The relationship of body habitus and respiratory function in Duchenne muscular dystrophy

Chew, Kenneth Keen Yip; Carey, Kate A.; Ho, Genevieve; Mallitt, Kylie‐Ann; Widger, John; Farrar, Michelle A.

doi:10.1016/j.rmed.2016.08.018

Cited by 15 publications

(15 citation statements)

References 36 publications

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“…Indeed, a significant association was found between serum albumin and respiratory muscle strength. In addition, in DMD, the BMI has been reported to be associated with the respiratory function 34 and the regional lean mass is reduced and associated with decreased strength 35 . We found a significant association between nutritional parameters, namely albumin, prealbumin (transthyretin) and BMI, and respiratory muscle strengths (MIP and MEP).…”

Section: Discussionmentioning

confidence: 54%

Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients

Fayssoil

Chaffaut

Prigent

et al. 2021

Pediatric Pulmonology

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Malnutrition and swallowing disorders are common in Duchenne muscular dystrophy (DMD) patients. We assessed, in adult DMD with home mechanical ventilation (HMV) and cough assist device, its prevalence and the relationships with respiratory muscle strength and long‐term respiratory prognosis. We reviewed the patients (n = 117, age 18–39 years [median 24]), followed in a reference center, from 2006 to 2015, to obtain clinical baseline, nutritional status, vital capacity (VC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). The median body mass index (BMI) was low (15.6 kg/m²). Included patients had severe restrictive respiratory function with a median VC of 10.5% [7–17] of the predicted value. All patients were on HMV. Prevalence of malnutrition, swallowing disorders, and gastrostomy were respectively 62%, 34%, and 11%. BMI and serum albumin level were significantly associated with MIP, MEP, and VC. The 1‐year/5‐years cumulative incidences of respiratory events (pulmonary sepsis and acute respiratory distress) were, respectively, 20.7%/44.5%. Using univariate analysis, predictive factors for respiratory events were swallowing disorders (p = .001), transthyretinemia (p = .034), MIP (p = .039), and MEP (p = .03) but not BMI or albuminemia. Using multivariate analysis, only swallowing disorders remained significantly associated with respiratory events (OR = 4.2, IC 95% 1.31–12.2, p = .01). In conclusion, this study highlights the interrelationships between nutritional intake, swallowing function, airway clearance, and respiratory function in adult DMD. A multidisciplinary approach focusing on these previous factors is essential to optimize DMD patient health.

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Section: Discussionmentioning

confidence: 54%

Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients

Fayssoil

Chaffaut

Prigent

et al. 2021

Pediatric Pulmonology

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“…However, it is likely, given the conflicting findings, that any effect on rate of decline in LF is small, and only one Steroid Naïve subject and no Steroid Users underwent spinal surgery. Likewise, the effects of body mass index and fat distribution have been examined for effect on FVC and sleep disordered breathing 25 26. While both spinal deformity and body weight/habitus have the potential to add bias to the current study, the effects are likely to be small, and analysing their contribution would require large patient numbers.…”

Section: Discussionmentioning

confidence: 99%

Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids

2020

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BackgroundUse of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival.AimTo describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between Steroid Users and Steroid Naïve subjects.MethodA retrospective cohort of adolescents with DMD initiating NIV over 10 years was conducted. Serial LF before and after NIV initiation was collated. Use of systemic glucocorticosteroids, adherence to NIV and presence of cardiac disease were assessed.ResultsTwenty-nine men started NIV, median age 14.66 years (IQR 2.35, 10.47–17.96). Nine were Steroid Users and eight were Steroid Naïve. Indications for NIV were apnoea–hypopnoea index >5 and/or nocturnal hypoventilation. LF is better (forced vital capacity (FVC) z-score −3.26 vs −5.41, p < 0.02) and decline slower (FVC z-score −0.58 per annum (pa) vs −0.68 pa, p<0.001) in Steroid Users compared with Steroid Naïve subjects. Following NIV initiation, FVC z-score decline slowed for the whole (−0.72 pa (95% CI −0.79 to 0.64) to −0.46 pa (95% CI −0.54 to 0.38) p < 0.001) and Steroid Naïve groups (−0.74 (95% CI −0.85 to 0.63) to −0.44 pa (95% CI −0.56 to 0.32) p < 0.001) but accelerated in the Steroid User group (−0.56 (95% CI −0.70 to 0.42) to −0.75 pa (95% CI −0.89 to 0.61) p < 0.001). Adherence to NIV and cardiac disease did not impact decline.ConclusionOverall, LF decline is reduced on NIV. Steroid Naïve patients have lower LF and faster decline, which slows following NIV initiation. An accelerated LF decline was seen on NIV in Steroid Users which requires further prospective research.

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“…In a retrospective study of 34 patients with Duchenne muscular dystrophy (DMD, mean age 13.7 years) Chew et al ( Chew et al, 2016 ) found that BMI was positively related to FVC, contrary to what one might expect in obese individuals ( Sharp et al, 1964 ; Thomas et al, 1989 ; Salome et al, 2010 ). The observed association may have been the result of corticosteroid therapy resulting in greater muscle mass and strength, although the study did not take into account changes in muscle and fat mass, edema or growth, also known to occur in DMD ( Durnin and Rahaman, 1967 ; Stanbury and Graham, 1998 ; Pessolano et al, 2003 ).…”

Section: Effects Of Obesitymentioning

confidence: 98%

Methods and Applications in Respiratory Physiology: Respiratory Mechanics, Drive and Muscle Function in Neuromuscular and Chest Wall Disorders

2022

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Individuals with neuromuscular and chest wall disorders experience respiratory muscle weakness, reduced lung volume and increases in respiratory elastance and resistance which lead to increase in work of breathing, impaired gas exchange and respiratory pump failure. Recently developed methods to assess respiratory muscle weakness, mechanics and movement supplement traditionally employed spirometry and methods to evaluate gas exchange. These include recording postural change in vital capacity, respiratory pressures (mouth and sniff), electromyography and ultrasound evaluation of diaphragmatic thickness and excursions. In this review, we highlight key aspects of the pathophysiology of these conditions as they impact the patient and describe measures to evaluate respiratory dysfunction. We discuss potential areas of physiologic investigation in the evaluation of respiratory aspects of these disorders.

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The relationship of body habitus and respiratory function in Duchenne muscular dystrophy

Abstract: The present study reinforces the importance of weight management in DMD, showing that a higher weight profile and lower adiposity have better respiratory outcomes. Furthermore, attention to body position with airway clearance techniques will maximize their effectiveness.

Cited by 15 publications

References 36 publications

Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients

Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients

Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids

Methods and Applications in Respiratory Physiology: Respiratory Mechanics, Drive and Muscle Function in Neuromuscular and Chest Wall Disorders

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