Impacto de las cirugías múltiples, en un solo tiempo quirúrgico, sobre el patrón de marcha en niños con parálisis cerebral

Introducción: La Parálisis Cerebral (PC) es la enfermedad neurológica más incapacitante en niños, su historia natural tiende al deterioro motor y funcional. Con este estudio se busca establecer sí las cirugías múltiples de miembros inferiores, en un tiempo quirúrgico, mantienen el nivel motor y funcional. Material y Método: Estudio analítico de cohortes. Se compara un grupo de pacientes sometidos a cirugías múltiples contra un grupo de pacientes no operados, en el Instituto de Ortopedia Infantil Roosevelt. Se evaluaron los pacientes con dos Laboratorios para el Análisis del Movimiento (LAM) y se midieron los desenlaces mediante el cambio en la puntuación del perfil de marcha (GPS) y el nivel funcional motor grueso (GMFCS). Resultados: 109 pacientes cumplieron con los criterios de selección, 67 pacientes fueron sometidos a cirugía y 42 pacientes no. Los pacientes operados mejoraron el GPS promedio (diferencia -1,94; p=0,002) comparado con los pacientes no operados (diferencia 1,74; p=0,001), indicando una mejoría significativa de la cinemática de la marcha. En un modelo de regresión logística predictivo, el paciente que es operado tiene una probabilidad del 78% de mantener su patrón de marcha, mientras que sí no se opera su probabilidad disminuye al 37%. El nivel funcional motor GMFCS no mostró cambios significativos entre los grupos. Discusión: Las cirugías múltiples de miembros inferiores mantienen de manera significativa el patrón de marcha en pacientes con PC. Se destaca el seguimiento de los pacientes mediante el LAM y se sugiere el uso del GPS para valorar resultados en este tipo de pacientes.

Avaliação do desempenho funcional de crianças com paralisia cerebral de acordo com níveis de comprometimento motor

Contextualization: Several studies have examined the mobility of this group of children, however little is known about the impact of motor function in activities of daily living, considering the seriousness of their neuromotor damage. Objective: Identify the functional differences of children with Cerebral Palsy with different levels of motor dysfunction and correlate these differences with the areas of mobility, self-care and social function in functional ability and caregiver´s assistance of these children. Methods: An l analytical cross-section search was developed, which were part 70 children / families aged from 4 to 7.5 years, in the Rehabilitation Center for Children. As tools were used the Pediatric Evaluation Disability Inventory (PEDI) and the Gross Motor Function Classification System (GMFCS). Data analysis was performed by ANOVA and Pearson's correlation tests. Results: The results show the functional variability of children CP in different severity levels of motor disfunction This variation was observed in the areas of mobility, self-care and social function. The results also showed a strong correlation between the domains mobility and self-care, mobility and social function. Conclusions: The variability shown by the children with CP, suggests the use of PEDI and GMFCS as this association appears to increase the understanding of how the gross motor functions are related to activities of daily living, describing the best commitments and their degree of impact on functional activities. This correlation demonstrates how mobility is crucial to evaluate the performance and guide the therapeutic practice, to develop the children´s potencial, and guide the caregiver in stimulation

Padrões neuropatológicos nas substâncias branca e cinzenta revelados por tomografia computadorizada ou ressonância magnética e déficits neurológicos correspondentes em crianças e com encefalopatia crônica não progressiva da infância

