bluuebunny/crossref_metadata_2025 huggingface dataset:
Measurement Integrity of the Family Needs Survey
Authors: SEXTON DAVID , BURRELL BRENDA , THOMPSON BRUCE
Published: December 2008

The present study investigated the measurement integrity of data collected using the Family Needs Survey (FNS). The data provided by 53 mothers of children with disabilities were subject to both classical test theory analyses and to factor analysis. The areas that the mothers considered “definite” needs were also compared with similar analyses conducted in a previous study. Results suggest that the FNS yields reasonably valid data regarding family needs.
Predicting Effective Intervention
Authors: JONES MOLLY M. , JACKSON KIRBY L.
Published: December 2008

Multiple logistic analysis, a research methodology being applied with increasing frequency in epidemiology, offers advantages that would be equally useful in early Intervention research Logistic regression provides the capability of assessing the degree of association of multiple factors such as subject or situational characteristics, with a dichotomous outcome such as benefiting or not benefiting from an intervention. The method results in an estimate of the probability of each outcome. We describe the methodology and its advantages and disadvantages.
Children Exposed to Cocaine
Authors: WILLIAMS BETTY FRY , HOWARD VIKKI F.
Published: December 2008

The 1990 President's National Drug Controi Strategy Report estimated that 100,000 babies who have been prenatally exposed to cocaine are born each year. Medical research has established risks for both cocaine-using pregnant women and their unborn children. Infants prenatally exposed to cocaine may experience a marked failure to adapt to the neonatal environment, a lack of appropriate interaction with caregivers, and language, cognitive and motor abnormalities that clearly place them at risk. Initial results of follow-up on these infants have suggested that some will suffer long-term educational and behavioral handicaps. Little has been empirically documented concerning the characteristics of children 18 months and older who were exposed to cocaine prenatally. There is an urgent need for study of this group to determine what, if any, long-term effects children prenatally exposed to cocaine may later exhibit. Such information could be vital to a wide range of professionals in health, education, social service, and childcare. Such research must proceed with caution, however, for other interacting explanations of any identified long-term effects must also be considered.
A Survey of Pediatricians on Early Identification and Early Intervention Services
Authors: SCOTT FRANCINE GORTLER , LINGARAJU SATHYAVATHI , KILGO JENNIFER L. , KREGEL JOHN , LAZZARI ANDREA
Published: December 2008

In order for Public Law 99-457 (P.L. 99-457) Part H to be effective, it is crucial that pediatricians be aware of and participate in community early intervention systems. Three hundred and forty-two pediatricians responded to a questionnaire regarding screening practices, referral methods, and training needs. Nearly all (97%) indicated that they routinely performed some type of developmental screening. Most respondents recommended more training in developmental/behavioral pediatrics in residency programs. Less than half of the pediatricians surveyed were familiar with all of the provisions of P.L. 99-457, even though 83% said they were aware of the legislation. The most frequently requested area of continuing education was developmental screening (66%), followed by requests for more information regarding community resources in early intervention (51%). Regional and local seminars were chosen as the type of continuing education most likely to be used by pediatricians. Continuing education is needed to prepare pediatricians for the new roles and skills needed to fully participate in the early intervention system.
Consumer Validation of an Index Characterizing the Functional Abilities of Young Children with Disabilities
Authors: BUYSSE VIRGINIA , SMITH TINA M. , BAILEY DONALD B. , SIMEONSSON RUNE J.
Published: December 2008

Current systems for characterizing infants and young children with disabilities have been associated with a number of problems. The ABILITIES Index was developed as a way to describe the functional abilities and limitations of children across nine domains. This article reports the results of a survey of parents and professionals in early intervention programs to determine their perceptions of the ABILITIES Index as a means of assessing its consumer validity. A total of 209 parents, 91 teachers, 93 therapists, and 55 Developmental Evaluation Center specialists in early intervention and preschool programs in North Carolina evaluated the ABILITIES Index. Mean satisfaction ratings across five dimensions were generally positive, with parents consistently rating the instrument more positively than other consumers. Subsequent analyses indicated that, overall, level of education did not predict positive appraisal of the ABILITIES Index. Consumers offered a number of suggestions for how the Index could be used in early intervention.
Home Literacy Experiences of Preschoolers Enrolled in Head Start and Special Education Programs
Authors: MARVIN CHRISTINE , MIRENDA PAT
Published: December 2008

