Respiratory muscle strength and quality of life in myotonic dystrophy patients

Araújo, Thaise Lucena; Resqueti, Vanessa; Bruno, Selma S.; Azevedo, Ingrid Guerra; Júnior, M.E. Dourado; Fregonezi, Guilherme A. F.

doi:10.1016/s2173-5115(10)70006-3

Cited by 8 publications

(9 citation statements)

References 27 publications

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“…One limitation of our study was that analyses of VC relative to other disease measured was limited by lack of baseline data for VC in 93 of our 110 participants as VC did not become a routine part of spirometry for our DM1 patients until a few years after our data collection starting point. (19,20). Data on the benefit of NIV use in DM1 is inconclusive.…”

Section: Discussionmentioning

confidence: 99%

Factors Influencing the Severity and Progression of Respiratory Muscle Dysfunction in Myotonic Dystrophy Type 1

et al. 2021

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Respiratory complications are the most common cause of death among patients with Myotonic Dystrophy type 1 (DM1), but the natural history of respiratory decline in DM1 patients is incompletely characterized and few predictors of the progression of respiratory dysfunction have been identified. To identify factors influencing the progression of respiratory dysfunction electronic medical records from 110 adult patients diagnosed with DM1 were reviewed along with data for respiratory symptoms and pulmonary function obtained from routine respiratory therapist clinical evaluations. At baseline, 70.9% had evidence of restrictive respiratory impairment. We examined various parameters of respiratory functional status, and found FVC (% predicted) correlated best with other measures of disease severity. Annual change in FVC was −1.42 (std error = 0.381). Greater CTG repeat size, higher MIRS rating, and longer disease duration were all correlated with lower baseline FVC but not with annual rate of change. Wide variability in clinical phenotype made determination of disease measures directly related to respiratory functional decline challenging.

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Section: Discussionmentioning

confidence: 99%

Factors Influencing the Severity and Progression of Respiratory Muscle Dysfunction in Myotonic Dystrophy Type 1

et al. 2021

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“…The Medical Outcome Study Short Form-36 (SF-36) [7] has been used to assess HRQoL in many neuromuscular conditions [8], including DM1. More specifically, some studies have reported that scores on each of the 8 domains of health in the SF-36 are lower in patients affected by DM1 than in either healthy controls or large samples of the general population [9,10], while others found that only physical health scores, but not mental health scores, are reduced in DM1 patients compared to normal subjects [11,12]. In addition, multivariate analysis of factors influencing QoL in DM1 patients have revealed that older age, severity of muscular involvement, depressive and anxious symptoms, and poor acceptance of illness were associated with reduced SF-36 scores [13,14].…”

Section: Introductionmentioning

confidence: 99%

Clinical, Psychosocial, and Central Correlates of Quality of Life in Myotonic Dystrophy Type 1 Patients

et al. 2013

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Aims: To identify sociodemographic, clinical, and central correlates of health-related quality of life (HRQoL) in DM1 patients. Methods: 200 DM1 patients had assessments of muscular impairment, CTG repeats, and intelligence. Validated instruments were used to assess sociodemographic and clinical factors as well as social support, social participation, daytime sleepiness, fatigue, personality, mood, and quality of life. Regression analysis was used to identify correlates of SF-36 physical and mental component summary scores. Results: Patients scored lower on all SF-36 physical health subscales compared with normative data but did not differ with respect to mental health function. Regression analysis revealed that psychological distress, fatigue, severe muscular impairment, emotional stability, not having worked within the last 12 months, and lower intellectual quotient were associated with lower scores in physical health function. Moreover, neuroticism, daytime sleepiness, dissatisfaction with social participation, and lower conscientiousness were associated with lower scores in mental health function. Conclusion: DM1 has an impact on SF-36 physical summary scores but not on mental summary scores. Factors such as fatigue, daytime sleepiness, psychological distress, unemployment, and social participation dissatisfaction that significantly affect HRQoL in DM1 are amenable to treatment and psychosocial interventions, namely by providing care that integrate health, social, and community services.

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“…Twenty-three studies assessed respiratory muscle strength. Eighteen used manometry [88][89][90][91][92][93][94][95][96][97][98][99][100]102,103,[108][109][110] (supplementary table 1).…”

Section: Respiratory Muscle Strengthmentioning

confidence: 99%

“…Fifteen studies of maximal expiratory pressure [88][89][90][91][92][93][96][97][98][99][100]102,103,108,110] 2D).…”

Section: Maximal Inspiratory and Expiratory Pressuresmentioning

confidence: 99%

“…Sniff nasal inspiratory pressure [91,96,103,109] and transdiaphragmatic pressure following a sniff maneuver (SniffPdi) and transdiaphragmatic pressure following supramaximal cervical stimulation of the phrenic nerves (twPdi) and gastric pressure following stimulation of the abdominal muscle nerve roots at the tenth vertebra (twPgas) [92] were reported (supplementary table 1). Sniff nasal inspiratory pressure mean values were between 46% to 80% in patients with DM1 and 70.64±19.6% in controls [91,109] (table 3).…”

Section: Sniff Inspiratory Pressurementioning

confidence: 99%

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Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review

Mateus

Costa

Viegas

et al. 2022

Neuromuscular Disorders

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Respiratory muscle strength and quality of life in myotonic dystrophy patients

Cited by 8 publications

References 27 publications

Factors Influencing the Severity and Progression of Respiratory Muscle Dysfunction in Myotonic Dystrophy Type 1

Factors Influencing the Severity and Progression of Respiratory Muscle Dysfunction in Myotonic Dystrophy Type 1

Clinical, Psychosocial, and Central Correlates of Quality of Life in Myotonic Dystrophy Type 1 Patients

Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review

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