A escoliose é considerada a quinta patologia mais comum em desenvolvimento na adolescência. Sendo que no Brasil a prevalência desta patologia varia de 2% a 4% em adolescentes na faixa etária de 10 a 16 anos. O método Pilates pode ser considerado como alternativa no tratamento conservador desse desvio postural. O objetivo deste estudo foi avaliar o método Pilates no alinhamento postural de adolescentes portadores de escoliose, por meio da eletromiografia e da simetrografia. Foi realizado um estudo exploratório no período de Fevereiro a Abril de 2015, com 22 adolescentes, com idade de 12 a 18 anos, de ambos os sexos, que apresentaram o desvio postural escoliose, fisicamente ativos, sem experiência em treinamento resistido do Método Pilates e que não tinham realizado cirurgia corretiva ortopédica na coluna vertebral e nem recebido diagnóstico de doença reumática. Os voluntários pesquisados preencheram termo de assentimento informado livre e esclarecido e os responsáveis preencheram o termo de consentimento livre e esclarecido. Os adolescentes investigados inicialmente foram submetidos à avaliação antropométrica, percentual de gordura, simetria corporal, teste de Adams e avaliação eletromiográfica nos músculos trapézio, eretores espinhais, oblíquos e reto abdominal, na posição sentada equilibrando um livro sobre a cabeça processados no domínio temporal (EMGAmp) e espectral (EMGFmed). Na sequência foram submetidos á 24 sessões com dois atendimentos semanais de um protocolo de tratamento constituído por exercícios do método Pilates. Após a aplicação deste protocolo, foram novamente submetidos às avaliações iniciais. Os dados foram analisados pela ANOVA e processados utilizando o software estatístico R versão 3.2.0. e teste de McNemar. Os resultados com relação à escoliose mostraram que nove adolescentes melhoraram o alinhamento postural (40,9%) (p=0,001) e também no encurtamento do trapézio (p=0.031) e nos ombros assimétricos (p=0.001). Relativo à EMGAmp na escoliose postural à direita (EPD) houve diferença entre antes e depois (p=0.081) e também entre os músculos (p=0.031); na escoliose torácica à direita (ETD) houve diferença entre antes e depois (p≈0), o lado direito do esquerdo (p=0.04) e também entre os músculos (p≈0); na escoliose torácica à esquerda (ETE) e na escoliose tóraco-lombar destro convexa (ETLDC) houve diferença entre antes e depois (p≈0) e entre os músculos (p≈0). Referente à EMGFmed na EPD houve diferença entre antes e depois (p≈0) e entre os músculos (p=0.001); na ETD houve diferença entre antes e depois (p≈0) e entre os músculos (p=0.007); na ETE e ETLDC houve diferença apenas entre antes e depois (p≈0); na ETLE houve diferença entre antes e depois (p=0.007). Em todos os casos de escoliose estudados, houve aumento da EMGFmed com destaque para os músculos OBLI e RA. Verificaram-se diferenças estatísticas nas variáveis estatura (p=0,003) e aumento na flexibilidade (p=0,000). Conclui-se que os resultados apontam para a importância dos exercícios do método Pilates como forma de tratamento conservador da escoliose, ocasionando mudanças no padrão de ativação neuromuscular. Porém em relação ao tempo de aplicação terapêutico relativo ao alinhamento postural, sugere-se desenvolvê-lo em prazos maiores. / Scoliosis is considered the fifth most common pathology in development during adolescence. And in Brazil the prevalence of this disease ranges from 2% to 4% in adolescents aged 10-16 years old. The Pilates method can be considered as an alternative to conservative treatment of this postural deviation. The aim of this study was to evaluate the Pilates method in the postural alignment of adolescents with scoliosis through electromyography and symmetrography. An exploratory study was conducted from February to April 2015, with 22 12 to 18 year old male and female adolescents who had the scoliosis postural deviation, physically active, without experience in resistance training of Pilates and who had not performed orthopedic corrective surgery on the spine and not been diagnosed with rheumatic disease. Both surveyed volunteers and their parents filled informed consents. The investigated teenagers initially underwent anthropometric measurements, body fat percentage, body symmetry, Adams test and electromyographic assessment in trapezius, erector spinal, obliques and rectus abdominis, in a seated position balancing a book on their heads processed in the time (EMGAmp ) and spectral domains (EMGFmed). After that they were submitted to 24 sessions with two weekly appointments of a treatment protocol consisting of Pilates exercises. After the application of this protocol, they were again subjected to initial assessments. Data were analyzed by ANOVA and processed by using the statistical software R version 3.2.0. and McNemar test. Results regarding scoliosis showed that nine adolescents improved postural alignment (40.9%) (p = 0.001) and