Height and body mass index distribution in children and adolescents with and without spina bifida.

OBJECTIVE: Children with spina bifida (SB) are at risk for stunting and overweight. However, height and Body Mass Index (BMI) z-score distribution in children and adolescents with SB are unclear. The aim of this study was to examine height and BMI z-score distribution in Brazilian children and adolescents with and without SB. This study further aimed to examine whether height and BMI z-scores differ between individuals with and without SB. METHOD: This study included 101 participants (SB: n = 18; non-SB: n = 83, aged 7-16 years). The World Health Organization (WHO) AnthroPlus software was used to calculate height and BMI z-scores. AnthroPlus z-score distribution graphs were used to examine individual z-scores based on the 2007 WHO normal distribution curve. Effects of the group (SB vs non-SB) on height and BMI z-scores were examined with sequential regression. RESULTS: In the WHO distribution graph analysis, height z-scores of participants with SB were slightly left-shifted compared to the WHO normal distribution curve. In the regression analysis, group (SB vs non-SB) was a significant predictor of height z-scores after controlling for sex and age (ΔR2 = 0.064, p = 0.010). BMI z-scores of participants with SB were right-shifted compared to the WHO normal curve. However, there was no contribution of the group to BMI z-scores (ΔR2 = 0.011, p = 0.301). CONCLUSIONS: These findings suggest that Brazilian children and adolescents with SB may be at risk for reduced height and increased BMI.

Height and body mass index distribution in children and adolescents with and without spina bifida

Abstract Objective Children with spina bifida (SB) are at risk for stunting and overweight. However, height and Body Mass Index (BMI) z-score distribution in children and adolescents with SB are unclear. The aim of this study was to examine height and BMI z-score distribution in Brazilian children and adolescents with and without SB. This study further aimed to examine whether height and BMI z-scores differ between individuals with and without SB. Method This study included 101 participants (SB: n= 18; non-SB: n= 83, aged 7-16 years). The World Health Organization (WHO) AnthroPlus software was used to calculate height and BMI z-scores. AnthroPlus z-score distribution graphs were used to examine individual z-scores based on the 2007 WHO normal distribution curve. Effects of the group (SB vs non-SB) on height and BMI z-scores were examined with sequential regression. Results In the WHO distribution graph analysis, height z-scores of participants with SB were slightly left-shifted compared to the WHO normal distribution curve. In the regression analysis, group (SB vs non-SB) was a significant predictor of height z-scores after controlling for sex and age (ΔR2= 0.064, p= 0.010). BMI z-scores of participants with SB were right-shifted compared to the WHO normal curve. However, there was no contribution of the group to BMI z-scores (ΔR2= 0.011, p= 0.301). Conclusions These findings suggest that Brazilian children and adolescents with SB may be at risk for reduced height and increased BMI.

Generation of percentile curves for strength and functional abilities for boys with Duchenne muscular dystrophy.

INTRODUCTION/AIMS: Considering the heterogeneity of the clinical manifestations of Duchenne muscular dystrophy (DMD), it is important to describe their various clinical profiles. Thus, in this study we aimed to develop percentile curves for DMD using a battery of measures to define the patterns of functional abilities, timed tests, muscle strength, and range of motion (ROM). METHODS: This retrospective data analysis was based on the records of patients with DMD using the Motor Function Measure (MFM) scale, isometric muscle strength (IS), dorsiflexion ROM, 10-meter walk test (10 MWT), and 6-minute walk test (6 MWT). Percentile curves (25th, 50th, and 75th percentiles) with MFM, IS, ROM, 10 MWT, and 6 MWT on the y axis and patient age on the x axis were constructed using the generalized additive model for location, scale, and shape, with Box-Cox power exponential distribution. RESULTS: There were records of 329 assessments of patients between 4 and 18 years of age. The MFM percentiles showed a gradual reduction in all dimensions. Muscle strength and ROM percentiles showed that the knee extensors were the most affected from 4 years of age, and dorsiflexion ROM negative values were noted from the age of 8 years. The 10 MWT showed a gradual increase in performance time with age. For the 6 MWT, the distance curve remained stable until 8 years, with a subsequent progressive decline. DISCUSSION: In this study we generated percentile curves that can help health professionals and caregivers follow the trajectory of disease progression in DMD patients.

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.

Translation and cross-cultural adaptation of the Charcot-Marie-Tooth disease Pediatric Scale to Brazilian Portuguese and determination of its measurement properties.

