Interrater reliability and convergent validity of the American Academy for Cerebral Palsy and Developmental Medicine methodology for conducting systematic reviews.

AIM: The aim of this study was to evaluate the interrater reliability and convergent validity of the American Academy for Cerebral Palsy and Developmental Medicine's (AACPDM) methodology for conducting systematic reviews (group design studies). METHOD: Four clinicians independently rated 24 articles for the level of evidence and conduct using AACPDM methodology. Study conduct was also assessed using the Effective Public Health Practice Project scale. Raters were randomly assigned to one of two pairs to resolve discrepancies. The level of agreement between individual raters and pairs was calculated using kappa (α=0.05) and intraclass correlations (ICCs; α=0.05). Spearman's rank correlation coefficient was calculated to evaluate the relationship between raters' categorization of quality categories using the two tools. RESULTS: There was acceptable agreement between raters (κ=0.77; p<0.001; ICC=0.90) and between assigned pairs (κ=0.83; p<0.001; ICC=0.96) for the level of evidence ratings. There was acceptable agreement between pairs for four of the seven conduct questions (κ=0.53-0.87). ICCs (all raters) for conduct category ratings (weak, moderate, and strong) also indicated good agreement (ICC=0.76). Spearman's rho indicated a significant positive correlation for the overall quality category comparisons of the two tools (0.52; p<0.001). CONCLUSIONS: The AACPDM rating system has acceptable interrater reliability. Evaluation of its study quality ratings demonstrated reasonable agreement when compared with a similar tool.

Effects of bisphosphonates in children with osteogenesis imperfecta: an AACPDM systematic review.

This systematic review of the effects of bisphosphonate treatment in children with osteogenesis imperfecta was conducted using the American Academy for Cerebral Palsy and Developmental Medicine methodology for developing systematic reviews of treatment interventions (Revision 1.1) 2004. Despite a large body of published literature, there have been only eight studies with a sufficiently high level of internal validity to be truly informative. These studies confirm improvement in bone density. Many, but not all studies, demonstrate reduction in fracture rate and enhanced growth. There has been extremely limited evaluation of broader treatment impacts such as deformity, need for orthopedic surgery, pain, functioning, or quality of life. Short-term side effects were minimal. Which medication and dosing regimen is optimal and how long patients should be treated are unclear. This body of evidence would be strengthened by a larger controlled trial, because many studies lacked adequate power to evaluate stated outcomes. These studies do not address the impacts of bisphosphonates in children with milder forms of osteogenesis imperfecta and severe forms that are not due to mutations in the type I pro-collagen gene (e.g. types VII and VIII). Additional research is needed into treatment of infants. More studies evaluating medication choices, optimal dosing, duration of treatment, post-treatment impacts, and long-term side effects are necessary.

Determination of body composition in children with cerebral palsy: bioelectrical impedance analysis and anthropometry vs dual-energy x-ray absorptiometry.

The object of this study was to determine whether bioelectrical impedance analysis (BIA) and anthropometry can be used to determine body composition for clinical and research purposes in children with cerebral palsy. Eight individuals with cerebral palsy (two female, mean age=10 years, mean gross motor function classification=4.6 [severe motor impairment]) recruited from an outpatient tertiary care setting underwent measurement of fat mass, fat-free mass, and percentage body fat using BIA, anthropometry (two and four skinfold equations), and dual-energy x-ray absorptiometry. Correlation coefficients were calculated for fat mass, fat-free mass, and percent body fat for these measures as determined by BIA and anthropometry when compared with dual-energy x-ray absorptiometry. Correlation coefficients were excellent for determination of fat-free mass for all methods (ie, all were above 0.9). Correlations were moderate for determination of fat mass and percent body fat (range=0.4 to 0.8).

Gynecological care for adolescents with disability: physician comfort, perceived barriers, and potential solutions.

OBJECTIVE: The goal of this study was to assess the barriers to gynecologic care for adolescents with disability in this state. DESIGN: Providers of gynecologic care in Utah were surveyed to assess existing conditions, attitudes, and comfort levels regarding this population. RESULTS: The survey was completed by 136 gynecologic clinicians (50% return rate). The comfort level among surveyed clinicians in providing gynecological care to disabled adolescents was relatively low, even for routine screening. Respondents identified the following as barriers to providing this care: time, reimbursement, inadequate knowledge, and disability-related issues (office access and preference to not serve this population). Suggested solutions included: continuing education programs, consultation to practices on serving both youth and individuals with disability, and better billing mechanisms. CONCLUSION: While many of the surveyed clinicians are comfortable providing care to adolescents with disability, a substantial number are not. Respondents perceive many barriers to providing care to this population, though the barriers appear modifiable. Potential interventions were identified which should be tested for their effectiveness in improving access to quality care for this underserved population.

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.

The contribution of tone to resting energy expenditure in children with moderate to severe cerebral palsy: Evaluation utilizing intrathecal baclofen injection.

The extent to which high muscle tone contributes to the caloric needs of children with cerebral palsy (CP) is debated. Understanding its contribution is important in predicting calorie needs in this population which frequently experiences under nutrition. Intrathecal baclofen is an innovative therapy now used for treatment of excessive tone. We prospectively studied the changes in resting energy expenditure (REE) observed in 12 children with CP undergoing intrathecal baclofen injection. REE was measured in the fasting state before intrathecal injection of 50 micrograms of baclofen. Patients remained fasting and REE measures were repeated four hours later. Tone changes were assessed using the Modified Ashworth Scale at the same time points. One child was excluded from study because he became increasingly agitated over the observation period and one child demonstrated no tone reduction after the baclofen injection. In the remaining 10 patients, the average REE prior to injection was 101 ± 17% of predicted and decreased to 84 ± 24% of predicted after injection (p<0.004). This study suggests that tone contributes substantially to the caloric needs of children with CP and must be carefully considered when providing nutritional recommendations.

