Management of drooling in children with cerebral palsy: A French survey.

AIM: To characterise children with cerebral palsy (CP) and pathological drooling in France, and to describe care pathways, assessment and treatment. METHOD: A transversal, observational, descriptive survey of the practices and opinions of 400 health professionals potentially involved in the care of children with CP, was carried out nationally across France in 2013. RESULTS: The response rate was 36%. Seventy-five questionnaires were returned and analysed (52%). A small proportion of children were specifically treated for drooling (<25%). Assessments were carried out in 75% of cases and 91% of professionals prescribed treatments. Use of assessment tools varied widely. The most common treatment was oro-facial rehabilitation (95% of professionals), followed by anticholinergic drugs (Scopolamine(®)) (94%) botulinum toxin injections (BT) (66%) and surgery (34%). Scopolamine was considered to be less effective than BT and to have more side effects. CONCLUSION: The rate of pathological drooling in children with CP is likely underestimated and under treated in France. There is a lack of knowledge regarding assessment tools. Aside from rehabilitation, current practice is to prescribe medication as the first-line treatment, however professionals consider that BT is more effective and has less side effects.

Physical and rehabilitation medicine (PRM) care pathways: "stroke patients".

This document is part of a series of documents designed by the French Physical and Rehabilitation Medicine Society (SOFMER) and the French Federation of PRM (FEDMER). These documents describe the needs for a specific type of patients; PRM care objectives, human and material resources to be implemented, chronology as well as expected outcomes. "Care pathways in PRM" is a short document designed to enable the reader (physicians, decision-maker, administrator, lawyer or finance manager) to quickly apprehend the needs of these patients and the available therapeutic care structures for proper organization and pricing of these activities. Stroke patients are divided into four categories according to the severity of the impairments, each one being treated according to the same six parameters according to the International Classification of Functioning, Disability and Health (WHO), while taking into account personal and environmental factors that could influence the needs of these patients.

Efficiency of flexible derotator in walking cerebral palsy children.

INTRODUCTION: The flexible derotator is one of the therapeutic resources used to combat primary and secondary abnormalities in walking cerebral palsy children. It was developed to reduce abnormal femoral and tibial torsions and lessen the latter's negative functional impact. OBJECTIVE: To determine the effect of wearing a flexible derotator on anatomic and functional parameters in walking cerebral palsy children. METHODS: We performed a retrospective study of walking cerebral palsy children by gathering data on bone-related parameters (femoral and tibial torsion) and functional parameters (distance and speed gait, and the energy expenditure index (EEI)). Fifteen walking cerebral palsy children were treated with the flexible derotator for one year and 15 untreated walking cerebral palsy children were included as controls. The two groups were compared in terms of the various parameters' change over time between the initial examination (the last examination prior to the start of the study or prior to use of the flexible derotator) and the final examination (after one year of follow-up). RESULTS: Right femoral anteversion and right and left external tibial torsion improved. There was a significant increase in distance and speed gait and a decrease in the EEI in walking cerebral palsy children. CONCLUSION: Our retrospective study revealed a significant improvement in functional parameters in children with cerebral palsy, as a result of wearing the flexible derotator for at least 6 hours a day for a year. Bone parameters only improved slightly. Use of the flexible derotator could improve these children's quality of life.

Clinical, electrophysiological and genetic studies of two families with mutations in the GDAP1 gene.

Mutations in the gene for the ganglioside-induced-differentiation-associated-protein 1 on 8q21 were recently reported to cause autosomal recessive Charcot-Marie-Tooth sensorimotor neuropathy. We report a detailed clinical, electrophysiological and genetic study of two young patients harbouring missense GDAP1 mutations. The two patients presented severe neuropathy with an early onset. One of the mutations (Tyr279Cys) has never been hitherto reported. Electrophysiological investigations suggested a predominant axonal damage in both patients. Despite the similitude of the type of mutations and electromyographic features, the clinical course was different for the patients, highlighting the complexity of genotype/phenotype relationships among GDAP1 mutations.

Training programs for children: literature review.

