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Movakic is a newly developed instrument for measurement of motor abilities in children with severe multiple disabilities, with a satisfactory feasibility and content validity and good inter-observer and test-retest reliability. The objective of this study was to investigate its construct validity and responsiveness to change. Sixty children with severe multiple disabilities (mean age 7.7 years, range 2-16) were measured using Movakic six times during 18 months. Construct validity was assessed by correlating Movakic scores with expert judgment. In order to assess responsiveness, scores during 3-months intervals were compared (mean score-changes and intraclass correlations) during which some children experienced meaningful events influencing motor abilities and during which others experienced no such event. Forty-five percent of children had a lower cognitive development level than 6-month, 52% had Gross Motor Function Classification System level V and 37% had level IV. For 27 children all measurements were completed, six children dropped out. Construct validity was good (r=0.50-0.71). Responsiveness was demonstrated by significantly larger score changes after events than when such events did not occur. Movakic is a valid instrument for measuring motor abilities in children with severe multiple disabilities. Results suggest responsiveness to change in motor abilities after meaningful events., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Based on a systematic review, psychometric characteristics of currently available instruments on motor abilities of children with disabilities were evaluated, with the aim to identify candidates for use in children with severe multiple (intellectual and motor) disabilities. In addition, motor abilities are essential for independent functioning, but are severely compromised in these children. The methodological quality of all studies was evaluated with the Consensus Based Standards for the Selection of Health Status Measurement Instruments (COSMIN) Checklist; overall levels of evidence per instrument were based on the Cochrane Back Review Group strategy. As a result, 18 studies with a total of eight instruments, developed for children with cerebral palsy (CLA, GMFM-88 and LE85), spinal muscular atrophy (MHFMS), neuromuscular diseases (MFM), disabilities 0-6 years (VAB, WeeFIM), and one developed specifically for children with severe multiple disabilities (TDMMT) were found. Strong levels of evidence were found for construct validity of LE85 and MFM and for responsiveness of WeeFIM, but reliability studies of these instruments had a limited methodological quality. Up to now studies of the TDMMT resulted in limited and unknown evidence for structural validity due to the poor methodological quality of reliability studies. In a next step, the clinical suitability of the instruments for children with severe multiple disabilities will be evaluate., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Background: Accurate prediction equations for estimating body composition and total energy expenditure (TEE) in children with severe neurologic impairment and intellectual disability are currently lacking., Objective: The objective was to develop group-specific equations to predict body composition by using skinfold-thickness measurements and bioelectrical impedance analysis (BIA) and to predict TEE by using data on mobility, epilepsy, and muscle tone., Design: Measures of body composition with the use of skinfold-thickness measurements (percentage of body fat) and BIA (total body water) were compared with those from isotope dilution (reference method) by using intraclass correlation coefficients (ICCs) and Bland and Altman limits of agreement analyses. With the use of the same methods, the outcomes of cerebral palsy-specific TEE equations were compared with those of the doubly labeled water method (reference method). Group-specific regression equations were developed by using forward-stepwise-multiple-correlation-regression analyses., Results: Sixty-one children with a mean (±SD) age of 10.1 ± 4.3 y (32 boys) were studied. A new equation based on the sum of 4 skinfold-thickness measurements did not improve agreement (n = 49; ICC = 0.61), whereas the newly developed BIA equation-which includes tibia length as an alternative for standing height-did improve agreement (n = 61; ICC = 0.96, SEE = 1.7 kg, R(2) = 0.92). The newly developed TEE equation, which uses body composition, performed better (n = 52; ICC = 0.87, SEE = 180 kcal, R(2) = 0.77) than did the equation of Schofield (n = 52; ICC = 0.82, SEE = 207 kcal, R(2) = 0.69)., Conclusions: Current cerebral palsy-specific equations for measuring body composition and energy expenditure are inaccurate. BIA is more accurate at assessing nutritional status in this population than is the measurement of skinfold thickness. The newly developed TEE equation, which uses body composition, provides a reasonable estimate of energy expenditure in these children despite its variability.