A survey was constructed to gather information concerning the home literacy experiences of preschool children enrolled in Head Start and early childhood special education classrooms. A total of 291 surveys were analyzed, representing 95 children considered at risk, 168 children with special education needs, and 28 peer models who had no developmental delays or disabilities. The results indicated a number of differences across the three groups regarding the priority placed on literacy at home, the ways in which children were involved in reading and writing activities, the amount of progress they were thought to have made in the past year, and the future expectations of the respondents in this area. Overall, the respondents for children with special education needs appeared to place the lowest priority on literacy development and have the lowest expectations in this regard. These respondents also reported providing fewer types of early literacy experiences to the children at home. Possible explanations are provided for the results. Implications for early childhood special education (ECSE) personnel are discussed.
Early Childhood Programs in the Public Schools
Authors: BARNETT W. STEVEN , FREDE ELLEN C.
Published: December 2008

Local education agencies (LEAs) have considerable discretion in the development and implementation of early childhood policy and programs. It is therefore useful to describe local implementation of early childhood policies, including those for young children with disabilities. A survey was conducted of early childhood programs administered by public schools in Massachusetts to obtain data on six aspects of program implementation: characteristics of the children served, program settings, service characteristics, teacher characteristics, family involvement activities, and funding and administration. Results revealed important variations among LEAs in integration, duration and frequency of services, staff-child ratios, teacher qualifications, and other aspects of policy implementation. Potential state responses are discussed.
Why Covariance? A Rationale for Using Analysis of Covariance Procedures in Randomized Studies
Authors: TAYLOR MATTHEW J. , INNOCENTI MARK S.
Published: December 2008

Analysis of covariance is a useful statistical procedure for data analysis even when carefully executed random assignment procedures are employed. Statistically adjusting for the effects of the covariates on the outcome can help account for pretreatment group differences—even with careful sampling. In addition, analysis of covariance can increase statistical power, thus reducing the Type II error rate. This paper uses diagrams to show how analysis of covariance manipulates data under a variety of conditions. Also discussed are the relationship between analysis of covariance and statistical power and issues related to covariate selection. Finally, a simulated data analysis is used to demonstrate the process of selecting covariates and to show the results with and without statistically adjusting for the effects of the covariate.
The Social Strategy Rating Scale
Authors: BECKMAN PAULA J. , LIEBER JOAN
Published: December 2008

In recent years, professionals in early childhood special education have been concerned with the social competence of young children with disabilities. Despite widespread interest in this topic, the availability of psychometrically sound, easy-to-use instruments to measure social competence has been limited. This study provides initial data concerning the psychometric properties of the Social Strategy Rating Scale. This scale was developed in order to provide a relatively simple method of measuring social competence in young children with disabilities across multiple contexts. Forty-seven toddlers with disabilities were observed at four data points over a 16-month period in three different social settings (with mothers, in dyads, and in a group setting). The scale was found to have relatively high interobserver agreement across items and high internal consistency. In addition, the scale seemed to detect differences that occurred over time and in varying contexts. Findings are discussed in terms of their application in early childhood intervention programs.
Parental Involvement in the Developmental Screening of Their Young Children
Authors: Henderson Laura W. , Meisels Samuel J.
Published: December 2008

Because multiple risk factors can influence child development, methods designed to screen young children for developmental problems should incorporate information from various sources in order to cover all potential areas of delay effectively. In this study we combined results from a standardized parent questionnaire with those of an individually administered developmental screening instrument (the Early Screening Inventory) in order to predict more accurately which children will be at risk for school failure. Our results show a decrease in misclassifications after combining the parent measure with the screening instrument, thus increasing the predictive accuracy of the developmental screening process. Implications of our findings are discussed within the context of screening from a perspective of multiple risks and multiple sources of input.
Using Growth Curve Analysis to Evaluate Child Change in Longitudinal Investigations
Authors: BURCHINAL MARGARET R. , BAILEY DONALD B. , SNYDER PATRICIA
Published: December 2008