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.

Serum prealbumin and albumin concentrations do not reflect nutritional state in children with cerebral palsy.

Serum prealbumin concentration (PALB) and albumin concentration (ALB) were evaluated as markers of undernutrition in 107 children with cerebral palsy (CP) age 2 to 18 years. PALB and ALB were rarely below the normal reference ranges and showed little to no correlation with anthropometric measures (eg, skinfolds, midarm fat area), growth (height), severity of CP (eg, feeding dysfunction, motor impairment), or general health.

Relationship of nutritional status to health and societal participation in children with cerebral palsy.

OBJECTIVES: To describe nutritional status in a population-based sample of children with moderate or severe cerebral palsy (CP) and to explore the relationships between nutritional status and health and functional outcomes. STUDY DESIGN: A population-based strategy was used to enroll children with CP at 6 geographic sites. Research assistants performed anthropometric assessment, determined severity of motor impairment, and interviewed caregivers with the Child Health Questionnaire and a questionnaire designed specifically for this study. Anthropometric measures were converted to Z scores and the relationship between health and nutritional status was assessed using regression models. RESULTS: Among the 235 participants, indicators of malnutrition were common. Poor nutritional status correlated with increased health care utilization (hospitalizations, doctor visits) and decreased participation in usual activities by the child and parent. CONCLUSIONS: Malnutrition is common in children with moderate or severe CP and associated with poorer health status and limitations in societal participation. Further studies are needed to determine the nature of these associations and how to manage nutrition in children with CP to optimize growth and health outcomes.

Secondary sexual characteristics in children with cerebral palsy and moderate to severe motor impairment: a cross-sectional survey.

OBJECTIVES: To compare the development of secondary sexual characteristics in children with cerebral palsy (CP) of moderate to severe motor impairment to children in the general population and to relate their sexual maturation to a measure of their body fat. METHODS: A multicenter, cross-sectional survey of 207 children who were 3 to 18 years of age and had CP of moderate to severe motor impairment (Gross Motor Functional Classification System [GMFCS] levels 3, 4, and 5) was conducted at 6 geographic sites; attempts were made to identify all eligible children through multiple methods and enroll them in the study. Trained research assistants performed anthropometric measurements, including subscapular skinfold thickness, determined GMFCS level, and assessed sexual maturation by Tanner stage. Secondary sexual characteristics were compared with the general population of children using cross-sectional surveys of the American Academy of Pediatrics Pediatric Research in Office Settings network and of the National Center for Health Statistics National Health and Nutrition Examination Survey (NHANES) III. Girls were classified as having begun puberty when they were at Tanner stage 2 or greater for pubic hair and breast development and to have completed puberty when they were at Tanner stage 4 or greater for pubic hair and breast development. Boys were classified as having begun puberty when they were at Tanner stage 2 or greater for pubic hair and genital development and to have completed puberty when they were at Tanner stage 4 or greater for pubic hair and genital development. RESULTS: The mean age (standard deviation) of subjects was 9.6 (4.6) years. Of the 207 subjects, 71% were white, 21% were black, and 8% were of other races; 59% were boys, and 41% were girls. Girls with CP (n = 84) entered puberty earlier than did boys with CP (n = 123). In contrast, girls with CP tended to complete puberty later than did boys with CP. Black boys and girls with CP (n = 43) entered puberty earlier than did white boys and girls with CP (n = 147). No difference between races was found in completion of puberty. Only for white children with CP were there a sufficient number of subjects for comparisons of sexual maturation to race-matched children in the general population, using data from the American Academy of Pediatrics Pediatric Research in Office Settings network and the NHANES III study. White girls with CP initiated pubic hair development (Tanner stage 2 or greater) earlier than in the general population, but the age of onset of breast development was similar to the general population, although the age distribution was different. A greater proportion of white girls with CP had early onset of breast development (Tanner stage 2 or greater), and a greater proportion had delayed onset of breast development than in the general population. White girls with CP completed breast development later than in the general population but not pubic hair development. For white boys ages 8 to 18 years with CP (n = 75), pubic hair and genital development both began earlier than in the general population, but genital development was completed later. The estimated median age of menarche for white girls with CP was 14.0 years, which was 1.3 years later (95% confidence interval: 0.7-2.3) than for the general population (estimated median age: 12.8 years; NHANES III). Relationships between sexual maturation and nutritional state, as assessed by subscapular skinfold thickness z score, were determined separately in white boys and in white girls with CP, between the ages of 8 and 18 years. For white girls with CP, more advanced sexual maturation was associated with more body fat, adjusting for age and GMFCS level (Spearman partial correlation: 0.41). In contrast, for white boys with CP, the opposite relationship pertained: more advanced sexual maturation was associated with less body fat (-0.29). CONCLUSIONS: The pattern of sexual maturation in children with CP of moderate to severe motor impairment differs from that of children in the general population. Puberty begins earlier but ends later in white children with CP, compared with white children in the general population. In addition, menarche occurs later in white girls with CP. More advanced sexual maturation was associated with more body fat in girls but less body fat in boys.