A Encefalopatia Crônica Não Progressiva da Infância (ECNP) é a sequela neurológica com maior comprometimento motor para a criança, e continua sendo na atualidade a hipóxicoisquemia perinatal a maior causa de lesão cerebral. É conhecida como Paralisia Cerebral, sendo definida por uma sequela de agressão encefálica, caracterizada, principalmente, por um transtorno persistente, mas não invariável do tônus, da postura e do movimento, que aparece na primeira infância. A caracterização da ECNP se faz considerando as condições anatômicas, etiológicas, semiológicas e não evolutiva. Neste estudo adotou-se a classificação baseada em aspectos anatômicos e clínicos, que enfatizam o sintoma motor, enquanto elemento principal do quadro clínico. A neuroimagem tem fundamental importância para o diagnóstico e prognóstico de lesões cerebrais, exercendo a importante função de descartar ou confirmar a presença de lesões em recém-nascidos e nas crianças com alterações no desenvolvimento. A Tomografia Cerebral (TAC) e a Ressonância Magnética do Crânio (RM) vêm desempenhando enorme papel para o estudo dos vários tecidos que constituem o sistema nervoso. Assim este estudo teve o objetivo geral de avaliar os padrões neuropatológicos nas substâncias branca e cinzenta, obtidos por TAC ou RM de Crânio, de pacientes com história clínica de ECNP hipóxico-isquêmica perinatal, correlacionando os dados obtidos por neuroimagem com os padrões motores obtidos por exame clínico-neurológico. Foram obedecidas as normas vigentes para estudo em seres humanos impostas pela Resolução CNS 196/96, submetida ao Comitê de Ética e Pesquisa da Plataforma Brasil sob o Nº 112168. A população foi constituída por pacientes com idade de zero a sete anos, de ambos os sexos, atendidos no Ambulatório de Paralisia Cerebral do Projeto Caminhar do Hospital Universitário Bettina Ferro de Souza (HUBFS), com diagnóstico de ECNP. A amostra do estudo foi composta por 15 crianças com diagnóstico de ECNP por Hipóxia neonatal. Para o diagnóstico radiológico em neuroimagem foram utilizados os dados dos laudos da TAC e da RM de Crânio. A avaliação clínico-neurológica utilizou para a avaliação do movimento o modelo da escala Gross Motor Function Classification System (GMFCS E&R), elaborada por Palisano, que gradua a criança em cinco níveis no qual o Nível I corresponde à normalidade e o Nível V a maior gravidade de limitação. Das 15 crianças avaliadas quanto ao movimento e a relação do Nível de Motricidade pela GMFCS E&R 05 crianças apresentavam nível V, 04 crianças nível IV, 05 crianças nível III e 01 criança nível II. Quanto ao imageamento cerebral 46% realizaram TAC e 54% RM do Crânio. A RM de Crânio apresentou-se neste estudo como a imagem de eleição, pois das 8 crianças que realizaram o exame, 6 apresentavam alterações. Ficou evidente que o exame por imagem de eleição para a criança que apresenta Encefalopatia Crônica não Progressiva é a RM de Crânio, podendo se adotar como protocolo para a conclusão diagnóstica, evitando expor a criança a uma carga elevada de RX como ocorre na TAC, e ainda, evitando gastos desnecessários para a saúde pública.

Classificação do grau de comprometimento motor e do índice de massa corpórea em crianças com paralisia cerebral

Children with cerebral palsy due to movement and posture disorders might have an injured nutritional state. This study's objective was to classify the body mass index of children with cerebral palsy and verify its relation with the injury level of gross motor function. Twenty children, male and female sex, with cerebral palsy with ages between 2 and 14 years participated in the study. They were chosen from a rehabilitation center with physiotherapy, occupational and speech therapy accompaniment. Children were classified according to the gross motor function classification scale. The anthropometric measures of each child weight, heel-knee length, estimated stature and body mass index were gauged. The body mass index was classified according to the Center for Disease Control and Prevention's body mass index percentile calculator for children and teenagers. The obtained data didn't show any relation between the gross motor function and body mass index. The reduced number of participants and the fact of all children be accompanied in dysphagia clinics and, when necessary, nutrition clinics, might have influenced these results.

Qualidade de vida em portadores de paralisia cerebral: associação com nível funcional e aspectos sócio-demográficos

Pós-graduação em Saúde Coletiva - FMB

The updated European Consensus 2009 on the use of Botulinum toxin for children with cerebral palsy

An interdisciplinary European group of clinical experts in the field of movement disorders and experienced Botulinum toxin users has updated the consensus for the use of Botulinum toxin in the treatment of children with cerebral palsy (CP). A problem-orientated approach was used focussing on both published and practice-based evidence. In part I of the consensus the authors have tabulated the supporting evidence to produce a concise but comprehensive information base, pooling data and experience from 36 institutions in 9 European countries which involves more than 10,000 patients and over 45,000 treatment sessions during a period of more than 280 treatment years. In part II of the consensus the Gross Motor Function Measure (GMFM) and Gross Motor Function Classification System (GMFCS) based Motor Development Curves have been expanded to provide a graphical framework on how to treat the motor disorders in children with CP. This graph is named "CP(Graph) Treatment Modalities - Gross Motor Function" and is intended to facilitate communication between parents, therapists and medical doctors concerning (1) achievable motor function, (2) realistic goal-setting and (3) treatment perspectives for children with CP. The updated European consensus 2009 summarises the current understanding regarding an integrated, multidisciplinary treatment approach using Botulinum toxin for the treatment of children with CP.

