypercapnia and hypoxia, congenital anomalies and systemic or respiratory infection can negatively impact respiratory muscle function in the newborn, while caffeine and synchronised or volume-targeted ventilation have a positive effect on respiratory muscle function compared to conventional, non-triggered or pressure-limited ventilation, respectively. Impact Respiratory muscle function is impaired in prematurely born neonates and infants with congenital anomalies, such as congenital diaphragmatic hernia. Respiratory muscle function is negatively affected by prolonged ventilation and infection and positively affected by caffeine and synchronised compared to non-synchronised ventilation modes. Point-of-care diaphragmatic ultrasound and neurally adjusted ventilator assist are recent diagnostic and therapeutic technological developments with significant clinical applicability.

scholarly journals Pulmonary and respiratory muscle function in response to 10 marathons in 10 days

2018 ◽  
Vol 119 (2) ◽  
pp. 509-518 ◽  
Author(s):  
Nicholas B. Tiller ◽  
Louise A. Turner ◽  
Bryan J. Taylor
Keyword(s):  
Muscle Function ◽  
Respiratory Muscle ◽  
Respiratory Muscle Function

Respiratory muscle function during a six-week period of normocapnic hyperpnoea training

2013 ◽  
Vol 188 (2) ◽  
pp. 208-213 ◽  
Author(s):  
David Johannes Walker ◽  
Thomas Ertl ◽  
Stephan Walterspacher ◽  
Daniel Schlager ◽  
Kai Roecker ◽  
...  
Keyword(s):  
Muscle Function ◽  
Respiratory Muscle ◽  
Respiratory Muscle Function
Sign in / Sign up

Export Citation Format

Share Document