Gasto energético na marcha em pacientes com mucopolissacaridose

Marcos Almeida Matos,Angelina Xavier Acosta,André Prado,Rosa Barreto,Gustavo Schenkel

doi:10.1590/s1413-78522013000200009

Marcos Almeida Matos, Angelina Xavier Acosta + Show 3 more

Open Access

https://doi.org/10.1590/s1413-78522013000200009

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Abstract

OBJECTIVE: The aim of this study is to evaluate energy expenditure in gait by mucopolysaccharidosis affected patients by means of a simple and adequate to the clinical environment methodology. METHODS: A cross-sectional study was carried out comparing energy expenditure during gait in 19 patients suffering from mucopolysaccharidosis (MPS Group) with 19 asymptomatic control individuals (Control Group). Energy expenditure was measured in calories (cal) using a Polar telemetric watch (model FT7) during a 50 meter walk. Variables such as age, weight, height, body mass index (BMI), initial hart rate, final hart rate, and walking time, were recorded. RESULTS: MPS Group showed a mean energy expenditure during gait of 2.84 cal (±1,01), versus 1.42 cal (±0,51), 100% higher than the Control Group; MPS also presented increased initial hart rate (22% higher), final hart rate (13% higher) and walking time (13% higher). CONCLUSIONS: Energy expenditure during gait in MPS patients was two times higher than control individuals; the methodology used showed to be a promising alternative, also adequate to the standard clinical environment. Level of Evidence III, Cross-sectional Comparative Study.

Highlights

(±0,51), 100% higher than the Control Group; MPS presented increased initial hart rate (22% higher), final hart rate (13% higher) and walking time (13% higher)
Energy expenditure during gait obtained by indirect calorimetry is the gold standard for evaluating the locomotor system efficiency and could be used to assess the degree of impairment of the musculoskeletal system of patients with MPS, due to its characteristics, it can only be done in metabolism testing laboratories.[4]
In relation to the Control Group, the MPS Group had energetic expenditure during gait 100% higher, initial heart rate 22% higher heart rate and final heart rate 13% higher, and walking time 25% higher

Summary

Introduction

Mucopolysaccharidosis (MPS) are an heterogeneous group of lysosomal storage diseases characterized by intracellular accumulation of glycosaminoglycans (GAG), causing damage and dysfunction of various degrees in multiple organs and systems, progressive and letally.[1]The involvement of the musculoskeletal system or “dysostosis multiplex” is a common point in all MPS.[1, 2] This involvement is characterized by osteoarticular deformities (kyphosis, scoliosis, knee valgus, equinus foot), stiff joints with loss of range of motion (MR) and upper motor neuron involvement (myelopathy, hypertonia and spasticity).[1,2]The progression of motor manifestations such as walking ability and deambulation, sedestation, and functional independence for daily life activities, has a direct relationship with the overall clinical severity of the disease.[1,2,3]Energy expenditure during gait obtained by indirect calorimetry is the gold standard for evaluating the locomotor system efficiency and could be used to assess the degree of impairment of the musculoskeletal system of patients with MPS, due to its characteristics, it can only be done in metabolism testing laboratories.[4]. (±0,51), 100% higher than the Control Group; MPS presented increased initial hart rate (22% higher), final hart rate (13% higher) and walking time (13% higher).

Results

Conclusion