BACKGROUND: The Charcot-Marie-Tooth disease Pediatric Scale (CMTPedS) has been used to measure aspects of disability in children with all types of Charcot-Marie-Tooth disease (CMT). OBJECTIVE: To translate and cross-culturally adapt the CMTPedS into Brazilian-Portuguese and determine its reliability and validity. METHODS: The translation and cross-cultural adaptation followed international guidelines recommendations. Twenty individuals with CMT were assessed. Two examiners assessed the participants for inter-rater reliability. Face validity was assessed by eight physical therapists that judged the relevance of each test item. The Bland-Altman analysis (bias) and standard error of measurement (SEM) complemented the analysis. Furthermore, intraclass correlation coefficients (ICC), weighted kappa (k), and internal consistency (Cronbach's alpha) was determined. RESULTS: The CMTPedS was successfully translated and cross-culturally adapted. Twenty children/youth were enrolled in the study. Of these, the majority (55%) were girls with a mean age of 13.9 (range: from 6 to 18) years. Regarding face validity, the CMTPedS-Br showed relevant items for assessing children and youth with CMT. The ICC for the total score showed excellent reliability (ICC2.1  = 0.93, 95% CI = 0.84, 0.97). The most reliable items were grip, dorsiflexion and plantar flexion strength while the least reliable items were pinprick, vibration, and gait. The internal consistency was excellent (α = 0.96, 95% CI = 0.91, 0.99) and the agreement showed small variability (bias = 0.15, 95% CI= -4.28, 4.60). CONCLUSION: The CMTPedS-Br showed adequate reliability and face validity to measure disability in individuals with CMT. This tool will allow Brazil to be part of multicentered studies on such a rare but debilitating condition.

Characterizing postural oscillation in children and adolescents with hereditary sensorimotor neuropathy.

Charcot Marie Tooth disease (CMT) has negative functional impact on postural control of children; however, it has not been widely studied. Stabilometry can provide insights about postural control and guide preventive interventions in immature perceptual and musculoskeletal systems as those seen in children with CMT. This cross-sectional study aimed to identify and interpret stabilometric variables that reflect the postural control of children with CMT. 53 subjects (age 6-17) were assigned to one of the two groups: CMT (15 males and 14 females with CMT) or Control (13 males and 11 females healthy). Quiet standing was tested in different conditions: with open and closed eyes on regular surface (open-regular, closed-regular) and foam surface (open-foam, closed-foam) using a force platform. The minimum of 2 and maximum of 3 trials of 30 seconds for each test condition provided the classical stabilometric variables and Romberg Quotient (RQv). CMT group showed increase of confidence ellipse area, mean velocity, mediolateral and anteroposterior velocities associated with decreased mean body oscillation frequency, as the complexity of tasks increased. CMT postural deficit was identified by greater and faster sway associated with these lower frequencies, when compared to Control.

Is it possible to stabilize the trunk using rhythmic stabilization in the upper limb? A cross-sectional study of asymptomatic individuals.

Objective: The study aim was to evaluate the immediate effect of rhythmic stabilization on local and distant muscles involved in a functional reach. Method: Prospective, observational cross-sectional study. Eight right-handed and non-impaired individuals (4 females and 4 males) aged 18-24 years (21.5 ± 1.58 years) were evaluated. Bilateral electromyographic recording of the biceps brachii, triceps brachii, multifidus lumbar, and rectus abdominis muscles was performed during three different tasks. Task 1 involved functional reach, while Task 2 involved rhythmic stabilization followed by a functional reach. Task 3 was similar to Task 2, but with 3 repetitions before a functional reach. Results: The results showed no difference between the tasks or sides. However, an interaction was observed between each side and muscles, with greater activation of the right multifidus lumbar muscle. Conclusion: Rhythmic stabilization during the task of reaching promotes an increase of multifidus activity ipsilateral to its application. Thus, this particular technique of proprioceptive neuromuscular facilitation can be useful for improving stability of the trunk and can be used in clinical practice for this purpose. Level of Evidence: 5.

Beneficial effects of ankle-foot orthosis daytime use on the gait of Duchenne muscular dystrophy patients.