This paper presents an overview of analytic methods for estimating growth curves or developmental trajectories from longitudinal data. Traditional models for studying change are contrasted with a relatively new method, hierarchical linear models. Growth curve analyses of data from an early intervention project demonstrate the strengths and weaknesses of the various analytic methods. Using this example, evidence is presented indicating how hierarchical linear models can overcome some of the methodological limitations of other analytic techniques currently used to evaluate change.
Small-Group Massed-Trial and Individually-Distributed-Trial Instruction With Preschoolers
Authors: CHIARA LORI , SCHUSTER JOHN W. , BELL JENNIFER K. , WOLERY MARK
Published: December 2008

This study compared the effectiveness and efficiency of using a small-group-massed-trial instructional format to an individually administered distributed-trial format in teaching preschool children with and without disabilities to name pictures. The trainer identified three pairs of pictures for each of 8 participants (37-59 months). Students learned the first pair in a small-group instructional arrangement with a massed-trial format and the second pair individually with a distributed-trial format. The third pair served as a no-instruction control set. Training included a constant time delay procedure with both formats, and an adapted alternating treatments design evaluated the effects. All children acquired the naming behaviors regardless of the instructional format; however, for 6 of the 8 children, distributed instruction was slightly more efficient. The procedures resulted in errorless learning for 3 children, and the embedded instruction resulted in fewer errors and lower error percentages for 3 of the 5 remaining children. These findings are discussed in terms of designing effective and efficient instructional programs in mainstreamed contexts.
Child Development Days
Authors: WRIGHT ARLENE , IRETON HAROLD
Published: December 2008

This article describes a comprehensive approach for supporting and educating parents of young children, age 2 1/2 to 3 1/2, and for identifying those children who need further evaluation for exceptional education needs. Professionals from education, public health, social services, and child care collaborate to provide parents with information about child development and community resources; have early childhood teachers observe children in a play setting; respond to parents' questions and concerns; and talk with parents about the teacher's observations of the child. Parents' concerns and teachers' observations are used to identify children for follow-up assessment for possible early childhood/special education services, or to refer parents to other services.
Preschoolers' Categorical and Sequential Toy Play: Change over Time
Authors: Malone D. Michael
Published: December 2008

Longitudinal change in the categorical and sequential toy play of 22 preschool children with cognitive delays was examined. Children were videotaped during home-based independent play sessions at Time 1 and approximately 13 months later at Time 2. No statistically significant Time 1 to Time 2 changes were observed for the proportion of time spent in various categorical play behaviors. Children's relative ranking on play sophistication remained stable over time. Statistically significant changes in the proportion of time spent in play sequences, the length of play sequences, single-scheme sequences, and unordered multischeme sequences were observed. Finally, the correspondence between Time 1-Time 2 difference scores on developmental age and behavioral measures provided modest support for the concept of progression on play competency.
Variables Affecting Engagement in Inclusive Preschool Classrooms
Authors: McCORMICK LINDA , NOONAN MARY JO , HECK RONALD
Published: December 2008

This study applies multilevel data analysis to explore variables associated with engagement in inclusive community-based preschool settings. Data were analyzed at 2 levels: within and between classes. Results indicated that most of the variance (89-95%) in children's engagement with peers, with adults, and with activities occurred at the within-class level. Disability status did not predict engagement, but activity characteristics were significantly related to engagement. Another variable, co-teacher team characteristics, was also found to be a significant predictor of engagement, but to a lesser extent. The findings are discussed in terms of their implications for inclusive preschool classrooms using the co-teaching model.
Patterns of Service Utilization
Authors: KOCHANEK THOMAS T. , BUKA STEPHEN L.
Published: December 2008

The purpose of this study was to examine relationships between service utilization patterns in early intervention programs and specific child, maternal, and service provider characteristics. Service utilization data for 133 infants and toddlers were gathered for 1 week out of every month for a 4 month duration. For each service encounter, the duration, location, type of service, and academic discipline of service provider was recorded. Findings revealed that families received an average of 1.7 hours per week of services (unduplicated hours). Older children (toddlers) and mothers with higher levels of education received significantly more service. Thirty-four percent of all services occurred in community-based settings and community-based services were utilized more when both mother and provider were college educated. Data also suggest that early intervention is no longer restricted to specialized centers but includes the universe of neighborhood resources.
Family Factors Associated with Externalizing Disorders in Preschoolers
Authors: STORMONT MELISSA
Published: December 2008

