Gasto energético na marcha em pacientes com mucopolissacaridose

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.

Functional Independence of Pediatric Patients With Mucopolysaccharidoses

“…The findings of this research project corroborate the general clinical characteristics described in the literature, regarding the significant decline in motor function evidenced in cases of mucopolysaccharidoses. 4 , 10 , 15 We believe that the alteration of hand function in patients with MPS, secondary to finger flexion deformities (claw hands) and joint stiffness, hinders the performance of daily activities, especially those that require manual ability, and has a direct impact on the functional performance of these individuals. 1 , 11 When studying the occurrence of carpal tunnel syndrome in children diagnosed with MPS type II, Know et al 16 stated that this condition has a genetic etiology; and late diagnosis, due to the more severe involvement of other organs followed by skeletal dysplasia and joint stiffness, contributes to the complete loss of hand function.…”

“… 8 , 9 These changes are progressive and have a significant impact on the functional independence of the individuals affected. 10 …”

Applying the functional independence measure to the assessment of patients with mucopolysaccharidosis

“…We believe that the low scores obtained in the Self-care and Locomotion subdomains are due to the clinically significant osteoarticular pathologies associated with Mucopolysaccharidoses 1 , 2 , 6 . Patients with Mucopolysaccharidoses present with musculoskeletal rigidity and articular deformities that impair mobility and locomotion 1 , 2 , 6 , 16 , 17 . There are also changes to the vertebral column characterized by kyphosis and scoliosis, and thoracolumbar vertebral rotation is a common finding.…”

“…There are also changes to the vertebral column characterized by kyphosis and scoliosis, and thoracolumbar vertebral rotation is a common finding. These changes lead to difficulty with gait and mobility in patients with Mucopolysaccharidoses and impact their activities of daily living, such as getting out of bed, going to the bathroom, walking and running 1 , 2 , 6 , 16 , 17 . Apart from these osteoarticular deformities, individuals with Mucopolysaccharidoses also have impaired cardiac, pulmonary and respiratory function and expend more energy in initiating and maintaining gait, which results in early fatigue 16 , 17 .…”

Avaliação do equilí­brio e risco de queda em pacientes com mucopolissacaridose VI

“…Quanto maior o aumento da idade, maior será a morbidade dos portadores devido às deformações osteomusculares e complicações cardiopulmonares [5,14,15]. O quadro clínico dificulta o equilíbrio, e consequentemente a marcha.…”