Richard C. Henderson

Feeding Dysfunction is Associated with Poor Growth and Health Status in Children with Cerebral Palsy

OBJECTIVE To describe parent-reported feeding dysfunction and its association with health and nutritional status in children with cerebral palsy. DESIGN Anthropometry was measured and z scores calculated. The Child Health Questionnaire was used to assess health status, and a categorical scale (none to severe) was used to classify subjects according to severity of feeding dysfunction. SUBJECTS 230 children (9.7+/-4.6 years; 59% boys) with moderate to severe cerebral palsy were recruited from 6 centers in the United States and Canada. STATISTICAL ANALYSES Descriptive statistics, the Kruskal-Wallis and Pearson chi2 tests. RESULTS Severity of feeding dysfunction was strongly associated with indicators of poor health and nutritional status. The mean weight z scores were -1.7, -2.5, -3.3, and -1.8 among children with none, mild, moderate, or severe (largely tube-fed) feeding dysfunction, respectively (P= .003). Similar results were observed for height z score (P=.008), triceps z score (P=.03), and poor Global Health score (part of the Child Health Questionnaire) (P<.001). Subjects who were tube fed were taller (P=.014) and had greater body fat stores (triceps z score, P=.001) than orally fed subjects with similar motor impairment. For subjects exclusively fed by mouth, a dose-response relationship was observed between feeding dysfunction severity and poor nutritional status. Subjects with only mild feeding dysfunction had reduced triceps z score (-0.9) compared with those with no feeding problems (-0.3). CONCLUSION For children with moderate to severe cerebral palsy, feeding dysfunction is a common problem associated with poor health and nutritional status. Even children with only mild feeding dysfunction, requiring chopped or mashed foods, may be at risk for poor nutritional status. Parental report of feeding dysfunction with a structured questionnaire may be useful in screening children for nutritional risk.

Bone mineral density and fractures in boys with Duchenne muscular dystrophy

The relationships between bone density, mobility, and fractures were assessed in 41 boys with Duchenne muscular dystrophy. Bone density in the lumbar spine was only slightly decreased while the boys were ambulatory (mean z-score, -0.8), but significantly decreased with loss of ambulation (mean z-score, -1.7). In contrast, bone density in the proximal femur was profoundly diminished even when gait was minimally affected (mean z-score, -1.6), and then progressively decreased to nearly 4 standard deviations below age-matched normals (mean z-score, -3.9). These are consistent with the findings that 18 (44%) of the boys sustained a fracture, 66% of these fractures involved the lower extremities, and there were no spinal compression fractures. Furthermore, four (44%) of nine boys who were walking with aids or support at the time of fracture never resumed walking after the fracture. Osteoporosis is most profound in the lower extremities of boys with Duchenne muscular dystrophy, and begins to develop early while still ambulating. Frequent fractures that may result in loss of ambulation are the clinical consequences.

Bone-mineral density in children and adolescents who have spastic cerebral palsy

Bone-mineral density was studied in a heterogeneous group of 139 children (mean age, nine years; range, three to fifteen years) who had spastic cerebral palsy. The evaluation included serum analyses and a nutritional assessment based on a dietary history and anthropometric measurements. The bone-mineral density of the proximal parts of the femora and the lumbar spine was measured with dual-energy x-ray absorptiometry and was normalized for age against a series of ninety-five normal children and adolescents who served as controls. Bone-mineral density varied greatly but averaged nearly one standard deviation below the age-matched normal means for both the proximal parts of the femora (-0.92 standard deviation) and the lumbar spine (-0.80 standard deviation). Ambulatory status was the factor that best correlated with bone-mineral density. Nutritional status, assessed on the basis of caloric intake, skinfolds, and body-mass index, was the second most significant variable. The pattern of involvement, durations of immobilization in a cast, and a calcium intake of less than 500 milligrams per day were additional factors of less significance. The age when the child first walked, previous fractures, use of anticonvulsants, and serum vitamin-D levels did not correlate with bone-mineral density after adjustment for covariance with the ambulatory status and the nutritional status. Serum levels of calcium, phosphate, alkaline phosphatase, and osteocalcin were not reliable indicators of low bone-mineral density.

