Undernutrition in children with profound intellectual and multiple disabilities (PIMD): its prevalence and influence on quality of life.

BACKGROUND: To estimate the prevalence of undernutrition among children with profound intellectual and multiple disabilities (PIMD) and to explore its influence on quality of life. METHODS: Seventy-two children with PIMD (47 male; 25 female; age range 2 to 15 years 4 months; mean age 8.6, SD 3.6) underwent an anthropometric assessment, including body weight, triceps skinfold thickness, segmental measures and recumbent length. Undernutrition was determined using tricipital skinfold percentile and z-scores of weight-for-height and height-for-age. The quality of life of each child was evaluated using the QUALIN questionnaire adapted for profoundly disabled children. RESULTS: Twenty-five children (34.7%) were undernourished and seven (9.7%) were obese. Among undernourished children only eight (32 %) were receiving food supplements and two (8%) had a gastrostomy, of which one was still on a refeeding programme. On multivariate analysis, undernutrition was one of the independent predictors of lower quality of life. CONCLUSION: Undernutrition remains a matter of concern in children with PIMD. There is a need to better train professionals in systematically assessing the nutritional status of profoundly disabled children in order to start nutritional management when necessary.

[Quality of life of very disabled children: a questionnaire about the role of health status and tube feeding]. / Qualité de vie de l'enfant polyhandicapé: questionnaire portant sur l'influence de l'état de santé et de l'alimentation entérale.

UNLABELLED: To resort to gastrostomy feeding is a difficult decision to take in children with severe disability, the objective being better quality of life. OBJECTIVES: To describe quality of life in children with severe disability and to look for factors which influence this quality of life, in particular gastrostomy. METHOD: Descriptive study in 28 patients, aged 1 to 18 years, with severe motor and mental disability, with or without gastrostomy. The studied factors were: health and nutritional status and quality of life. The quality of life was evaluated with QUALIN questionnaire, specifically designed for infants. RESULTS: This questionnaire was pertinent and well accepted by the family. Quality of life was influenced by capacity of communication (P=0.006), quality of sleeping (P=0.004), digestive problems (P<0.05) and age (P=0.031). CONCLUSION: Gastrostomy did not impair quality of life. Its benefit was estimated as high as 8.71/10 in average by parents.

[Scoliosis outcome in cerebral palsy patients with total body involvement: a descriptive study of 61 children and adults, with or without spinal fusion]. / Retentissement de la scoliose chez les patients polyhandicapés: étude descriptive de 61 enfants et adultes polyhandicapés avec ou sans arthrodèse vertébrale.

INTRODUCTION: The literature is controversial about how to manage scoliosis in cerebral palsy patients with total body involvement. Spinal fusion is admitted as the only means to stop the evolution of the curve but remains associated with many complications. OBJECTIVES: The aim of this descriptive study was to formulate hypotheses concerning the interest of spinal fusion in this population. METHODS: This descriptive cross-sectional study was conducted in 61 patients aged 4 to 48 years (mean 24 years, median 20 years) with severe scoliosis (Cobb score>40 degrees ). Each patient had undergone recent X-ray of the spine and hips and a clinical exam. Data were collected on orthopaedic and functional status, health level (pulmonary, digestive, nutrition, skin parameters), pain, and subjective quality of life (sQoL). RESULTS: Data were collected for all 61 patients (35 females, 26 males), 16 underwent spinal fusion at a mean age of 16.5 years (prevalence of 32.0%). No significant difference was found between patients with a spinal instrumentation and others in variables tested. A difference might be present with functional level, but only results from a longitudinal study would be conclusive. Pain was prevalent (19.7%), not treated sufficiently (only 50% receive treatment), and contributes to poor sQoL (p<0.042). Among the other data collected, nutritional level has the greatest influence on general health status (p<0.05). CONCLUSIONS: Systematic spinal fusion cannot be generalized for this population of patients with cerebral palsy and total body involvement. However, health status and quality of life could be improved with better care of nutritional problems and pain.

[Pain, osteopenia and body composition of 22 patients with Duchenne muscular dystrophy: a descriptive study]. / Douleur, ostéopénie et composition corporelle chez 22 patients atteints de dystrophie musculaire progressive de Duchenne. Etude descriptive.