Outcome at two years of age in a Swiss national cohort of extremely preterm infants born between 2000 and 2008

Background While survival rates of extremely preterm infants have improved over the last decades, the incidence of neurodevelopmental disability (ND) in survivors remains high. Representative current data on the severity of disability and of risk factors associated with poor outcome in this growing population are necessary for clinical guidance and parent counselling. Methods Prospective longitudinal multicentre cohort study of preterm infants born in Switzerland between 240/7 and 276/7 weeks gestational age during 2000–2008. Mortality, adverse outcome (death or severe ND) at two years, and predictors for poor outcome were analysed using multilevel multivariate logistic regression. Neurodevelopment was assessed using Bayley Scales of Infant Development II. Cerebral palsy was graded after the Gross Motor Function Classification System. Results Of 1266 live born infants, 422 (33%) died. Follow-up information was available for 684 (81%) survivors: 440 (64%) showed favourable outcome, 166 (24%) moderate ND, and 78 (11%) severe ND. At birth, lower gestational age, intrauterine growth restriction and absence of antenatal corticosteroids were associated with mortality and adverse outcome (p < 0.001). At 360/7 weeks postmenstrual age, bronchopulmonary dysplasia, major brain injury and retinopathy of prematurity were the main predictors for adverse outcome (p < 0.05). Survival without moderate or severe ND increased from 27% to 39% during the observation period (p = 0.02). Conclusions In this recent Swiss national cohort study of extremely preterm infants, neonatal mortality was determined by gestational age, birth weight, and antenatal corticosteroids while neurodevelopmental outcome was determined by the major neonatal morbidities. We observed an increase of survival without moderate or severe disability.

Highly efficient ketone body treatment in multiple acyl-CoA dehydrogenase deficiency-related leukodystrophy.

BACKGROUND Multiple acyl-CoA dehydrogenase deficiency- (MADD-), also called glutaric aciduria type 2, associated leukodystrophy may be severe and progressive despite conventional treatment with protein- and fat-restricted diet, carnitine, riboflavin, and coenzyme Q10. Administration of ketone bodies was described as a promising adjunct, but has only been documented once. METHODS We describe a Portuguese boy of consanguineous parents who developed progressive muscle weakness at 2.5 y of age, followed by severe metabolic decompensation with hypoglycaemia and coma triggered by a viral infection. Magnetic resonance (MR) imaging showed diffuse leukodystrophy. MADD was diagnosed by biochemical and molecular analyses. Clinical deterioration continued despite conventional treatment. Enteral sodium D,L-3-hydroxybutyrate (NaHB) was progressively introduced and maintained at 600 mg/kg BW/d (≈3% caloric need). Follow up was 3 y and included regular clinical examinations, biochemical studies, and imaging. RESULTS During follow up, the initial GMFC-MLD (motor function classification system, 0 = normal, 6 = maximum impairment) level of 5-6 gradually improved to 1 after 5 mo. Social functioning and quality of life recovered remarkably. We found considerable improvement of MR imaging and spectroscopy during follow up, with a certain lag behind clinical recovery. There was some persistent residual developmental delay. CONCLUSION NaHB is a highly effective and safe treatment that needs further controlled studies.

Uncertainty and Fuzzy Sets: Classifying the Situation

The so called “Plural Uncertainty Model” is considered, in which statistical, maxmin, interval and Fuzzy model of uncertainty are embedded. For the last case external and internal contradictions of the theory are investigated and the modified definition of the Fuzzy Sets is proposed to overcome the troubles of the classical variant of Fuzzy Subsets by L. Zadeh. The general variants of logit- and probit- regression are the model of the modified Fuzzy Sets. It is possible to say about observations within the modification of the theory. The conception of the “situation” is proposed within modified Fuzzy Theory and the classifying problem is considered. The algorithm of the classification for the situation is proposed being the analogue of the statistical MLM(maximum likelihood method). The example related possible observing the distribution from the collection of distribution is considered.

Validación de un instrumento para evaluar la carga del cuidador en parálisis cerebral

Esta investigación pretende desarrollar y validar una escala para evaluar la carga del cuidador del niño con parálisis cerebral (PC) de niveles funcionales GMFCS (Gross Motor Function Classification System) IV y V.

