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PLoS One 2021 31;16(3):e0249472. Epub 2021 Mar 31.

Division of Rehabilitation Science, Department of Rehabilitation Medicine, University of Minnesota, Minneapolis, Minnesota, United States of America.

Female carriers of Duchenne muscular dystrophy (DMD) presenting with DMD symptomology similar to males with DMD, such as skeletal muscle weakness and cardiomyopathy, are termed manifesting carriers. There is phenotypic variability among manifesting carriers including the age of onset, which can range from the first to fourth decade of life. In females, estrogen levels typically begin to decline during the fourth decade of life and estrogen deficiency contributes to loss of muscle strength and recovery of strength following injury. Read More

J Anesth 2021 Mar 29. Epub 2021 Mar 29.

Department of Anesthesia and Pain Medicine, Pusan National University, Yangsan Hospital, 20 Geumo-ro, Beomeo-ri, Mulgeumeup, Yangsan, 50612, Republic of Korea.

Management of Duchenne muscular dystrophy (DMD) cardiomyopathy is increasingly important for the survival of these patients. Left ventricular assist device (LVAD) is an alternative treatment for refractory heart failure in DMD. A 20-year-old man with DMD and dilated cardiomyopathy underwent surgery for LVAD implantation. Read More

Open Heart 2021 Mar;8(1)

Pediatrics, Division of Pediatric Cardiology, Riley Hospital for Children at Indiana University Health, Indianapolis, Indiana, USA.

Cardiac disease is now the leading cause of death in Duchenne muscular dystrophy (DMD). Clinical evaluations over time have demonstrated asymptomatic cardiac troponin elevations and acute elevations are associated with symptoms and cardiac dysfunction in DMD. Clinicians require a better understanding of the relationship of symptoms, troponin levels and progression of cardiac disease in DMD. Read More

Eur Heart J 2021 Mar 22. Epub 2021 Mar 22.

APHP, Cochin Hospital, Cardiology Department, FILNEMUS, Paris-Descartes, Sorbonne Paris Cité University, Paris, France.

Aims: To estimate the effect of prophylactic angiotensin-converting enzyme inhibitors (ACEi) on survival in Duchenne muscular dystrophy (DMD).

Methods And Results: We analysed the data from the French multicentre DMD Heart Registry (ClinicalTrials.gov: NCT03443115). Read More

Biomedicines 2021 Feb 20;9(2). Epub 2021 Feb 20.

SU, INSERM UMRS974, AIM, Center of Research in Myology, Pitié-Salpêtrière Hospital, 75013 Paris, France.

In skeletal muscle, long noncoding RNAs (lncRNAs) are involved in dystrophin protein stabilization but also in the regulation of myocytes proliferation and differentiation. Hence, they could represent promising therapeutic targets and/or biomarkers for Duchenne and Becker muscular dystrophy (DMD/BMD). DMD and BMD are X-linked myopathies characterized by a progressive muscular dystrophy with or without dilatative cardiomyopathy. Read More