OBJECTIVES: To study the link between pain, osteopenia and body composition in patients with Duchenne muscular dystrophy and to present a detailed questionnaire to evaluate their pain. MATERIALS AND METHODS: Twenty-two boys with Duchenne muscular dystrophy, mean age 11.4+/-4.0 years, were examined between February and March 2003. They were asked to complete a detailed questionnaire and undergo a global assessment of pain on a visual analog scale and muscular testing. They were also asked about a history of fractures. Their bone mineral content at the lumbar spine and femoral neck levels, as well as their body composition in fat and lean mass, were assessed by dual energy absorptiometry. RESULTS: The mean age for walking incapacity was 8.8+/-1.7 years. The youngest patients, who were still able to walk, had a higher level of pain than patients who depended on wheelchairs. No significant correlation was established between pain and osteopenia. One in 2 patients had spontaneous pain, and mobilization was painful for 21. The score obtained by detailed questioning about pain correlates with the average pain scores on visual analog scales. The bone mineral content was lower, especially in the lower limbs, had decreased before the inability to walk and was correlated with muscular weakness. Fractures were more frequent in mobile patients and usually occurred after a fall. CONCLUSION: Although pain in Duchenne muscular dystrophy has not been extensively studied, it is frequent and significant. Twenty-one patients had moderate to severe pain. The youngest patients had intense pain, especially during mobilisation. To evaluate this pain, we propose to use the mean results of 2 visual analog scales associated with a detailed questionnaire. However, in this study, Duchenne muscular dystrophy, pain and osteopenia were not correlated. Dual X-ray absorptiometry provides interesting information about bone mineral content, fat body mass and lean body mass. The fat body mass was higher than normal in our patients. The bone mineral content and lean body mass were lower than that for normal children, because the dystrophic process advances with age. The fracture prevalence was high, especially in young patients. Falling was the most common mechanism of fracture.

Mandibular bone mineral density as a predictor of skeletal osteoporosis.

A considerable amount of work has been performed on methods of detecting individuals with low bone mass at an early stage. Some researchers have considered if dental radiographs could have a role in the detection of individuals with osteoporosis. A basic requirement for this would be that bone mass in the jaw relates significantly to that of other skeletal sites in which osteoporosis is a significant problem. The first aim of this study was to investigate the relationship between mandibular bone mineral density (BMD) and that of other skeletal sites commonly used for bone densitometry in the detection of osteoporosis. The second aim was to assess the validity of mandibular BMD as a predictor of BMD in these other sites. 40 edentulous females underwent dual energy X-ray absorptiometry (DXA) of the lumbar spine (L2-L4), DXA of the right femoral neck, single photon absorptiometry (SPA) of the proximal and distal forearm and DXA of the mandible. Significant correlations were observed between BMD in the mandibular body, ramus and symphysis and all other skeletal sites (p < 0.02). Five patients (12.5%) had age matched Z-scores of -1.0 or lower in all three non-mandibular sites (lumbar spine, femoral neck and forearm). Using these patients as the proportion of the population with a positive finding of "low bone mass", the sensitivity and specificity of mandibular BMD in predicting low bone mass for these patients was determined. Where a diagnostic threshold for low mandibular BMD was set at one standard deviation below the mean, the mandibular body BMD measurement gave high sensitivity (0.8) and specificity (0.97), the symphysis BMD low sensitivity (0.4) but a high specificity (0.77), while the ramus BMD had a moderate level of sensitivity (0.6) and high specificity (0.91). It is concluded that mandibular BMD assessed by DXA correlates significantly with BMD measurements of other important skeletal sites. The higher correlation coefficients and the greater sensitivity and specificity for the body of mandible suggest that this site should be used for any potential clinical application of dental radiographs in detection of osteoporosis.

Reflected line spread function of the human ocular fundus.

Characterisation of the reflection from the fundus of the eye is important for the development of an improved theoretical description of photoscreening. Measurement has been made of the reflected line spread function of the human fundus in vivo using a slit-lamp microscope with video camera and frame-grabber. Line spread functions representative of red, green, and blue light were obtained from fundus sites just temporal to the macula, 10 degrees temporal to the macula, away from the macula temporal, and 30 degrees temporal to the macula. The results show that the lateral spreading of light is greatest for red light, and support the view that red light penetrates further into the fundus before being reflected than green or blue.

