Dmd – clinical care

This study aims to investigate whether children with Duchenne muscular dystrophy (DMD) have sleep disorders in addition to evaluate the sleep quality and daytime sleepiness in mothers who are primary caregivers using questionnaires. Demographic and clinical data in addition to gross motor functional status of 24 patients with a DMD diagnosis were measured using gross motor function classification system (GMFCS). The patients were given the sleep disturbance scale for children (SDSC) in order to evaluate their sleep features.
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research

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Journal of Clinical Sleep Medicine,Volume 17, Issue 10, Page 1973-1980, October 2021.
Source: Journal of Clinical Sleep Medicine : JCSM - Category: Sleep Medicine Authors: Source Type: research
Journal of Clinical Sleep Medicine, Ahead of Print.
Source: Journal of Clinical Sleep Medicine : JCSM - Category: Sleep Medicine Authors: Source Type: research
Although intellectual disability was included in the first description of Duchenne muscular dystrophy (DMD), the cognitive and neurodevelopmental (ND) components of DMD and Becker muscular dystrophy (BMD) remain overshadowed by their motor, cardiac, and pulmonary manifestations. To assess the ND comorbidity burden in dystrophinopathy patients at Le Bonheur Children's Hospital neuromuscular clinic, abstraction of the electronic medical record was performed for 46 patients. Diagnoses of ADHD, epilepsy, autism spectrum, obsessive compulsive, oppositional defiant, mood, anxiety, and sleep disorders, along with discussion of an...
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
In conclusion, our study demonstrated that elevated cumulative SBP or DBP was independently associated with increased risk of CVD in the Chinese population. Among participants with 15-year cumulative BP levels higher than the median, that is, 1970.8/1239.9 mmHg-year for cumulative SBP/DBP, which was equivalent to maintaining SBP/DBP level higher than 131/83 mmHg in 15 years, the CVD risk would increase significantly irrespective of whether or not the BP measurements at one examination was high. Our findings emphasize the importance of cumulative BP level in identifying individuals with high risk of CVD in the future. ...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Myotonic dystrophy type 1 is the most common form of muscular dystrophy in adults, and is primarily characterized by muscle weakness and myotonia, yet some of the most disabling symptoms of the disease are cognitive and behavioral. Here we evaluated several of these non-motor symptoms from a cross-sectional time-point in one of the largest longitudinal studies to date, including full-scale intelligence quotient, depression, anxiety, apathy, sleep, and cerebral white matter fractional anisotropy in a group of 39 adult-onset myotonic dystrophy type 1 participants (27 female) compared to 79 unaffected control participants (46...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Journal of Clinical Sleep Medicine, Ahead of Print.
Source: Journal of Clinical Sleep Medicine : JCSM - Category: Sleep Medicine Authors: Source Type: research
The MDCC is a Federal Advisory Committee composed of members representing the NIH, CDC, FDA and other agencies as well as patient advocacy groups (please see mdcc.nih.gov for more information). We meet twice a year to discuss topics of interest to our members and the muscular dystrophy research, care and patient/advocacy communities. The main topic for our June meeting will be respiratory and sleep complications.For more information go tohttps://www.mdcc.nih.gov/Air date: 6/2/2021 10:00:00 AM
Source: Videocast - All Events - Category: General Medicine Tags: Upcoming Events Source Type: video
Conclusions: This survey highlighted specifics of LTHNIV in RD, showing that patients with NMD affected by ALS are the biggest group. In comparison to Eurovent, LTHNIV has increased in NMD while it has decreased in CWD. Bi-level PSV-ST,PSV+VT, PCV are the most used modes and oro-nasal mask is the preferred interface.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Noninvasive ventilatory support Source Type: research
Conclusions: In DMD, flows and volumes are significantly lower than predicted since childhood, starting to be halved by the age of 15, while the flow-volume loop area three years earlier. The flow-volume loop area seems to be a promising index to early identify the deterioration of lung function in DMD, since it combined both volume and flow data.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric respiratory physiology and sleep Source Type: research
Background: Current evidence shows that steroids exert a beneficial effect on muscle strength of patients with Duchenne muscular dystrophy.Aim: To investigate the impact of treatment with steroids on lung function of children with Duchenne muscular dystrophy.Methods: Data from medical records of 60 boys with Duchenne was processed. The age at which treatment with steroids was suggested is considered as baseline time. Lung function was evaluated based on %pred values (for age, height and gender) of FVC, FEV1 and PEF.Results: Out of 49 boys initially offered treatment with steroids 29 finally started (14 prednisolone, 7 defl...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric respiratory physiology and sleep Source Type: research
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