Physical function and performance measures of children and adolescents with Charcot-Marie-Tooth disease.

This study aimed to verify whether there are differences in foot type, physical function, and performance between children and adolescents with Charcot-Marie-Tooth disease (CMT) and their healthy counterparts, and whether an interval of 6 months, from the initial assessment, reveals any significant changes on physical performance and other specific physical measures of the lower limbs. Subjects and Methods: Participants between 6 and 17 years of age, with CMT (CMT group n = 40), were compared to healthy participants (Control group, n = 49). Twenty participants with CMT completed the follow-up. We collected anthropometric, goniometric (ankle and knee), and dynamometric (inversion, eversion, plantar flexors, dorsiflexors, knee, hip extensors) measures, agonist/antagonist ratios, feet alignment (Foot Posture Index - FPI), muscle power (Long Jump), Pediatric Balance Scale (PBS), and 10-meter walk test (10MWT). Results: Compared to Control, CMT showed reduced passive range of motion (ROM), weakness in all evaluated muscles, increased agonist/antagonist ratios, predominance of varus and lower performance (PBS and Long Jump). After 6 months, CMT measures did not change. Conclusion: The cross-sectional analysis showed reduced ROM, strength, power, and distal muscle imbalance, as well as secondary limitations (PBS and 10MWT) in children and adolescents with CMT. These biomechanical and functional alterations did not change at the 6-month follow-up.

Proposta de um protocolo de treinamento físico em reabilitação cardiovascular fase II na insuficiência cardíaca avançada / Proposal of a physical training protocol in phase II cardiovascular rehabilitation in advanced heart failure

A insuficiência cardíaca (IC) é uma síndrome clínica complexa de caráter sistêmico, definida como disfunção cardíaca que ocasiona inadequado suprimento sanguíneo para atender às necessidades metabólicas dos tecidos. A IC crônica acarreta alterações periféricas e centrais, que são funcionais (como um mecanismo de compensação) a curto prazo, mas podem ter consequências negativas a longo prazo, resultando em capacidade de exercício reduzida. Claramente, esses pacientes apresentam massa muscular diminuída quando comparados com indivíduos saudáveis, além de alterações no tipo de fibra muscular, o que cursa com capacidade de exercício reduzida que pode ser expressa pelo menor VO2 pico comparado com indivíduos saudáveis...

Balance and muscle power of children with Charcot-Marie-Tooth / Equilíbrio e potência muscular em crianças com doença de Charcot-Marie-Tooth

BACKGROUND: In certain diseases, functional constraints establish a greater relationship with muscle power than muscle strength. However, in hereditary peripheral polyneuropathies, no such relationship was found in the literature. OBJECTIVE: In children with Charcot-Marie-Tooth (CMT), to identify the impact of muscle strength and range of movement on the static/dynamic balance and standing long jump based on quantitative and functional variables. METHOD: The study analyzed 19 participants aged between 6 and 16 years, of both genders and with clinical diagnoses of CMT of different subtypes. Anthropometric data, muscle strength of the lower limbs (hand-held dynamometer), ankle and knee range of movement, balance (Pediatric Balance Scale) and standing long jump distance were obtained by standardized procedures. For the statistical analysis, Pearson and Spearman correlation coefficients were used. RESULTS: There was a strong positive correlation between balance and the muscle strength of the right plantar flexors (r=0.61) and dorsiflexors (r=0.59) and a moderate correlation between balance and the muscle strength of inversion (r=0.41) and eversion of the right foot (r=0.44). For the long jump and range of movement, there was a weak positive correlation with right and left plantar flexion (r=0.20 and r=0.12, respectively) and left popliteal angle (r=0.25), and a poor negative correlation with left dorsiflexion (r=-0.15). CONCLUSIONS: The data on the patients analyzed suggests that the maintenance of distal muscle strength favors performance during balance tasks, while limitations in the range of movement of the legs seem not to be enough to influence the performance of the horizontal long jump. .

CONTEXTUALIZAÇÃO: Em algumas doenças, limitações funcionais têm maior relação com a potência do que com a força muscular, porém, nas polineuropatias periféricas hereditárias, tal relação não foi relatada na literatura. OBJETIVO: Identificar, a partir da análise de variáveis quantitativas e funcionais, o impacto da força dos músculos dos membros inferiores e das amplitudes de movimento (ADM) sobre o equilíbrio estático/dinâmico e o salto horizontal de crianças com doença de Charcot-Marie-Tooth (CMT). MÉTODO: Foram incluídos 19 participantes de seis a 16 anos, de ambos os sexos, com diagnóstico clínico de CMT de subtipos variados. Foram obtidos, de forma padronizada, dados antropométricos, força muscular dos membros inferiores (Hand-Held Dynamometer), ADM de tornozelos e joelhos, equilíbrio (Escala de Equilíbrio Pediátrica) e distância de salto horizontal (Long Jump). Para a análise estatística, utilizaram-se os Coeficientes de Correlação de Pearson e Spearman. RESULTADOS: Houve forte correlação positiva entre equilíbrio e força muscular de flexores plantares direito (r=0,61) e dorsiflexores (r=0,59) e correlação moderada com inversores dos pés (r=0,41) e eversores do pé direito (r=0,44). Considerando o Long Jump e as ADM, houve fraca correlação positiva para ADM de flexão plantar direita (r=0,20), esquerda (r=0,12) e ângulo poplíteo esquerdo (r=0,25) e fraca correlação negativa para dorsiflexão esquerda (r=-0,15). CONCLUSÕES: Os dados dos pacientes analisados sugerem que a manutenção de força muscular distal favorece o desempenho em tarefas de equilíbrio. E as restrições nas ADM passivas de membros inferiores parecem ...

Balance and muscle power of children with Charcot-Marie-Tooth.

BACKGROUND: In certain diseases, functional constraints establish a greater relationship with muscle power than muscle strength. However, in hereditary peripheral polyneuropathies, no such relationship was found in the literature. OBJECTIVE: In children with Charcot-Marie-Tooth (CMT), to identify the impact of muscle strength and range of movement on the static/dynamic balance and standing long jump based on quantitative and functional variables. METHOD: The study analyzed 19 participants aged between 6 and 16 years, of both genders and with clinical diagnoses of CMT of different subtypes. Anthropometric data, muscle strength of the lower limbs (hand-held dynamometer), ankle and knee range of movement, balance (Pediatric Balance Scale) and standing long jump distance were obtained by standardized procedures. For the statistical analysis, Pearson and Spearman correlation coefficients were used. RESULTS: There was a strong positive correlation between balance and the muscle strength of the right plantar flexors (r=0.61) and dorsiflexors (r=0.59) and a moderate correlation between balance and the muscle strength of inversion (r=0.41) and eversion of the right foot (r=0.44). For the long jump and range of movement, there was a weak positive correlation with right and left plantar flexion (r=0.20 and r=0.12, respectively) and left popliteal angle (r=0.25), and a poor negative correlation with left dorsiflexion (r=-0.15). CONCLUSIONS: The data on the patients analyzed suggests that the maintenance of distal muscle strength favors performance during balance tasks, while limitations in the range of movement of the legs seem not to be enough to influence the performance of the horizontal long jump.