Chromatic analysis of photorefraction: a model for the Otago photoscreener.

A model is presented for photorefractive testing of the living eye with the circular knife-edge Otago photoscreener. Wavelength-dependent fundal remittance and line spread functions are introduced and used to account for the yellow-orange-red range of colours observed in pupillary images. The response of the photoscreener to errors of focus is described in terms of luminance and dominant wavelength profiles.

Pupillary irradiance distributions for light backscattered in the human eye.

An investigation of linear knife-edge photoscreening devices for detecting focusing errors, refractive defects and other optical artifacts in the human eye is described. Pupillary irradiance distributions calculated for a model eye are shown to agree qualitatively with distributions recorded experimentally using video frame grabbing equipment. The form of the pupillary irradiance distribution from a cyclopleged human eye supports a diffuse-like characterization for the reflection/scattering processes at the retina. The extreme sensitivity of the instruments enables real-time detection of refractive effects from tear films on the cornea and real-time tracking of floaters.

[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.

[Pelvis obliquity, hip excentration and scoliosis in a population of 120 polyhandicaped adults. Descriptive study]. / Bassin oblique, luxation de hanche et scoliose dans une population de 120 adultes polyhandicapés.Etude descriptive.

UNLABELLED: The objective of this study is to give objective data about neuro-orthopaedic status of the population of polyhandicapped patients for improving both our medical and surgical practices. MATERIAL AND METHOD: It was a multicentre, cross-sectional study to describe the population of polyhandicapped patients over 15 years of age. Each person was provided with clinical examination and X rays to detect scoliosis, pelvis obliquity and hip excentration (subluxation or dislocation) and other factors associated with polyhandicap. Polyhandicap was defined as a severe handicap linking physical and mental disability and responsible for extreme restriction of autonomy. RESULTS: One hundred and twelve persons were studied. Mean age was 27.2 years. Etiology of the handicap was neonatal asphyxia (38.3% of patients). 69.0% of patients had no possibility to turn over in lying position. Orthopaedic status was serious : 75.9% scoliosis, 65.2% pelvis obliquity (75% left and 25% right), 19.1% hip dislocation. General condition was precarious : one patient in two had a weight judged lower than normal weight, 32.5% had bedsores, 45.2% suffered from hip pain and only 13.6% received treatment against pain. DISCUSSION AND CONCLUSION: The description of general and neuro-orthopaedic status of these 120 people with polyhandicap cannot leave us indifferent. Although life expectancy in this population was lower than in normal populations, we are faced with life process and no with accompanying the terminally ill. We urgently need to review preventive care in terms of quality and quantity as well as surgical proposals for treating spasticity in children and adults.

[Pelvic obliquity and scoliosis in non-ambulatory patients with cerebral palsy: a descriptive study of 234 patients over 15 years of age]. / Bassin oblique et scoliose chez le patient infirme moteur cérébral non-marchant: étude descriptive de 234 patients de plus de 15 ans.

PURPOSE OF THE STUDY: Children with cerebral palsy who cannot walk have an oblique pelvis and scoliosis. There is a certain degree of controversy in the literature on the best way to manage this difficult situation. We present a descriptive analysis of a population of non-ambulatory adults with cerebral palsy in order to formulate hypotheses concerning the factors determining scoliosis. MATERIAL AND METHODS: This descriptive cross-sectional study was conducted in 234 patients aged over 15 years who had cerebral palsy and could not walk. Physical examination and an x-ray of the pelvis and spine in the reclining position were obtained for all patients. The following variables were recorded: luxation and subluxation of the hip, spontaneous deviation attitude, ability or not to turn over in bed, pelvic obliquity, history of bone surgery, defective hip abduction. The statistical analysis accounted for laterality and pelvis obliquity to the scoliosis convexity and the laterality of the hip excentration. RESULTS: Scoliosis was observed in 66.2% of the patients; it was more than 60 degrees in 34.5%. Two basic groups were distinguished: thoracolumbar scoliosis (41.6%) and lumbar scoliosis (41.6%). The prevalence of oblique pelvi was 59.9% with important difference by side: 31.6% right oblique and 68.4% left oblique pelvi. We were unable to find any relationship between the side of the pelvic obliquity and the side of the scoliosis convexity, the side of the hip excentration, or the deviation attitude, but the deviation attitude appeared to be a risk factor for pelvic obliquity, which itself was a risk factor for excentration, which was a risk factor for scoliosis. DISCUSSION: Scoliosis is an important problem in this population. Hip luxation is a direct risk factor for scoliosis, but the deviation attitude and pelvic obliquity are intermediary stages. The prevalence of oblique pelvi was greater on the left than the right. This finding should be confirmed in other series before hypotheses can be formulated concerning this difference.