Health status of children with moderate to severe cerebral palsy

The aim of the study was to evaluate the health of children with cerebral palsy (CP) using a global assessment of quality of life, condition-specific measures, and assessments of health care use. A multicenter population-based cross-sectional survey of 235 children, aged 2 to 18 years, with moderate to severe impairment, was carried out using Gross Motor Function Classification System (GMFCS) levels III (n = 56), IV (n = 55), and V (n = 122). This study group scored significantly below the mean on the Child Health Questionnaire (CHQ) for Pain, General Health, Physical Functioning, and Impact on Parents. These children used more medications than children without CP from a national sample. Fifty-nine children used feeding tubes. Children in GMFCS level V who used a feeding tube had the lowest estimate of mental age, required the most health care resources, used the most medications, had the most respiratory problems, and had the lowest Global Health scores. Children with the most severe motor disability who have feeding tubes are an especially frail group who require numerous health-related resources and treatments. Also, there is a relationship among measures of health status such as the CHQ, functional abilities, use of resources, and mental age, but each appears to measure different aspects of health and well-being and should be used in combination to reflect childrens overall health status.

Promotion of Physical Fitness and Prevention of Secondary Conditions for Children With Cerebral Palsy: Section on Pediatrics Research Summit Proceedings

Inadequate physical fitness is a major problem affecting the function and health of children with cerebral palsy (CP). Lack of optimal physical activity may contribute to the development of secondary conditions associated with CP such as chronic pain, fatigue, and osteoporosis. The purpose of this article is to highlight the content and recommendations of a Pediatrics Research Summit developed to foster collaborative research in this area. Two components of physical fitness—muscle strength and cardiorespiratory fitness—were emphasized. Although there is evidence to support the use of physical fitness interventions, there are many gaps in our current knowledge. Additional research of higher quality and rigor is needed in order to make definitive recommendations regarding the mode, intensity, frequency, and duration of exercise. Outcome measurements have focused on the body functions and structures level of the International Classification of Functioning, Disability and Health (ICF), and much less is known about effects at the activities and participation levels. Additionally, the influence of nutritional and growth factors on physical fitness has not been studied in this population, in which poor growth and skeletal fragility have been identified as serious health issues. Current intervention protocols and outcome measurements were critically evaluated, and recommendations were made for future research.

Fracture prediction and the definition of osteoporosis in children and adolescents: the ISCD 2007 Pediatric Official Positions.

Osteoporosis in adults has been defined on the basis of densitometric criteria, but at present the term osteoporosis does not have a widely recognized definition in pediatrics. Consequently, the International Society for Clinical Densitometry (ISCD) 2007 Position Development Conference reviewed the literature describing the relationship between bone densitometric studies and fractures in apparently healthy children and adolescents, and prepared Official Positions regarding the definition of osteoporosis in children and adolescents. The ISCD Official Positions with respect to the above issues, as well as the rationale and evidence used to derive these positions, are presented here.

Growth and Health in Children With Moderate-to-Severe Cerebral Palsy

BACKGROUND. Children with cerebral palsy frequently grow poorly. The purpose of this study was to describe observed growth patterns and their relationship to health and social participation in a representative sample of children with moderate-severe cerebral palsy. METHODS. In a 6-site, multicentered, region-based cross-sectional study, multiple sources were used to identify children with moderate or severe cerebral palsy. There were 273 children enrolled, 58% male, 71% white, with Gross Motor Function Classification System levels III (22%), IV (25%), or V (53%). Anthropometric measures included: weight, knee height, upper arm length, midupper arm muscle area, triceps skinfold, and subscapular skinfold. Intraobserver and interobserver reliability was established. Health care use (days in bed, days in hospital, and visits to doctor or emergency department) and social participation (days missed of school or of usual activities for child and family) over the preceding 4 weeks were measured by questionnaire. Growth curves were developed and z scores calculated for each of the 6 measures. Cluster analysis methodology was then used to create 3 distinct groups of subjects based on average z scores across the 6 measures chosen to provide an overview of growth. RESULTS. Gender-specific growth curves with 10th, 25th, 50th, 75th, and 90th percentiles for each of the 6 measurements were created. Cluster analyses identified 3 clusters of subjects based on their average z scores for these measures. The subjects with the best growth had fewest days of health care use and fewest days of social participation missed, and the subjects with the worst growth had the most days of health care use and most days of participation missed. CONCLUSIONS. Growth patterns in children with cerebral palsy were associated with their overall health and social participation. The role of these cerebral palsy-specific growth curves in clinical decision-making will require further study.

