Due to respiratory weakness, late-stage Duchenne muscular dystrophy (DMD) patients may suffer from chronic aspiration, which is sometimes treated using tracheostomy. However, definitive laryngectomy has not been described in the literature as an aspiration prevention modality in DMD, especially in patients with contraindications to tracheostomy. A case is presented for a patient with advanced stage Duchenne muscular dystrophy suffering from chronic aspiration pneumonia and excessive oral secretions who became ventilator dependent. A tracheostomy was placed, but was noted to have excessive secretions and high cuff pressures, which have been known to be associated with worsened swallow dysfunction as well as tracheoinnominate fistula. The patient therefore was considered for total laryngectomy, which he underwent successfully. Post-operatively, the patient was noted to have improved subjective quality of life, engaged in an oral diet, and had less secretions surrounding his tracheostoma post-operatively. Aspiration prevention surgeries are done to improve quality of life by improving oral intake, decreasing the need for frequent suctioning, and can sometimes allow for speech. It is important to consider quality of life for DMD patients as more of these patients are living into their 30s with the aid of mechanical ventilation. Laryngectomy is a surgery that can definitively correct chronic aspiration while allowing for oral intake. Laryngoscope, 134:3677-3678, 2024.
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http://dx.doi.org/10.1002/lary.31357 | DOI Listing |
Geroscience
January 2025
Department of Physical Medicine and Rehabilitation, University of Missouri, Columbia, MO, USA.
Sarcopenia, the pathological age-related loss of muscle mass and strength, contributes to physical decline, frailty, and diminished healthspan. The impact of sarcopenia is expected to rise as the aging population grows, and treatments remain limited. Therefore, novel approaches for enhancing physical function and strength in older adults are desperately needed.
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January 2025
John Walton Muscular Dystrophy Research Centre, Newcastle University, Newcastle upon Tyne, UK; Newcastle Hospitals NHS Foundation Trusts, Newcastle upon Tyne, UK. Electronic address:
Cardiomyopathy is a common co-morbidity in individuals with Duchenne muscular dystrophy (DMD). This retrospective single centre study investigated the relationship between age at loss of ambulation (LOA) and late stage left ventricular ejection fraction (LVEF) in 84 individuals (> 16 years old) with DMD taking glucocorticoid and ACE inhibitors treatment. Regression analyses showed a positive correlation between later age at LOA and higher LVEF in adulthood (linear regression estimate 1.
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BridgeBio Gene Therapy, Palo Alto, California, USA.
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January 2025
Department of Integrative Biology and Physiology, University of California, Los Angeles, CA 90095-1606; Molecular, Cellular & Integrative Physiology Program, University of California, Los Angeles, CA 90095-1606; Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, University of California, Los Angeles, CA 90095. Electronic address:
Duchenne muscular dystrophy (DMD) is a lethal, muscle-wasting, genetic disease that is greatly amplified by an immune response to the diseased muscles. The mdx mouse model of DMD was used to test whether the pathology can be reduced by treatments with a CTLA4-Ig fusion protein that blocks costimulatory signals required for activation of T-cells. CTLA4-Ig treatments reduced mdx sarcolemma lesions and reduced the numbers of activated T-cells, macrophages and antigen presenting cells in mdx muscle and reduced macrophage invasion into muscle fibers.
View Article and Find Full Text PDFPLoS One
January 2025
Little Steps Association for Children with Duchenne Muscular Dystrophy and Becker Muscular Dystrophy, Kefar Saba, Israel.
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