Respiratory Care of the Patient with Duchenne Muscular Dystrophy

Finder, Jonathan D.; Birnkrant, David J.; Carl, John; Farber, Harold J.; Gozal, David; Iannaccone, Susan T.; Kovesi, Tom; Kravitz, Richard M.; Panitch, Howard B.; Schramm, Craig M.; Schroth, Mary K.; Sharma, Girish; Sievers, L.; Silvestri, Jean M.; Sterni, Laura M.

doi:10.1164/rccm.200307-885st

Cited by 619 publications

(133 citation statements)

References 104 publications

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“…These changes are similar to those seen in any patient with an underlying neuromuscular weakness, such as Duchenne muscular dystrophy and spinal muscle atrophy 45. Patients with Pompe disease are also at an increased risk for aspiration pneumonia (see GI section below).…”

Section: Pulmonarysupporting

confidence: 58%

“…Both manual and mechanical techniques used in treating other neuromuscular disorders should be used to assist cough and preserve pulmonary function 45,55. Liberal use of bronchodilators should be used in conjunction with these airway clearance techniques and assisted coughing maneuvers (i.e., The Cough Assist In-Exsufflator)60 to maximize the patient’s pulmonary toilet care.…”

Section: Pulmonarymentioning

confidence: 99%

“…Liberal use of bronchodilators should be used in conjunction with these airway clearance techniques and assisted coughing maneuvers (i.e., The Cough Assist In-Exsufflator)60 to maximize the patient’s pulmonary toilet care. Inspiratory muscle training may also be important 45. Bronchodilators and steroids (both inhaled and/or oral) can be useful when an asthmatic process is present.…”

Section: Pulmonarymentioning

confidence: 99%

See 2 more Smart Citations

Pompe disease diagnosis and management guideline

Kishnani

Steiner

Bali

et al. 2006

Genetics in Medicine

495

583

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Section: Pulmonarysupporting

confidence: 58%

Section: Pulmonarymentioning

confidence: 99%

Section: Pulmonarymentioning

confidence: 99%

See 1 more Smart Citation

Pompe disease diagnosis and management guideline

Kishnani

Steiner

Bali

et al. 2006

Genetics in Medicine

495

583

View full text Add to dashboard Cite

“…In this respect, NIPPV may have positive cardioprotective effects in DMD patients with respiratory insufficiency. In accordance with these reports, we treated DMD patients with either full-time or part-time ventilation according to the guidelines for respiratory management [14,15]. …”

Section: Discussionmentioning

confidence: 99%

Outcomes of Cardiac Involvement in Patients with Late-Stage Duchenne Muscular Dystrophy under Management in the Pulmonary Rehabilitation Center of a Tertiary Referral Hospital

Kwon

Kang²,

Kim

et al. 2012

Cardiology

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Objectives: The purpose of this study was to investigate the clinical outcome as well as the sequential changes of cardiac function in late-stage Duchenne muscular dystrophy (DMD) patients by 2-dimensional echocardiography. Methods: A total of 31 individuals (initial age: 21.6 ± 5.0 years, range: 15–35 years) with late-stage DMD (Swinyard-Deaver’s stage 7 or 8) were enrolled. All of these patients had respiratory insufficiency and were on ventilator support. Sequential echocardiographic data were collected over at least 3 years. Repeated measures analysis of variance was used to compare changes in left ventricular ejection fraction (LVEF) over time. Results: The sequential change in the mean LVEF showed no significant differences with initial, 1-, 2-, and 3-year follow-up LVEFs which were 42.2, 42.9, 43.8 and 42.6%, respectively (p = 0.320). In terms of the clinical outcome, all but 1 patient survived during the follow-up period of 46.5 ± 9.1 months. Conclusions: The cardiac function in late-stage DMD patients showed a stabilization of LVEF on adequate ventilatory support and optimal cardiac medication therapy until their mid-30s. In addition, considering the favorable clinical outcome in our study, the process of cardiac involvement in late-stage DMD may demonstrate that in some patients it is nonprogressive.

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“…Respiratory failure is inevitable in DMD 1. Multidisciplinary care and ventilatory support have increased longevity,2, 3 and corticosteroids and idebenone may stabilize the respiratory function in nonambulatory patients 4, 5.…”

Section: Introductionmentioning

confidence: 99%

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

Mankodi

Kovacs

Norato

et al. 2017

Ann Clin Transl Neurol

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ObjectiveTo examine the diaphragm and chest wall dynamics with cine breathing magnetic resonance imaging (MRI) in ambulatory boys with Duchenne muscular dystrophy (DMD) without respiratory symptoms and controls.MethodsIn 11 DMD boys and 15 controls, cine MRI of maximal breathing was recorded for 10 sec. The lung segmentations were done by an automated pipeline based on a Holistically‐Nested Network model (HNN method). Lung areas, diaphragm, and chest wall motion were measured throughout the breathing cycle.ResultsThe HNN method reliably identified the contours of the lung and the diaphragm in every frame of each dataset (~180 frames) within seconds. The lung areas at maximal inspiration and expiration were reduced in DMD patients relative to controls (P = 0.02 and <0.01, respectively). The change in the lung area between inspiration and expiration correlated with percent predicted forced vital capacity (FVC) in patients (r s = 0.75, P = 0.03) and was not significantly different between groups. The diaphragm position, length, contractility, and motion were not significantly different between groups. Chest wall motion was reduced in patients compared to controls (P < 0.01).InterpretationCine breathing MRI allows independent and reliable assessment of the diaphragm and chest wall dynamics during the breathing cycle in DMD patients and controls. The MRI data indicate that ambulatory DMD patients breathe at lower lung volumes than controls when their FVC is in the normal range. The diaphragm moves normally, whereas chest wall motion is reduced in these boys with DMD.

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Respiratory Care of the Patient with Duchenne Muscular Dystrophy

Cited by 619 publications

References 104 publications

Pompe disease diagnosis and management guideline

Pompe disease diagnosis and management guideline

Outcomes of Cardiac Involvement in Patients with Late-Stage Duchenne Muscular Dystrophy under Management in the Pulmonary Rehabilitation Center of a Tertiary Referral Hospital

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

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