Bone density in children and adolescents with cystic fibrosis

OBJECTIVE To assess bone mineralization in children and adolescents with cystic fibrosis. DESIGN A cross-sectional, observational study of bone mineral density (BMD) in the lumbar vertebrae and the proximal femur of 62 patients aged 4.9 to 17.8 years (mean, 10.7 years). The age-normalized BMD findings (z scores) were correlated with multiple variables, including measures of pulmonary disease, nutritional status and growth, genotype, calcium intake, and serum 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D levels. RESULTS The mean BMD z score was -1.03 +/- 0.14 (+/- SE) in the lumbar vertebrae and -0.71 +/- 0.17 in the proximal femur. The BMD in this age range declined relative to normal values at a rapid rate of roughly 1 SD every 6 to 8 years. The BMD z scores correlated well with multiple measures of disease severity, particularly weight and forced expiratory volume in 1 second. CONCLUSIONS Significant osteoporosis in adults with CF results at least in part from a failure to accumulate bone mineral at a normal rate during skeletal growth and development. The cause of this is likely multifactorial. With increasing longevity, the skeletal consequences of CF become an important consideration.

Bone density and other possible predictors of fracture risk in children and adolescents with spastic quadriplegia.

Forty‐three patients with spastic quadriplegia (mean age 7.9 years, range 3.3 to 17.2 years) underwent bone mineral density (BMD) measurement of the lumbar spine and were evaluated between 2.6 and 5.5 years (mean 3.8) later to determine whether this measurement had predicted risk of fracture over the subsequent period of observation. Other potential risk factors that were evaluated include body weight z score, serum vitamin D levels, previous fracture, and hip spica casting. The baseline measurements showed that BUD falls further below normal with increasing age and was more than one standard deviation below age‐matched normal mean in 38 of the 43 patients. Fracture rate did not differ between those with low and those with very low spinal BMD. Similarly, serum vitamin D levels and body weight z scores were not predictive of fracture. However, fracture rate was over fourfold greater following spica casting and more than threefold greater following an initial fracture. Fracture rates in the study group were similar to those reported for age‐ and sex‐matched normal children, though generally the location of the fractures and mechanisms of injury differed.

Kyphosis and fractures in children and young adults with cystic fibrosis

The purpose of this study was to examine children and adolescents with cystic fibrosis for an increased frequency of fracture and excessive thoracic kyphosis, which may result from inadequate skeletal mineralization. In a survey of 143 patients (ages 4.7 to 21.9 years; mean, 11.3 years), the fracture rate for male patients from birth to 5 years of age was higher than for female patients and both rates were comparable with those for normal children. In contrast, female patients 6 to 16 years of age with cystic fibrosis had a higher-than-normal fracture rate and a higher rate than their male counterparts. Review of the chest radiographs showed that thoracic kyphosis correlated with age and with disease severity as judged by Brasfield scoring. In the > 15-year-old age group, kyphosis exceeding 40 degrees, the upper limit of normal, was found in 77% of the female patients and 36% of the male patients. The cause of these findings is uncertain and perhaps multifactorial, but osteopenia is likely a contributing factor. As the life expectancy of patients with cystic fibrosis continues to increase, the skeletal consequences, particularly in female patients, may become increasingly significant.