Perioperative Evaluation of Respiratory Muscle Strength after Scoliosis Correction in Patients with Duchenne Muscular Dystrophy.
Conclusions: By focusing on respiratory muscle strength, our results suggest that scoliosis correction in patients with DMD might have a favorable effect on respiratory function. PMID: 29093790 [PubMed]
ConclusionsTc-MEP and SSEP monitoring is feasible, particularly when Tc-MEPs are recorded from the abductor hallucis muscle in patients with DMD. Similar TMS/CMAP and Tc-MEP/CMAP ratios show that there were no differences observed in cortical excitability between the groups. IONM seems a feasible and valuable neurophysiological tool to signal possible surgically induced damage to the spinal cord during scoliosis correction surgery in patients with DMD.
Conclusions: Deflazacort presents an additional, FDA-approved corticosteroid option for patients that offers improved quality of life for DMD patients. However, there is weak evidence to support these benefits; a full risk-benefit analysis considering adverse events, efficacy, cost, and previous trials of steroid therapy is necessary when selecting therapy. Further research will help clarify deflazacort's optimal dose, duration of treatment, and impact on quality of life. PMID: 32019318 [PubMed - as supplied by publisher]
Rationale: Facioscapulohumeral muscular dystrophy (FSHD) is the third most common muscular dystrophy, which is associated with facial, shoulder girdle, and paraspinal muscle atrophy. Most of the patients develop hypokyphosis and hyperlordosis in the course of the disease, to preserve standing posture. Corrective fusion is contraindicated in these patients as the surgery results with loss of compensatory hyperlordosis and leads to loss of trunk balance while standing. Although spinal fusion in neuromuscular scoliosis is a known treatment option, there are no studies in the literature on the spinal fusion of this specific ...
CONCLUSION: There is a need to improve awareness and provision of palliative care services for DMD patients in Singapore where discussion of end-of-life care is often considered taboo. Prevention and correction of scoliosis and provision of appropriate ventilatory support may improve quality of life in DMD patients. PMID: 32246708 [PubMed - as supplied by publisher]
Conclusion: This single center study adds to the real-world evidence associating deflazacort with improved clinical outcomes. PMID: 31922454 [PubMed - as supplied by publisher]
ConclusionPublished health state utilities document the substantial HRQoL impacts of DMD, particularly with disease progression. Additional research in patient utilities for additional health states, particularly in non-ambulatory DMD patients, is warranted.
This article reviews the epidemiology, clinical manifestations, genetic cause, management, and new and emerging therapies for this condition. RECENT FINDINGS New studies have highlighted how oral corticosteroids have changed the natural history of the disease, prolonging ambulation in boys with Duchenne muscular dystrophy and reducing the risk of developing scoliosis and subsequent surgical correction, improving cardiac health, and increasing long-term survival. Additionally, recent publications have provided insights into how newer and emerging treatment options are becoming more common for this condition. With gene th...
Conclusions: DMD is a rare disease in our area.57,8% of patients require MV and Cough assist and are controlled by pulmonologistsMainly MV starts in the adolescence and in 45,4% of cases after an acute process.Recently, a process coordination between Pediatrics and Pulmonologists has begun to improve the care of these patients.
CONCLUSIONS: Since there were no RCTs or quasi-RCTs available to evaluate the effectiveness of standing devices in people with DMD, studies are needed to investigate the effectiveness of standing devices in this population. PMID: 31606891 [PubMed - as supplied by publisher]
Several types of coagulation disorders are reported in patients with Duchenne muscular dystrophy (DMD). Those include repeated reports of increased rate of bleeding-complications following scoliosis surgery but also reports of thromboembolic complications. Personalized survey-links were forwarded to 682 registered patients with a genetically confirmed diagnosis of DMD via the German DMD-registry of the Treat-NMD-network. Data on the degree of mobility, disposition to hematoma, epistaxis and gum bleeding, occurrence of postsurgical hemorrhage, stroke, deep vein thrombosis, cardiac thromboembolism, long-term medication and a...