Pulmonary function in Duchenne muscular dystrophy related to stage of disease

Inkley, Scott R.; Oldenburg, Frederick C.; Vignos, Paul J.

doi:10.1016/0002-9343(74)90611-1

Cited by 205 publications

(91 citation statements)

References 15 publications

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“…PA-ao 2 = ((barometric pressure-47 × FIO 2 ) -(PaCO 2 /R)) -PaO 2 The respiratory quotient (R) was assumed to be 0.8; fractional inspiratory oxygen (FIO 2 ) was always 0.21 (room air). On the same night a polysomnographic recording was obtained.…”

Section: Protocolmentioning

confidence: 99%

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Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy

Barbé¹,

Quera-Salva²,

McCann³

et al. 1994

Eur Respir J

132

125

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S Sl le ee ep p--r re el la at te ed d r re es sp pi ir ra at to or ry y d di is st tu ur rb ba an nc ce es s i in n p pa at ti ie en nt ts s w wi it th h D Du uc ch he en nn ne e m mu us sc cu ul la ar r d dy ys st tr ro op ph hy y Four patients (67%) showed symptoms that suggest sleep-related respiratory disturbances. At night, the apnoea-hypopnoea index (AHI) was 11±6. The patients with more symptoms during the daytime had the highest AHI scores. Most of the apnoeas (85%) were central, particularly during rapid eye movement (REM) sleep. Sleep architecture was well-preserved. Arterial desaturation (>5% below baseline) occurred during 25±23% of total time. AHI correlated with daytime PaO 2 , and AHI in REM sleep correlated with age. A stepwise multivariate analysis showed that PaO 2 and, to some extent, the degree of airflow obstruction were significantly correlated with AHI.We conclude that sleep-related respiratory disturbances are frequently present in patients with Duchenne muscular dystrophy. Therefore, physicians should look for symptoms related to sleep-related respiratory disturbances in these patients. Furthermore, sleep-related respiratory disturbances should be strongly suspected in older Duchenne muscular dystrophy patients, particularly if diurnal arterial hypoxaemia is concurrently present.

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Section: Protocolmentioning

confidence: 99%

“…Patients generally die before the age of 20 yrs, mostly because of respiratory complications [2]. These include mucus retention, repeated respiratory infections and impaired gas exchange [3].…”

mentioning

confidence: 99%

Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy

Barbé¹,

Quera-Salva²,

McCann³

et al. 1994

Eur Respir J

132

125

View full text Add to dashboard Cite

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“…1 Approximately 90% of patients with Duchenne muscular dystrophy who do not use ventilatory support die from pulmonary complications associated with respiratory muscle weakness between 16 and 19 years of age, and uncommonly after age 25. [2][3][4][5][6] Ninety percent of episodes of pneumonia and acute respiratory SEE THE RELATED EDITORIAL ON PAGE 878 failure (ARF) that require intubation occur during otherwise benign upper-respiratory infection, mainly because of inability to cough effectively. 7 If unable to wean from the ventilator, these intubated patients conventionally undergo tracheotomy for ongoing tracheostomy ventilation.…”

Section: Introductionmentioning

confidence: 99%

Duchenne Muscular Dystrophy: Continuous Noninvasive Ventilatory Support Prolongs Survival

2011

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OBJECTIVE: To describe survival outcomes with noninvasive ventilation (NIV) for full ventilatory support, and a mechanically assisted cough and oximetry protocol in a series of patients with Duchenne muscular dystrophy. METHODS: We monitored end-tidal carbon dioxide (P ETCO 2 ), S pO 2 , vital capacity, maximum insufflation capacity, and cough peak flow. Nocturnal NIV was initiated for symptomatic hypoventilation. An oximeter and mechanically assisted cough device were prescribed when the patient's maximum assisted cough peak flow fell below 300 L/min. Patients used up to continuous NIV and mechanically assisted cough to return S pO 2 to > 95% during intercurrent respiratory infections or as otherwise needed. We recorded respiratory and cardiac hospitalizations and mortality, and quantified survival by duration of continuous NIV dependence (ie, unable to maintain oxygenation without the ventilator). RESULTS: With advancing Duchenne muscular dystrophy, 101 nocturnal-only NIV users extended their NIV use throughout the daytime hours and required it continuously for 7.4 ؎ 6.1 years to 30.1 ؎ 6.1 years of age, with 56 patients still alive. Twenty-six of the 101 became continuously dependent without requiring hospitalization. Eight tracheostomized users were decannulated to NIV. Thirty-one consecutive unweanable intubated patients were extubated to NIV plus mechanically assisted cough. Of the 67 deaths (including 8 patients who died from heart failure before requiring ventilator use), 34 (52%) were probably cardiac, 14 (21%) were probably respiratory, and 19 (27%) were of unknown or other etiology. CONCLUSIONS: Continuous NIV along with mechanically assisted cough and oximetry as needed can prolong life and obviate tracheotomy in patients with Duchenne muscular dystrophy. Unweanable patients can be decannulated and extubated to NIV plus mechanically assisted cough.

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“…This failure is most commonly seen in Duchenne dystrophy, but sometimes occurs in Becker, limb-girdle, and facioscapulohumeral dystrophies, in which respiratory failure also parallels more severe locomotor incapacity [1,2]. We present two brothers with a myopathic disease in which the distribution of weakness, clinical course, heredity, and muscle pathology most closely resembled a limb-girdle type of dystrophy.…”

Section: Introductionmentioning

confidence: 98%

Chronic respiratory failure in limb-girdle muscular dystrophy: Successful long-term therapy with nasal bilevel positive airway pressure

Robertson

Roloff

1994

Pediatric Neurology

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Chronic respiratory failure is a major factor contributing to mortality in progressive neuromuscular disorders. Among the muscular dystrophies, respiratory failure most commonly occurs with Duchenne dystrophy, while in Becker, limb-girdle, and facioscapulohumeral dystrophies, respiratory failure is infrequent and generally occurs in the more severe cases that have progressed to a nonambulatory, advanced functional stage. We report two brothers with a myopathic disease in which the distribution of weakness, initial clinical course, heredity, and muscle pathology most closely resembled a limb-girdle type of dystrophy. Both brothers, however, presented with chronic alveolar hypoventilation and respiratory failure when their locomotor disabilities were still mild. Respiratory failure was reversed, and satisfactory ventilation has been maintained for more than a year using a type of noninvasive intermittent positive pressure ventilation, with a bilevel positive airway pressure device (Bi-PAP), administered through a nasal mask during sleeping hours. These cases demonstrate an unusual presentation of limb-girdle dystrophy, and document that nocturnal, nasal administration of continuous airway pressure using the Bi-PAP device may be sufficient to maintain adequate long-term ventilation in some patients with neuromuscular causes of respiratory failure, and thus significantly improve quality of life and delay the need for more complex or invasive forms of assisted ventilation.

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Pulmonary function in Duchenne muscular dystrophy related to stage of disease

Cited by 205 publications

References 15 publications

Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy

Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy

Duchenne Muscular Dystrophy: Continuous Noninvasive Ventilatory Support Prolongs Survival

Chronic respiratory failure in limb-girdle muscular dystrophy: Successful long-term therapy with nasal bilevel positive airway pressure

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