there was also an improvement in the shortening of the trapezius (p = 0.031) and asymmetrical shoulders (p = 0.001). Concerning the EMGAmp in right postural scoliosis (EPD), there was a significant difference between before and after (p = 0.081) and also between muscles (p = 0.031); in the right thoracic scoliosis (ETD) there was a difference between before and after (p≈0), the right side of the left (p = 0:04) and between muscles (p≈0); in the left thoracic scoliosis (ETE) and convex left thoracolumbar scoliosis (ETLDC) differences between before and after (p≈0) and between muscles (p≈0) were found. Concerning the EMGFmed in the EPD there was a difference between before and after (p≈0) and between muscles (p = 0.001); in the ETD there was a difference between before and after (p≈0) and between muscles (p = 0.007); in ETE and ETLDC there was a difference only between before and after (p≈0); in ETLE there was a difference between before and after (p = 0.007). In all cases of scoliosis studied here, there was an increase of EMGFmed notably in the OBLI and RA muscles. There were statistical differences in height variables (p = 0.003) and increased flexibility (p = 0.000). One can conclude that the results point to the importance of Pilates exercises as a form of conservative treatment of scoliosis, causing changes in the pattern of neuromuscular activation. However, in relation to the therapeutic application time relative to postural alignment, it is suggested to develop it in longer periods.

This project was undertaken with the following aims: 1) To generate a data base concerning the management of children with congenital spinal anomalies, who are known to form a significant proportion of the patients being treated in the Department of Paediatric Urology at Red Cross Hospital. 2) To assess the results of the management of these children, with special attention to the goals of therapy, namely preservation of renal function, establishment of urinary continence and protection from urinary tract infection. 3) To compare the treatment methods and results obtained to those reported in the literature. 4) To evaluate critically the treatment methods and results obtained with a view to identifying areas where improvements are possible.

Indiana University-Purdue University Indianapolis (IUPUI) / Skeletal manifestations including short stature, osteoporosis, kyphoscoliosis, and tibial dysplasia cumulatively affect approximately 70% of patients with neurofibromatosis type 1 (NF1). Tibial pseudarthrosis, the chronic non-union of a spontaneous fracture, is a debilitating skeletal malady affecting young children with NF1. These non-healing fractures respond poorly to treatment and often require amputation of the affected limb due to limited understanding of the causative mechanisms. To better understand the cellular and molecular pathogenesis of these osseous defects, we have established a new mouse model which recapitulates a spectrum of skeletal pathologies frequently observed in patients with NF1. Nf1flox/-;Col2.3Cre mice, harboring Nf1 nullizygous osteoblasts on a Nf1+/- background, exhibit multiple osseous defects which are closely reminiscent of those found in NF1 patients, including runting (short stature), bone mass deficits, spinal deformities, and tibial fracture non-union. Through adoptive bone marrow transfer studies, we have demonstrated that the Nf1 haploinsufficient hematopoietic system pivotally mediates the pathogenesis of bone loss and fracture non-union in Nf1flox/-;Col2.3Cre mice. By genetic ablation of a single Nf1 allele in early myeloid development, under the control of LysMCre, we have further delineated that Nf1 haploinsufficient myeloid progenitors and osteoclasts are the culprit lineages mediating accelerated bone loss. Interestingly, conditional Nf1 haploinsufficiency in mature osteoclasts, induced by CtskCre, was insufficient to trigger enhanced lytic activity. These data provide direct genetic evidence for Nf1’s temporal significance as a gatekeeper of the osteoclast progenitor pool in primitive myelopoiesis. On the molecular level, we found that transforming growth factor-beta1 (TGF-β1), a primary mediator in the spatiotemporal coupling of bone remodeling, is pathologically overexpressed by five- to six- fold in both NF1 patients and in mice. Nf1 deficient osteoblasts, the principal source of TGF-β1 in the bone matrix, overexpress TGF-β1 in a gene dosage dependent fashion. Moreover, p21Ras dependent hyperactivation of the Smad pathway accentuates responses to pathological TGF-β1 signals in Nf1 deficient bone cells. As a proof of concept, we demonstrate that pharmacologic TβRI kinase inhibition can rescue bone mass defects and prevent tibial fracture non-union in Nf1flox/-;Col2.3Cre mice, suggesting that targeting TGF-β1 signaling in myeloid lineages may provide therapeutic benefit for treating NF1 skeletal defects.