Clinical assessment underestimates fat mass and overestimates resting energy expenditure in children with neuromuscular diseases

Barja, Salesa; Pérez, Regina

doi:10.1016/j.clnesp.2016.03.079

Cited by 5 publications

(7 citation statements)

References 17 publications

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“…BIA is a non-invasive test, easy to perform, low-cost, and sensitive to fat mass changes (Barja, 2016). The predictive equations for BIA validated for the healthy population should be applied with caution in patients with DMD since their body composition is affected by the disease, sedentary lifestyle, and the prolonged use of steroids (Elliott et al, 2015).…”

Section: Discussionmentioning

confidence: 99%

When age matters: boys with Duchenne muscular dystrophy have growth delay and a fat mass accumulation, as they get older

Cunha

Grilo

Costa

et al. 2021

RSD

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Anthropometry and body composition evaluation in boys with Duchenne muscular dystrophy (DMD) are challenging, but crucial methods to evaluate the nutritional status, and better anthropometric reference values and body composition predictive equations are needed for this population. Based on these aspects, this study aimed to investigate the hypothesis that changes in anthropometric parameters and body composition of boys with DMD occur according to age. A cross-sectional study with 49 individuals diagnosed with DMD at the neurological outpatient facility at the Onofre Lopes University Hospital in Natal, Brazil, was performed between September 2016 and March 2019. These individuals underwent anthropometric and body composition evaluation. According to age, the participants were divided into four groups: G1 (2.6 - 8.2y), G2 (8.5 - 10.8y), G3 (11.0 - 14.0y), and G4 (15.9 - 23.0y). The parameters weight-for-age (W/A) (p=0.025), tricipital skinfold (TSF) (p=0.027), adductor pollicis muscle (p=0.041), and corrected arm muscle area (cAMA) (p=0.005) were different among the groups. Regarding anthropometric parameters, was prevalence in the categories of appropriate W/A and a height-for-age (H/A), and eutrophy for body mass index-for-age (BMI/A). For the TSF, there was a higher frequency of severe malnutrition or obesity. The cAMA indicated severe malnutrition in most individuals. As for %FM, high adiposity was more frequent, increasing over age groups (G1 to G4). The boys with DMD presented different patterns of anthropometric and body composition parameters. An increase of fat mass and a decrease of lean mass with age/disease progression were observed.

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Section: Discussionmentioning

confidence: 99%

When age matters: boys with Duchenne muscular dystrophy have growth delay and a fat mass accumulation, as they get older

Cunha

Grilo

Costa

et al. 2021

RSD

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“…To test whether age, BW, SL, TL, ventilatory status, and nusinersen treatment had a significant impact on energy expenditure, we plotted in Figure 1 the relations between these variables and 19 (16,25) 19 (16,41) 20 (17,28) Sex Girls 15 (12,20) 12 (11,15) 14 (14,15) 14 (13,17) 14 (13,17) BMI, kg Relation between age, weight, body, and tibia length and measured resting energy expenditure (mREE) according to the ventilatory status (yes compared with no) and nusinersen treatment (yes compared with no). A) Plots the relation between age, weight, supine and tibia length, and mREE according to the ventilatory status (yes compared with no).…”

Section: Influence Of Demographic Anthropometric and Clinical Variamentioning

confidence: 99%

“…The gold standard method for the determination of REE is indirect calorimetry (IC) (11); however, IC devices are rarely available in clinics, and nutritional care professionals often have to rely on predictive equations to estimate REE. WHO (12) and Schofield (13) equations have been studied to a limited extent in treatment-naïve SMA patients, all showing overestimation of energy requirements compared with REE obtained by IC (4,14,15). The International Standards of Care for SMA recommended 9-11 kcal/height (cm) regardless of age, sex, and clinical status (16), without external validation studies confirming this energy recommendation.…”