[Prevalence and morbidity of hip excentration in cerebral palsy: review of the literature]. / Prévalence et morbidité de l'excentration de hanche du sujet infirme moteur cérébral: étude de la littérature.

PURPOSE OF THE STUDY: Excentration of the hip is one of the main orthopedic complications of ante and perinatal cerebral palsy. Excentration can progress to dislocation even if tenotomy is performed. What is the prevalence and morbidity of hip excentration? METHODS: We reviewed the pertinent literature on the prevalence and morbidity of hip excentration in patients with cerebral palsy based on the Medline and Reedoc databases. RESULTS: Data presented in the literature vary greatly. Populations are representative of the referral network of the teams reporting the study rather than the cerebral palsy population in general. Signs reported were pain, difficult nursing procedures, difficult sitting position, pressure sores and fractures, but only one sign can be taken fully into account: 50 p. cent of the dislocated hip joints were painful. DISCUSSION: No one study gives a precise assessment of the prevalence of hip excentration and displacement and its consequences in patients with cerebral palsy. In a population of quadriplegic patients who do not walk, the question is whether complementary tenotomy should be performed, knowking the difficult operative and postoperative situation of such procedures. In this population, walking is an exceptional goal of bone surgery, pain relief and patient comfort are more common goals. Data in this literature (50 p. 100 of the dislocated hips are painful) suggest a prudent approach. However, there is no known marker which can be used to distinguish hips which will become painful from those which will remain pain free. In addition, irreversible cartilage degeneration can cause pain contraindicating reconstructive surgery.

[Initial management of congenital varus equinus clubfoot by Ponseti's method]. / Prise en charge initiale du pied bot varus équin congénital selon la méthode de Ponseti.

The choice of first-line treatment for congenital varus equine clubfoot remains a controversial issue largely dependent on experience. In France, functional treatment predominates. In 1948, Ponseti proposed reducing the deformity with successive casts. Although cast treatment is a very old method, Ponseti's method is original because it is based on strict rules established from anatomic evidence. The goal is not to correct the apparent deformation, but on the contrary to impose a simultaneous supination and abduction of the foot. Once the calcaneopedal block has been derotated, percutaneous tenotomy of the Achilles tendon is performed. We relate our experience with this method and recall the precise technique used to make the casts. After the cast, derotation braces are worn at night but rehabilitation exercises are not required. We emphasize the quality of the clinical reduction achieved as well as the smaller number of patients who require surgery at walking age.

[Neurosurgical treatment of spasticity: indications in children]. / Traitement neurochirurgical de la spasticité: indications chez l'enfant.

Today, we have several efficient neurosurgical treatments of spasticity in children with cerebral palsy. A good indication is possible only if a consensus about the goal of the surgery is found between the surgeon, the child and his/her family, and the reeducation team. This goal is not always functional. Clinical examination is not limited to the analytical assessment of spasticity, but must take into account the general and orthopedic state of the child, and his/her functional evolution, cognitive abilities, habits and general environment. The struggle against spasticity is part of a therapeutical programme which extends over several years. It must be considered before muscular contractures. On lower limbs, in the cases of general spasticity, we propose posterior rhizotomy or intrathecal baclofen administration. Posterior rhizotomy is proposed when a more aggressive intervention is preferred for some muscular groups or when the child's general environment does not allow for the medical care imposed by intrathecal administration. In the case of localized spasticity, botulinum toxin injection permits delaying until the child reaches the age for selective neurotomy. On upper limbs, in children with quadriplegia the indication is essentially physical and occupational therapy. We cannot count on the positive side effects of rhizotomy or intrathecal administration of baclofen on the upper limbs. In children with hemiplegia, with localized or global spasticity of the upper limb, botulinum toxin is proposed as the first form of treatment. Neurotomy or rhizotomy can follow toxin, according to the efficacy of the toxin.