Background & Aims: The majority of children with cerebral palsy and intellectual disability has a poor nutritional state compared with their healthy peers. Several studies have found reduced daily energy intake in this population. The hypothesis is tested that low daily energy intake correlates with poor nutritional state., Methods: In a population-based sample of 176 children with severe generalized cerebral palsy and intellectual disability (mean age 10 years, SD 2 months; 16% GMFCS score 4; 84% GMFCS score 5) anthropometric parameters (weight, upper arm and tibia length, biceps, triceps, subscapular and suprailiacal skinfold thickness, mid upper arm circumference) were measured and dietary intake was registered., Results: No correlation was found between energy intake(%EAR) and anthropometric Z-scores. Higher age, female gender, mobility, and to a lesser extent the absence of tube feeding predicted lower anthropometric Z-scores., Conclusions: In children with severe generalized cerebral palsy and intellectual disability nutritional state is not primarily determined by energy intake. Differences in energy expenditure presumably play an important role, although more research is needed to clarify the complex association between energy intake and nutritional state. Individualized nutritional care is suggested, preferably based on energy expenditure, in order to avoid malnutrition, but also overweight., (Copyright © 2010 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Background & Aims: Accurately measuring nutritional status in children with severe cerebral palsy (CP) is a challenge. This review seeks to assess the validity of skinfold measurements and bioelectrical impedance analysis (BIA) for measuring body composition in children with severe CP., Methods: We executed a literature search on the validation of both methods in children with severe CP. To be eligible for inclusion, a study had to report on a statistical comparison between these two methods and any method of reference. The QUADAS tool was used for quality assessment., Results: The search strategy resulted in 1549 studies of which 5 studies eventually met the inclusion criteria. When comparing body composition outcomes of skinfold measurements to a reference method, correlation coefficients were found ranging from 0.406 to 0.988. Correlation coefficients between body composition data of BIA and a reference method ranged from 0.515 to 0.95., Conclusions: Although a number of authors found favorable agreement between skinfold measurements and BIA in comparison with reference methods, the small numbers studied, the lack of methodological quality measured by QUADAS, and the use of inappropriate analytical methods hamper solid conclusions., (Copyright 2009 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Aim: Children with severe cerebral palsy (CP) are at risk for developing low bone mineral density (BMD) and low-impact fractures. The aim of this study was to provide a systematic literature review of the epidemiology of fractures and low BMD in children with severe CP, with an emphasis on risk factors. Gross Motor Function Classification System (GMFCS) levels IV and V were criteria for severe cerebral palsy., Method: The literature (PubMed) was searched and eligible studies were given a level of evidence score using the Scottish Intercollegiate Guidelines Network criteria., Results: Seven studies were found concerning epidemiology of fractures, 11 studies described epidemiology of low BMD, and 14 studies concerned risk factors. The methodological quality of most of these studies was poor. Five studies were considered well-conducted with low risk of confounding and bias. In these studies, the incidence of fractures in children with moderate to severe CP approached 4% per year, whereas the prevalence of low BMD in the femur was 77%. Limited ambulation, feeding difficulties, previous fractures, anticonvulsant use, and lower body fat mass were associated with low BMD z-scores., Interpretation: There is only a limited amount of high-quality evidence on low BMD and fractures in children with severe CP.

This study assessed the clinical indicators and severity of dysphagia in a representative sample of children with severe generalized cerebral palsy and intellectual disability. A total of 166 children (85 males, 81 females) with Gross Motor Function Classification System Level IV or V and IQ<55 were recruited from 54 daycare centres. Mean age was 9 years 4 months (range 2 y 1 mo-19 y 1 mo). Clinically apparent presence and severity of dysphagia were assessed with a standardized mealtime observation, the Dysphagia Disorders Survey (DDS), and a dysphagia severity scale. Additional measures were parental report on feeding problems and mealtime duration. Of all 166 participating children, 1% had no dysphagia, 8% mild dysphagia, 76% moderate to severe dysphagia, and 15% profound dysphagia (receiving nil by mouth), resulting in a prevalence of dysphagia of 99%. Dysphagia was positively related to severity of motor impairment, and, surprisingly, to a higher weight for height. Low frequency of parent-reported feeding problems indicated that actual severity of dysphagia tended to be underestimated by parents. Proactive identification of dysphagia is warranted in this population, and feasible using a structured mealtime observation. Children with problems in the pharyngeal and esophageal phases, apparent on the DDS, should be referred for appropriate clinical evaluation of swallowing function.