BACKGROUND: Orthosis use prevents muscle contracture and prolongs ambulation in patients with Duchenne muscular dystrophy (DMD). However, its biomechanical effects on gait are unclear. This study assessed the effects of daytime and night-time use of Articulated Ankle Foot Orthosis (AFO) on kinematic, kinetic and spatial/temporal gait parameters of DMD patients. METHODS: Twenty ambulatory patients (4-12years of age) were assigned to one of three groups: no orthosis (NoO; n=7), night-time orthosis (NiO; n=7), day-time orthosis (DO; n=6). All subjects were evaluated once (Ev1) and five of them were re-evaluated between five and seven months after Ev1 (Ev2). FINDINGS: Cross-sectional analysis with linear mixed-effects models (ANOVA) showed increased peak dorsiflexion angle and dorsiflexor moment and decreased plantar flexion angle and ankle joint power generation for the DOwith group when compared to the NoO group (P<0.05). The DOwith group also showed decreased peak hip flexion angle, hip power absorption, plantar flexion angle and increased peak dorsiflexion moment when compared to the NoO group (P<0.05). Analysis of gait cycle curves showed significant and clinically relevant changes in kinematic and kinetic parameters for the DOwith group when compared to the other experimental groups. Longitudinal analysis suggest that night-time use of Articulated AFO can promote positive changes in gait parameters of DMD patients, when used before the functional deficit is too advanced. INTERPRETATION: Early daytime and night-time use of Articulated AFO changed gait and minimized typical compensations seen in DMD patients, thus it is recommended in order to prolong gait ability.

Gait energy expenditure in children with Duchenne muscular dystrophy: case study / El gasto energético durante la marcha de los niños con distrofia muscular de Duchenne: un estudio de caso / Gasto energético na marcha de crianças com distrofia muscular de Duchenne: estudo de caso

This case study aimed to verify the model of Rose et al.1 as a feasible to assess energy expenditure in gait of children with Duchenne muscular dystrophy (DMD). Three DMD patients aged 6, 7 and 8 years old participated of this study. It was obtained weight, height, leg length measurement (LLM), resting and gait heart rate (HR) held on as 55-meter oval circuit performed during a two-minute test at each speed. Energy expenditure was calculated using the HR. It was performed a descriptive analysis (average) and these were compared, individually, to normative data. The average gait speed of these three patients was similar to the normative data for slow speed and lower considering comfortable and fast speed. The energy expenditure to slow speed of the patients 2 and 3 was similar to the normality, and lowest for patient 1; at comfortable speed, the energy expenditure obtained for all patients was similar; at fast speed, the patients 1 and 2 presented similar to normal values, but the patient 3 presented higher energy expenditure. It was concluded that the energy expenditure evaluation using HR was easily executed in the clinical practice and it can help therapeutic choices. For patient 3, an aerobic training could be indicated and for the others, they could keep the routine assessments.

Este estudio de caso tuvo como objetivo verificar si el modelo de Rose et al es factible para evaluar el gasto de energía en la marcha de niños con distrofia muscular de Duchenne (DMD). Participaron tres niños con DMD con edades de 6, 7 y 8 años. Fueron evaluados peso, altura, longitud de las extremidades inferiores (LEI), frecuencia cardiaca (FC) en reposo y la marcha realizada en el circuito ovalado de 55 m durante un exámen de 2 minutos en cada velocidad. El gasto energético fue calculado por la FC. Se realizó un análisis descriptivo de los datos (media) y éstos fueron comparados de forma individual con los datos normativos. La velocidad media (VM) de la marcha de los tres pacientes fue igual a los datos normativos en la etapa velocidad lenta y menor en las etapas de velocidad confortable y rápida. El gasto de energía en la velocidad lenta de los pacientes 2 y 3 fue similar a la normalidad, y menor para el paciente 1; en la velocidad confortable, el gasto de energía de todos los pacientes fue similar; en la velocidad rápida, los pacientes 1 y 2 tuvieron valores similares a lo normal, pero el paciente 3 tuvo mayor gasto energético. Se concluyó que la evaluación del gasto energético por la FC se llevó a cabo fácilmente en la clínica, y pudo ayudar en la elección de conductas. Para el paciente 3 podría ser indicado un entrenamiento aeróbico y para los demás mantener ese protocolo de evaluación en las visitas siguientes.

Este estudo de caso objetivou verificar se o modelo de Rose et al. 1 é factível para avaliar o gasto energético na marcha em crianças com distrofia muscular de Duchenne (DMD). Participaram três crianças com DMD e idades de 6, 7 e 8 anos. Foram avaliados peso, altura, comprimento dos membros inferiores (CMMII), frequência cardíaca (FC) de repouso e de marcha realizada em circuito oval de 55 m durante um teste de 2 minutos em cada velocidade. O gasto energético foi calculado pela FC. Foi realizada análise descritiva dos dados (média) e estes foram comparados, individualmente, com dados normativos. A velocidade média (Vm) da marcha dos três pacientes foi igual aos dados normativos na etapa velocidade lenta e menor nas etapas de velocidade confortável e rápida. O gasto energético na velocidade lenta dos pacientes 2 e 3 foi similar à normalidade, e menor para o paciente 1; na velocidade confortável, o gasto energético de todos os pacientes foi similar; na velocidade rápida, os pacientes 1 e 2 apresentaram valores similares ao normal, porém o paciente 3 teve maior gasto energético. Concluiu-se que a avaliação do gasto energético pela FC foi facilmente executada na clínica, podendo auxiliar na eleição de condutas. Para o paciente 3 poderia ser indicado um treinamento aeróbio e para os demais manter esse protocolo de avaliação nas visitas subsequentes.