[Clinical assessment of spasticity in children]. / Evaluation clinique de l'enfant spastique.

This article presents the four principle steps of assessment of children with cerebral palsy. Weakness of some muscles groups and spasticity of others and the subsequent functional impairment can be identified by observation. Analytical assessments of spasticity with the Ashworth and Tardieu scales were compared in 30 children with cerebral palsy, mean age 8.5 years. The results pointed out the complimentary nature of these two scales: The Gross Motor Function Measure, which is a validated scale, is specific for children with cerebral palsy. This measure provides a useful assessment of outcome and a convenient means of evaluating the time-course. Joint assessment is also essential to identify muscle contracture. Assessment of spasticity in children with cerebral palsy must be conducted by a multidisciplinary team to take into account all the aspects involved.

A French observational study of botulinum toxin use in the management of children with cerebral palsy: BOTULOSCOPE.

BACKGROUND: Dystonia and spasticity are common symptoms in children with Cerebral Palsy (CP), whose management is a challenge to overcome in order to enable the harmonized development of motor function during growth. AIM: To describe botulinum toxin A (BTX-A) use and efficacy as a treatment of focal spasticity in CP children in France. METHODS: This prospective observational study included 282 CP children mostly administered according to French standards with BTX-A in lower limbs. Realistic therapeutic objectives were set with parents and children together before treatment initiation and assessed using the Visual Analogue Scale (VAS). Child management was recorded and the efficacy of injections was assessed during a 12-month follow-up period by physicians (Modified Ashworth Scale, joint range of motion, Physician Rating Scale, Gillette Functional Assessment Questionnaire and Gross Motor Function Measure-66) and by patients/parents (Visual Analogue Scale). RESULTS: BTX-A treatment was administered in different muscle localizations at once and at doses higher than those recommended by the French Health Authorities. Children were treated in parallel by physiotherapy, casts and ortheses. Injections reduced spasticity and improved joint range of motion, gait pattern and movement capacity. Pain was reduced after injections. BTX-A administration was safe: no botulism-like case was reported. The log of injected children who were not included in the study suggested that a large population could benefit from BTX-A management. CONCLUSIONS: We showed here the major input of BTX-A injections in the management of spasticity in CP children. The results are in favor of the use of BTX-A as conservative safe and efficient treatment of spasticity in children, which enables functional improvement as well as pain relief.

Compensation of fabry-perot surface defects. 2: silicon oxide compensating layers.

The properties of a Fabry-Perot etalon, with surface defects compensated by irradiating a silicon oxide film, are computed for the case in which the initial surface defects function is rectangular. As a result of absorption, the compensating layer of silicon oxide must be deposited before the reflecting coating if the etalon is to be used at uv wavelengths. For visible wavelengths, the compensating layer may be a half-wave silicon oxide film deposited over the reflecting coating. An example is discussed in which the rms deviation from the mean thickness of an etalon is reduced from 2.37 nm to 0.80 nm. An automatic self-compensating process is proposed in which the radiation transmitted by an etalon may be used to produce an appropriate change in the thickness profile of a silicon oxide film.

Optical anisotropy in thin films deposited obliquely: in situ observations and computer modeling.

Normal incidence optical anisotropies are monitored during the growth of dielectric and metal films deposited at 45 degrees by logging the quantities R( perpendicular),R( ||), T( perpendicular) and T( ||) as functions of mass thickness d. For both ZrO(2) and Ti(2)O(3), the experimental profile of (R( perpendicular) - R( ||)) vs d is consistent with a refractive anisotropy that remains nearly constant during deposition. Gold, silver, and aluminum (deposited in the absence of oxygen) are shown to exhibit characteristic anisotropy vs d profiles that can be associated with the refractive index resonance in a globular metal film. The anisotropic effects observed in metals, both during deposition and during subsequent argon-ion sputter etching, are modeled by considering the film to comprise a mixture of crystallite-and void-defined materials and by adopting simplified structural hysteresis loops.