During the analysis of interrupter resistance (R(int))-measurements, most authors reject post-interruption tracings based on the shape of the pressure-time and flow-time curves. However, objective criteria for rejection are lacking. We aimed to formulate explicit rejection criteria that correspond to eyeballing the curve pattern (daily practice), in order to simplify the analysis. Inter-observer agreement within and between both methods was studied. Results obtained with the developed rejection criteria were compared to those of current practice (eyeballing) using 54 measurements (807 interruptions) of children with severe neurological impairment. Inter-observer agreement on rejection was similar using the criteria or eyeballing (85.6% vs. 82.8%). Using the criteria, more individual interruptions were rejected (43.4% vs. 29.8% using eyeballing), while discarding total measurements (<5 remaining interruptions) was similar (9.2% vs. 7.4% using eyeballing). Results using only the criteria for pressure-time curves were comparable to eyeballing. Outcome values were comparable between any of the used rejection methods and not rejecting at all. In this first detailed study on rejection of post-interruption tracings, explicit rejection criteria were developed. None of the rejection methods influenced the outcome value relevantly. However, rejection criteria can contribute to the standardization of the R(int) technique and simplify decision-making in daily practice., ((c) 2006 Wiley-Liss, Inc.)

Objective: The need is strong for an accurate and easy-to-perform test to evaluate the nutritional state of children who have a severe generalized cerebral palsy, defined as a severe motor handicap and an intellectual disability. For that purpose, we determined the feasibility of bioelectrical impedance analysis (BIA) in these children and evaluated their nutritional state., Methods: BIA recordings were done in 35 children who had a severe generalized cerebral palsy using a single-frequency BIA device. In addition, arm span and body weight were determined. Components of feasibility were whether the children tolerated the recording and felt comfortable and whether the recording could be performed in a reproducible way (prescribed body position and stable resistance and reactance values). All recordings were performed at specialized children's daycare centers or schools., Results: One child (3%) did not tolerate the recording, whereas the remaining 34 children (71%) felt comfortable. Most children (74%) could be placed in the prescribed position, but stability of resistance values was low. Stability of resistance values was positively influenced by older age, a quiet location for the recording, feeling comfortable, and a small number of people in the room. For 29 children, we were able to calculate values for total body water and fat-free mass. Compared with age-matched reference values, these values were significantly decreased in all age groups., Conclusions: The present pilot study has demonstrated that BIA recording is a feasible nutritional assessment method in children who have severe generalized cerebral palsy. Because the test procedure was well tolerated by most children, its value for use in this specific population deserves further investigation.

Background: In children with severe generalized cerebral palsy, pneumonias are a major health issue. Malnutrition, dysphagia, gastro-oesophageal reflux, impaired respiratory function and constipation are hypothesized risk factors. Still, no data are available on the relative contribution of these possible risk factors in the described population. This paper describes the initiation of a study in 194 children with severe generalized cerebral palsy, on the prevalence and on the impact of these hypothesized risk factors of recurrent pneumonias., Methods/design: A nested case-control design with 18 months follow-up was chosen. Dysphagia, respiratory function and constipation will be assessed at baseline, malnutrition and gastro-oesophageal reflux at the end of the follow-up. The study population consists of a representative population sample of children with severe generalized cerebral palsy. Inclusion was done through care-centres in a predefined geographical area and not through hospitals. All measurements will be done on-site which sets high demands on all measurements. If these demands were not met in "gold standard" methods, other methods were chosen. Although the inclusion period was prolonged, the desired sample size of 300 children was not met. With a consent rate of 33%, nearly 10% of all eligible children in The Netherlands are included (n = 194). The study population is subtly different from the non-participants with regard to severity of dysphagia and prevalence rates of pneumonias and gastro-oesophageal reflux., Discussion: Ethical issues complicated the study design. Assessment of malnutrition and gastro-oesophageal reflux at baseline was considered unethical, since these conditions can be easily treated. Therefore, we postponed these diagnostics until the end of the follow-up. In order to include a representative sample, all eligible children in a predefined geographical area had to be contacted. To increase the consent rate, on-site measurements are of first choice, but timely inclusion is jeopardized. The initiation of this first study among children with severe neurological impairment led to specific, unexpected problems. Despite small differences between participants and non-participating children, our sample is as representative as can be expected from any population-based study and will provide important, new information to bring us further towards effective interventions to prevent pneumonias in this population.