Intrinsically photosensitive retinal ganglion cells: classification, function and clinical implications.

PURPOSE OF REVIEW: The discovery of a new class of intrinsically photosensitive retinal ganglion cells (ipRGCs) revealed their superior role for various nonvisual biological functions, including the pupil light reflex, and circadian photoentrainment. RECENT FINDINGS: Recent works have identified and characterized several anatomically and functionally distinct ipRGC subtypes and have added strong new evidence for the accessory role of ipRGCs in the visual system in humans. SUMMARY: This review summarizes current concepts related to ipRGC morphology, central connections and behavioural functions and highlights recent studies having clinical relevance to ipRGCs. Clinical implications of the melanopsin system are widespread, particularly as related to chronobiology.

Fully complex-valued radial basis function networks: orthogonal least squares regression and classification

We consider a fully complex-valued radial basis function (RBF) network for regression and classification applications. For regression problems, the locally regularised orthogonal least squares (LROLS) algorithm aided with the D-optimality experimental design, originally derived for constructing parsimonious real-valued RBF models, is extended to the fully complex-valued RBF (CVRBF) network. Like its real-valued counterpart, the proposed algorithm aims to achieve maximised model robustness and sparsity by combining two effective and complementary approaches. The LROLS algorithm alone is capable of producing a very parsimonious model with excellent generalisation performance while the D-optimality design criterion further enhances the model efficiency and robustness. By specifying an appropriate weighting for the D-optimality cost in the combined model selecting criterion, the entire model construction procedure becomes automatic. An example of identifying a complex-valued nonlinear channel is used to illustrate the regression application of the proposed fully CVRBF network. The proposed fully CVRBF network is also applied to four-class classification problems that are typically encountered in communication systems. A complex-valued orthogonal forward selection algorithm based on the multi-class Fisher ratio of class separability measure is derived for constructing sparse CVRBF classifiers that generalise well. The effectiveness of the proposed algorithm is demonstrated using the example of nonlinear beamforming for multiple-antenna aided communication systems that employ complex-valued quadrature phase shift keying modulation scheme. (C) 2007 Elsevier B.V. All rights reserved.

Need for a Government-wide budget classification structure for Federal research and development information : report to the Congress /

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Unicellular pheromone glands of the pentatomid bug Nezara viridula (Heteroptera : Insecta): Ultrastructure, classification, and proposed function

Male Nezara viridula produce sex pheromones from many independent single cells, each with a duct that opens onto the ventral abdominal surface. Despite the presence of along duct and an associated end complex (in the form of a cupule and microvillus saccule), the structural organization of the cells that comprise the gland conform to Class 1 epidermal gland cell classification : a single cell surrounds the entire secretory complex. Each cuticular cupule contains a central bed of filaments and opens into a narrow tubular ductule that leads from the base of the cupule through the epidermis to the cuticle to open externally as a pore. The cuticle of the cupule is continuous with that of the ductule and has the appearance of three layers, although the inner (middle) layer may be a gap formed during construction of the complex. In young adult males, just molted, the ultrastructure of the cells and their inclusions indicate that they are not active. The region of the cell that is distal to the abdominal cuticle is reduced and the proximal region, surrounding the duct, is enlarged when compared with sexually mature (3-4 weeks old) adult males. At maturity the pheromone cells are enlarged distally around the cupule, but are reduced to a narrow sleeve proximally, around the ductule. Two characteristic cell profiles are evident, based on the shape of the cupule and the organelle content. Type A shows a broad opening to the cupule, an abundance of mitochondria, and few vesicular bodies. Type B has an elongated, narrow, vase-like opening to the cupule, few mitochondria, and numerous vesicular bodies. Type B cells are smaller and more abundant than Type A. Distribution within the epidermal layer also differs. It is likely that the different types represent cells producing different secretion profiles. However, the secretions retained by the standard fixation protocol within mature cells of both types look similar and appear to collect as crystalline bodies within the lumen. This may represent a common storage mechanism.

GPCRTree:online hierarchical classification of GPCR function

G protein-coupled receptors (GPCRs) play important physiological roles transducing extracellular signals into intracellular responses. Approximately 50% of all marketed drugs target a GPCR. There remains considerable interest in effectively predicting the function of a GPCR from its primary sequence.