Ventilatory parameters and maximal respiratory pressure changes with age in Duchenne muscular dystrophy patients

Gayraud, Jérôme; Ramonatxo, M; Rivier, François; Humberclaude, Véronique; Petrof, Basil J.; Matecki, Stéfan

doi:10.1002/ppul.21204

Cited by 58 publications

(67 citation statements)

References 29 publications

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“…The consequence of the structural tissue damage in DMD patients is an impairment of respiratory function, which has been described as a restrictive ventilatory syndrome [18], [19], [20], [21], [22]. DMD patients show a positive correlation between the rates of decrease in vital capacity (VC) and forced expiratory volume in 1 second (FEV 1 ) and a decrease in maximal inspiratory pressure as the disease progresses [23]. Other factors, such as an alteration of the elastic properties of elastin and collagen fibers, a decrease in surfactant activity and fibrosis reduces the ventilatory system compliance [24], [25], [26], [27].…”

Section: Introductionmentioning

confidence: 99%

Ventilatory Chemosensory Drive Is Blunted in the mdx Mouse Model of Duchenne Muscular Dystrophy (DMD)

et al. 2013

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Duchenne Muscular Dystrophy (DMD) is caused by mutations in the DMD gene resulting in an absence of dystrophin in neurons and muscle. Respiratory failure is the most common cause of mortality and previous studies have largely concentrated on diaphragmatic muscle necrosis and respiratory failure component. Here, we investigated the integrity of respiratory control mechanisms in the mdx mouse model of DMD. Whole body plethysmograph in parallel with phrenic nerve activity recordings revealed a lower respiratory rate and minute ventilation during normoxia and a blunting of the hypoxic ventilatory reflex in response to mild levels of hypoxia together with a poor performance on a hypoxic stress test in mdx mice. Arterial blood gas analysis revealed low PaO2 and pH and high PaCO2 in mdx mice. To investigate chemosensory respiratory drive, we analyzed the carotid body by molecular and functional means. Dystrophin mRNA and protein was expressed in normal mice carotid bodies however, they are absent in mdx mice. Functional analysis revealed abnormalities in Dejours test and the early component of the hypercapnic ventilatory reflex in mdx mice. Together, these results demonstrate a malfunction in the peripheral chemosensory drive that would be predicted to contribute to the respiratory failure in mdx mice. These data suggest that investigating and monitoring peripheral chemosensory drive function may be useful for improving the management of DMD patients with respiratory failure.

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Section: Introductionmentioning

confidence: 99%

Ventilatory Chemosensory Drive Is Blunted in the mdx Mouse Model of Duchenne Muscular Dystrophy (DMD)

et al. 2013

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“…Respiratory impairment in neuromuscular diseases is assessed by the measurement of maximal inspiratory pressure (PImax), slow vital capacity (VC) and, more recently, sniff nasal inspiratory pressure (SNIP). Studies describing longitudinal follow-up of respiratory function for o2 years in children with BMD or DMD have been based on PImax measurements [2][3][4] and lung volumes [2][3][4][5][6][7]. At the time of writing, there was no published prospective study describing longitudinal assessment of inspiratory muscle strength by SNIP measurements.…”

Section: Introductionmentioning

confidence: 99%

Sniff nasal inspiratory pressure in the longitudinal assessment of young Duchenne muscular dystrophy children

et al. 2012

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Traditional measures of respiratory function in children with Duchenne muscular dystrophy (DMD) are based on maximal inspiratory pressure (PImax) and vital capacity (VC). Sniff nasal inspiratory pressure (SNIP) measurements are easily performed by young children with neuromuscular disorders. The clinical value of SNIP in the longitudinal assessment of respiratory weakness remains to be assessed. The objective of the present study was to assess longitudinally the changes in SNIP, PImax and VC with age in DMD children. We hypothesised that their longitudinal assessment would show an earlier decline in SNIP than VC.A 3-year, prospective follow-up, at 6-month intervals of, 33 steroid-naïve, 5-20-year-old DMD patients was analysed using a linear mixed model. SNIP measurements were reliable (within-session coefficient of variation 8%). SNIP and VC increased until 10.5 and 12.5 years of age, respectively, and declined thereafter, while PImax did not change with age.SNIP was an earlier marker of decline in respiratory muscle strength (at 10.5 years) than VC (at 12.5 years) in young DMD patients. SNIP longitudinal assessment is useful in the detection of inspiratory strength decline in young DMD patients when VC values remain within normal values and as an outcome measure in clinical trials for emerging therapeutics in young DMD patients from the age of 5 years. @ERSpublications Earlier sniff nasal inspiratory pressure than vital capacity decline in follow-up of Duchenne muscular dystrophy children

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“…Since skeletal muscle weakness is the main physiological consequence of dystrophin defects in DMD, expiratory muscle function has been proposed as a potential index of disease progression [10]. Maximal respiratory pressures (MRPs) are important values to be measured in a comprehensive respiratory function test, since they are indicators of respiratory muscle strength.…”

Section: Introductionmentioning

confidence: 99%

Analysis of Pulmonary Function Test in Korean Patients With Duchenne Muscular Dystrophy: Comparison of Foreign and Korean Reference Data

et al. 2016

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ObjectiveTo determine the abnormal pulmonary function value in Korean Duchenne muscular dystrophy (DMD) patients, we performed a comparative analysis of the patients' pulmonary function value expressed as % of the overseas reference data and Korean healthy children and adolescent reference data.MethodsWe performed pulmonary function test (PFT) in a total of 27 DMD patients. We compared the patients' FVC% and FEV1% of the overseas reference data with those of the Korean children and adolescent reference data. Also, we compared the patients' MIP% and MEP% of the prediction equation data with those of the Korean children and adolescent reference data.ResultsAge of the subjects ranged from 8 to 16 years (12.03±2.27 years). The mean maximal expiratory pressure (MEP), maximal inspiratory pressure (MIP), vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and peak cough flow (PCF) were 36.93±9.5 cmH2O, 45.79±17.46 cmH2O, 1.4±0.43 L, 1.45±0.45 L, 1.40±0.41 L, and 206.25±61.21 L/min, respectively. The MIP%, MEP%, and FVC% of the Korean children and adolescent reference data showed statistically significant higher values than those of the prediction equation data.ConclusionWe observed a clear numeric difference between Korean DMD patients' pulmonary function value expressed as % of the overseas data and inland data. To perform a precise assessment of respiratory function and to determine appropriate respiratory therapy, pulmonary function values of Korean DMD patients should be interpreted taking into account the inland normal pulmonary function test data.

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Ventilatory parameters and maximal respiratory pressure changes with age in Duchenne muscular dystrophy patients

Cited by 58 publications

References 29 publications

Ventilatory Chemosensory Drive Is Blunted in the mdx Mouse Model of Duchenne Muscular Dystrophy (DMD)

Ventilatory Chemosensory Drive Is Blunted in the mdx Mouse Model of Duchenne Muscular Dystrophy (DMD)

Sniff nasal inspiratory pressure in the longitudinal assessment of young Duchenne muscular dystrophy children

Analysis of Pulmonary Function Test in Korean Patients With Duchenne Muscular Dystrophy: Comparison of Foreign and Korean Reference Data

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