Elsevier

Brain and Development

Volume 34, Issue 3, March 2012, Pages 206-212
Brain and Development

Original article
Decreased resting energy expenditure in patients with Duchenne muscular dystrophy

https://doi.org/10.1016/j.braindev.2011.05.005Get rights and content

Abstract

Background

Skeletal muscle metabolism is a major determinant of resting energy expenditure (REE). Although the severe muscle loss that characterizes Duchenne muscular dystrophy (DMD) may alter REE, this has not been extensively investigated.

Methods

We studied REE in 77 patients with DMD ranging in age from 10 to 37 years using a portable indirect calorimeter, together with several clinical parameters (age, height, body weight (BW), body mass index (BMI), vital capacity (VC), creatine kinase, creatinine, albumin, cholinesterase, prealbumin), and assessed their influence on REE. In addition, in 12 patients maintaining a stable body weight, the ratio of energy intake to REE was calculated and defined as an alternative index for the physical activity level (aPAL).

Results

REE (kcal/day, mean ± SD) in DMD patients was 1123 (10–11 years), 1186 ± 188 (12–14 years), 1146 ± 214 (15–17 years), 1006 ± 136 (18–29 years) and 1023 ± 97 (⩾30 years), each of these values being significantly lower than the corresponding control (p < 0.0001). VC (p < 0.001) was the parameter most strongly associated with REE, followed by BMI (p < 0.01) and BW (p < 0.05). The calculated aPAL values were 1.61 (10–11 years), 1.19 (12–14 years), 1.16 (15–17 years), and 1.57 (18–29 years).

Conclusion

The REE in DMD patients was significantly lower than the normal value in every age group, and strongly associated with VC. Both the low REE and PAL values during the early teens, resulting in a low energy requirement, might be related to the obesity that frequently occurs in this age group. In contrast, the high PAL value in the late stage of the disease, possibly due to the presence of respiratory failure, may lead to a high energy requirement, and thus become one of the risk factors for development of malnutrition.

Introduction

Resting energy expenditure (REE) is defined as the minimum energy necessary for maintaining the metabolically active components of fat-free mass (FFM) [1], [2], and about 30%, 20%, and 20% of REE is consumed by the liver, muscle and brain, respectively [3]. The REE of healthy individuals is determined principally by several clinical parameters, such as gender, body size, body composition, and age, and many predictive equations are available for calculating REE based on these parameters [4]. REE is used to establish daily energy requirements by multiplying the empirically obtained physical activity level (PAL) by the estimated REE [4]. In contrast to healthy subjects, it has been recommended that REE in diseased individuals be measured, and not estimated [5]. With recent advances in technology, indirect calorimetry, a gold standard for measuring REE, has become easier to perform, and the equipment more portable and affordable, thus being generally applicable in a clinical setting [6], [7].

Duchenne muscular dystrophy (DMD) is an X-linked recessive disease characterized by progressive weakness and wasting of skeletal muscle. Because skeletal muscle metabolism is a major determinant of REE [3], the severe muscle loss in DMD may alter REE. However, REE in DMD has not been investigated in detail [8], [9], [10], [11]. In the present study, we investigated REE, several clinical parameters, and their influence on REE in a large series of DMD patients at various disease stages. In addition, in a proportion of these patients, we investigated the yet poorly understood pathomechanism underlying the nutritional problems associated with DMD, including obesity and malnutrition, which are known to occur at the early and late stages of the disease, respectively [12], [13].

Section snippets

Patients

We studied 77 patients with DMD (age range 10–37 years, mean ± SD 20 ± 5 years), who were institutionalized or admitted to the National Center Hospital of Neurology and Psychiatry, National Hospital Organization (NHO) Higashisaitama National Hospital, NHO Matsue Medical Center, NHO Nishibeppu National Hospital, NHO Suzuka National Hospital, and NHO Tokushima National Hospital. Fifty-five of the patients had been diagnosed as having DMD by dystrophin immunohistochemistry of muscle biopsy specimens (28

Clinical parameters of patients with DMD

The characteristics of the DMD patients are presented in Table 1. All the patients showed varying degrees of decrease in VC (%VC  65%). On the other hand, almost all patients maintained their VE (⩾100 ml/min/kg), except for one patient showing severe respiratory failure with an extremely low VC (VC = 310 ml). The ln CK level showed a linear decrease with increasing age. The serum levels of creatinine, albumin, cholinesterase, and prealbumin were lower than the normal limit in 77 of 77, 5 of 77, 12

Discussion

The present study demonstrated that REE in DMD patients aged 10–37 years was significantly lower than the corresponding value in controls of every age group. Two previous reports have also demonstrated a decrease of REE in patients with DMD. Hankard et al. found a 13% decrease of REE in non-obese patients aged 9.9 ± 0.6 years [8]. Gonzalez-Bermejo et al. found a 34% decrease of REE in patients aged 25 ± 4 years who had received nocturnal mechanical ventilation [11]. Interestingly, the study of Zanardi

Acknowledgements

We thank the patients and their families for their participation in the study. This study was supported in part by grants from the Research Grant (20B-11) for Nervous and Mental Disorders from the Ministry of Health, Labor and Welfare of Japan.

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