O efeito da rizotomia dorsal seletiva no quadro clínico e nos cuidados diários de crianças com paralisia cerebral espástica / Effect of the selective dorsal rhizotomy on the clinical presentation and daily care of children with spastic cerebral palsy

A Paralisia Cerebral (PC) acomete 4 a cada 1000 crianças no mundo,representando uma grave problema para o sistema de saúde. Essa entidade nosológica, caracterizada por déficit motor instalado durante o desenvolvimento cerebral, pode acarretar a condição clínica denominada espasticidade, uma hipertonia muscular causada por exarcebação do arco reflexo medular na ausência da inibição advinda de vias superiores. A espasticidade acarreta diversos comprometimentos motores e funcionais para a criança, dificultando o posicionamento e os cuidados de higiene. É de fundamental importância, portanto, o estudo da associação de intervenções cirúrgicas e tratamentos fisioterapêuticos que proporcionem controle da espasticidade. Objetivo: avaliar o impacto da Rizotomia Dorsal Seletiva (RDS) no quadro clínico de crianças espásticas e na realização dos cuidados diários. Métodos: Participaram do estudo 7 crianças com espasticidade, GMFCS de 4 a 5, de 5 a 11 anos de idade. Antes e depois da cirurgia, os seguintes dados foram avaliados: grau de espasticidade dos grupos musculares adutores do quadril e isquiotibiais nos membros inferiores e para o grupo flexor do cotovelo nos membros superiores (escala de Ashworth Modificada); medida do ângulo poplíteo unilateral e bilateral, do ângulo de abdução do quadril e de dorsiflexão do tornozelo com goniometria. Além disso,foi aplicado questionário às famílias para avaliar o grau de dificuldade para os cuidados diários e o grau de satisfação após a RDS. Conclusões: Houve redução da espasticidade no pós-operatório em todos os grupos musculares testados, em todos os pacientes. Houve alteração significativada goniometria para o ângulo poplíteo bilateral (p<0,05). Das 7 famílias questionadas, 6 (85,7%) relataram melhora para o posicionamento,alimentação, higiene e facilidade para vestir e instalar órteses. Desse modo, a RDS mostra-se uma opção para o tratamento de casos de espasticidade refratária ao tratamento clínico em crianças com...

Cerebral Palsy (CP) affects 4 in every 1000 children in the world, representing a serious problem for the health care systems. This disease, characterized by motor deficit engendered during brain development, may lead to the clinical condition known as spasticity, a muscle hypertonia caused by aggravation on the spinal cord reflex when the inhibition of the upper airway is absent. Spasticity causes various functional and motor impairments for the child, making positioning and hygiene difficult. The study of the association of surgery and physical therapeutic treatments that provide control of spasticity is of fundamental importance. Objective: To evaluate the impact of selective dorsalrhizotomy (SDR) in the clinical framework of spastic children and the routine of daily care. Methods: The study included seven children withspasticity, 4-5 GMFCS, 5 to 11 years old. Before and after surgery, the following data were evaluated: the degree of spasticity of the adductor muscle groups of the hip and hamstrings in the legs and the elbow flexor group of upper limbs by using a Modified Ashworth Scale, and the measurement of the unilateral and bilateral popliteal angle, the angle of hip abduction and ankle dorsiflexion by using goniometry. In addition, a questionnaire was applied to families to assess the degree of difficulty for the daily care and satisfaction after the SDR. Conclusions: Reduction of post-operative spasticity in all muscle groups tested in all patients. There was a significant change in the angle goniometry for bilateral popliteal movement (p <0.05). Of the seven families questioned, six (85.7%) reported improvement of the positioning, feeding, hygiene and easy-to-install orthoses. Thus, SDR appears as an option to medical treatment in the treatment of spasticity in refractory cases in CP children with severe spastic quadriplegia, being able to improve their quality of life and that of their care-givers.