INTRODUCTION: Training programs are increasingly being prescribed for occupational therapy for adults affected by chronic illness and/or handicap, but their use is more recent for children and teenagers. OBJECTIVE: A review of the literature to synthesize information concerning training programs for children, whether healthy or with disease or handicap, considering the target population, methodology, the results and limitations. METHODS: We searched the Medline database with use of the key words retraining, training, training programs, physical activity, physical training, fitness program, sport, children, disability, and handicap. We also searched references of the selected articles for appropriate studies. DISCUSSION/CONCLUSION: Physical activity seems to be a good means of primary preventing adverse health in the healthy child and secondary prevention in children with chronic disease or handicap. Thus, training programs could be adapted and integrated into the global treatment of sick or handicapped children in the health care situation or in the home. These programs are feasible and do not undermine children's health, but few studies have shown clear data on the methods of the programs. The suggested training programs, not always validated, included two to five sessions from 30 to 60 min/week, for 6-16 weeks, of variable activity and intensity, adapted to the condition and the objectives of therapy. Training programs adapted to children should be validated to allow their accessibility by health care professionals dealing with children with chronic disease and/or handicap.

[Quality of life of a population of 54 ambulatory children with cerebral palsy. A cross-sectional study]. / Qualité de vie d'une population de 54 enfants infirmes moteurs cérébraux marchants. Etude transversale.

UNLABELLED: To evaluate the impact of handicap on quality of life in children with cerebral palsy and living in family. METHOD: A descriptive study by investigation was conducted in children with cerebral palsy, with the ability to walk, from 4 to 12 years of age, and in their family. Quality of life was evaluated with the AUQUEI questionnaire. Another questionnaire was given to the parents to collect the different variables, which could influence quality of life of the child. RESULTS: The mean age was 9 years. Associations between the studied variables and the quality of life were observed for motor function, number of siblings and the quantity of hours of reeducation given by the family. Parents evaluated their quality of life on the visual scale at 72.23 mm (95CI: 51.60-92.84). There was no correlation between the assessment of quality of life of parents and children. DISCUSSION-CONCLUSION: The better the child's motor function the less satisfied he was. This result shows the poor correlation between quality of life and general condition, impairment or disability. The greater the number of children in the family the less satisfied the child was within the family and the more satisfied the child was at school. A handicapped child has perhaps a more difficult place in the close family and an easier time in a school where the environment is more neutral. The more time that is devoted by the family to reeducation the less the child is satisfied. Quality of life seems to be influenced negatively by the demands of physical therapy, which do not correspond to the reality of the abilities of the child.

A rational approach to improving productivity in recombinant Pichia pastoris fermentation.

A Mut(S) Pichia pastoris strain that had been genetically modified to produce and secrete sea raven antifreeze protein was used as a model system to demonstrate the implementation of a rational, model-based approach to improve process productivity. A set of glycerol/methanol mixed-feed continuous stirred-tank reactor (CSTR) experiments was performed at the 5-L scale to characterize the relationship between the specific growth rate and the cell yield on methanol, the specific methanol consumption rate, the specific recombinant protein formation rate, and the productivity based on secreted protein levels. The range of dilution rates studied was 0. 01 to 0.10 h(-1), and the residual methanol concentration was kept constant at approximately 2 g/L (below the inhibitory level). With the assumption that the cell yield on glycerol was constant, the cell yield on methanol increased from approximately 0.5 to 1.5 over the range studied. A maximum specific methanol consumption rate of 20 mg/g. h was achieved at a dilution rate of 0.06 h(-1). The specific product formation rate and the volumetric productivity based on product continued to increase over the range of dilution rates studied, and the maximum values were 0.06 mg/g. h and 1.7 mg/L. h, respectively. Therefore, no evidence of repression by glycerol was observed over this range, and operating at the highest dilution rate studied maximized productivity. Fed-batch mass balance equations, based on Monod-type kinetics and parameters derived from data collected during the CSTR work, were then used to predict cell growth and recombinant protein production and to develop an exponential feeding strategy using two carbon sources. Two exponential fed-batch fermentations were conducted according to the predicted feeding strategy at specific growth rates of 0.03 h(-1) and 0.07 h(-1) to verify the accuracy of the model. Cell growth was accurately predicted in both fed-batch runs; however, the model underestimated recombinant product concentration. The overall volumetric productivity of both runs was approximately 2.2 mg/L. h, representing a tenfold increase in the productivity compared with a heuristic feeding strategy.