Section: Introductionmentioning

confidence: 99%

Predictive energy equations for spinal muscular atrophy type I children

Bertoli

Amicis

Bedogni

et al. 2020

The American Journal of Clinical Nutrition

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Background Knowledge on resting energy expenditure (REE) in spinal muscular atrophy type I (SMAI) is still limited. The lack of a population-specific REE equation has led to poor nutritional support and impairment of nutritional status. Objective To identify the best predictors of measured REE (mREE) among simple bedside parameters, to include these predictors in population-specific equations, and to compare such models with the common predictive equations. Methods Demographic, clinical, anthropometric, and treatment variables were examined as potential predictors of mREE by indirect calorimetry (IC) in 122 SMAI children consecutively enrolled in an ongoing longitudinal observational study. Parameters predicting REE were identified, and prespecified linear regression models adjusted for nusinersen treatment (discrete: 0 = no; 1 = yes) were used to develop predictive equations, separately in spontaneously breathing and mechanically ventilated patients. Results In naïve patients, the median (25th, 75th percentile) mREE was 480 (412, 575) compared with 394 (281, 554) kcal/d in spontaneously breathing and mechanically ventilated patients, respectively (P = 0.009). In nusinersen-treated patients, the median (25th, 75th percentile) mREE was 609 (592, 702) compared with 639 (479, 723) kcal/d in spontaneously breathing and mechanically ventilated patients, respectively (P = 0.949). Both in spontaneously breathing and mechanically ventilated patients, the best prediction of REE was obtained from 3 models, all using as predictors: 1 body size related measurement and nusinersen treatment status. Nusinersen treatment was correlated with higher REE both in spontaneously breathing and mechanically ventilated patients. The population-specific equations showed a lower interindividual variability of the bias than the other equation tested, however, they showed a high root mean squared error. Conclusions We demonstrated that ventilatory status, nusinersen treatment, demographic, and anthropometric characteristics determine energy requirements in SMAI. Our SMAI-specific equations include variables available in clinical practice and were generally more accurate than previously published equations. At the individual level, however, IC is strongly recommended for assessing energy requirements. Further research is needed to externally validate these predictive equations.

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“…Thus, clinically, fat mass (FM) is underestimated, whereas resting energy expenditure (REE) is overestimated. [4] However, indirect calorimetry is not always available, making it necessary to estimate REE through equations. BEE according to the Harris–Benedict equation causes nutrient estimates to be excessive.…”

Section: Introductionmentioning

confidence: 99%

“…[32] Moreover, the REE/BW of patients with DM1 is significantly lower than that of healthy subjects, and there is no significant difference in REE/LBM. [33] Previous studies on body composition and REE have also reported several types of Duchenne muscular dystrophy [4,29,34–36] and Becker muscular dystrophy [37,38] but fewer myotonic dystrophy types.…”

Section: Introductionmentioning

confidence: 99%

Assessment of body composition, metabolism, and pulmonary function in patients with myotonic dystrophy type 1

et al. 2022

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Abnormal body composition in myotonic dystrophy type 1 (DM1) are affected by energy intake above resting energy expenditure (REE). We aim to investigate the characteristics and relationship between body composition, REE, and pulmonary function in patients with DM1, and to examine their changes in 1 year. The study design was a single-center, cross-sectional, and longitudinal study of body composition, REE characteristics, and pulmonary function. Twenty-one male patients with DM1 and 16 healthy volunteers were registered in the study. Body composition was measured using dual-energy X-ray absorptiometry (DEXA). Fat mass (FM) index (kg/m 2 ), fat-FM index (kg/m 2 ), and skeletal mass index (kg/m 2 ) were calculated. The measurements were taken breath by breath with a portable indirect calorimeter. The REE was calculated using the oxygen intake (VO 2 ) and carbon dioxide output (VCO 2 ) in the Weir equation. Basal energy expenditure (BEE) was calculated by substituting height, weight, and age into the Harris-Benedict equation. The study enrolled male patients with DM1 (n = 12) and healthy male volunteers (n = 16). Patients with DM1 (n = 7) and healthy volunteers (n = 14) could be followed in 1 year. The body composition of patients with DM1 was significantly higher in the FM index and significantly lower in the fat-FM index and skeletal mass index. The REE of patients with DM1 was significantly lower and was not associated with body composition. Patients with DM1 had poor metabolism that was not related to body composition. FM was high and lean body mass was low. Abbreviations: BEE= basal energy expenditure, BMI = body mass index, BW = body weight, CONUT = controlling nutritional status, CPF = cough peak flow, CTG = cytosine-thymine-guanine, DEXA = dual-energy X-ray absorptiometry, DM1 = myotonic dystrophy type 1, FEV1 = forced expiratory volume in 1 second, FFMI = fat free mass index, FM = fat mass, FMI = fat mass index, FVC = forced vital capacity, HR = heart rate, LBM = lean body mass, MIRS = muscular disability rating scale, REE = resting energy expenditure, RR = respiratory rate, SMI = skeletal mass index, TV = tidal volume, VC = vital capacity, VCO 2 = carbon dioxide output, VE = minute ventilation, VO 2 /HR = O 2 pulse, VO 2 = oxygen intake.

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Clinical assessment underestimates fat mass and overestimates resting energy expenditure in children with neuromuscular diseases

Cited by 5 publications

References 17 publications

When age matters: boys with Duchenne muscular dystrophy have growth delay and a fat mass accumulation, as they get older

When age matters: boys with Duchenne muscular dystrophy have growth delay and a fat mass accumulation, as they get older

Predictive energy equations for spinal muscular atrophy type I children

Assessment of body composition, metabolism, and pulmonary function in patients with myotonic dystrophy type 1

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