This review presents literature on family factors associated with externalizing behavior in preschool children. Studies that identify family patterns of preschoolers with both hyperactivity and aggression and longitudinal studies on family factors associated with negative behavioral outcomes in children were reviewed. Overall, the research documents that negative family characteristics are more prevalent in families of preschoolers with externalizing behavior disorders than in families of comparison preschoolers. When compared with families of preschoolers with only hyperactivity, preschoolers with hyperactivity and aggression had families with the most negative characteristics. Longitudinal research documented that certain family characteristics are associated with negative behavioral outcomes in children. Implications from these findings for both research and practice are given.
Evaluating the Efficacy of Case Method Instruction
Authors: SNYDER PATRICIA , McWILLIAM P. J.
Published: December 2008

Student outcomes were investigated in a preservice training course where the Case Method of Instruction (CMI) was the predominant instructional strategy. Sixty-seven graduate students taking an interdisciplinary families course completed preand post-test measures to evaluate their attitudes about family-centered early intervention and their ability to apply family-centered principles to realistic case situations. Students also completed a final examination composed of knowledge and application questions, and rated the course and instructor. Statistically significant and noteworthy changes in attitudes and application skills occurred. Final examination indicated students not only possessed important knowledge related to working with families, but they also could apply the problem-solving process used in CMI to a novel case. Course and instructor evaluations revealed high levels of satisfaction with the design and delivery of course content. Findings of this study support the premise that CMI is an effective instructional strategy and can be used successfully in early intervention training contexts.
Qualitative Analysis of Latino Parents' Religious Interpretations of Their Child's Disability
Authors: SKINNER DEBRA , RODRIGUEZ PATRICIA , BAILEY DONALD B.
Published: December 2008

The purpose of this study is to demonstrate three ways of analyzing qualitative interview data: content, cultural models, and narrative analyses. We draw upon the same set of interview data with 250 Latino parents of young children with mental retardation or developmental delay to illustrate these three types of analyses. Interwoven with the analyses are suggestions for ways in which studies in early intervention could be extended by considering different approaches to examining qualitative data.
Male Adolescent Bullying and the School Shooter
Authors: Reuter-Rice Karin
Published: December 2008

An extensive review of the literature reveals that adolescent male victims of peer bullying suffer somatic and emotional consequences from being victimized. Limited research on school shooters found that a significant number of them were adolescents who were targets of bullies and claimed their shootings were in response to their victimization. To date, there is no profile of the school shooter, although research has suggested various dynamics that contribute to an environment that can predispose a community to a school shooting. No published nursing research has examined the relationship between bullying and the school shooter or that of adolescents who experience peer bullying. Despite findings from other disciplines, such as law enforcement, education, sociology, and psychology, school nurses need to be part of the team to recognize the bullied teen and intervene before there are serious life-threatening consequences.
A Comparison of the Number of Hours of Sleep in High School Students Who Took Advanced Placement and/or College Courses and Those Who Did Not
Authors: Jin Qiushuang , Shi Qian
Published: December 2008

This study investigated the association between sleep deprivation and enrollment in Advanced Placement (AP) and/or college courses among high school students. Approximately 4,000 surveys were distributed, and 2,197 completed surveys were returned from students in Grades 9 to 12 at 15 high schools in Iowa. Findings indicated the majority of high school students were sleep deprived. Sleep deprivation was significantly associated with enrollment in AP/college courses. Results indicated that enrollment in AP/college courses had a greater impact on younger students than older students. Compared with non–AP/college course takers, AP/college course takers slept approximately 20 minutes less per night. Specifically, 9th- and 10th-grade AP/college course takers slept approximately 1 hour less and 40 minutes less, respectively. In addition, students enrolled in two or more AP/college classes received 1 hour less and 30 minutes less among 10th and 11th graders, respectively. These results provide useful information on adolescent sleep patterns for school nurses.
SIP response of artificial biofilms
Authors: Ntarlagiannis Dimitrios , Ferguson Andrew
Published: December 2008

In recent years, research into the use of geophysical methods for monitoring microbial activity within the subsurface has advanced. One of the most promising methods, induced polarization (IP), has indirectly shown sensitivity to changes in the presence of microbial cells and especially biofilms. The aim of this study is to conclusively show, and quantify if possible, the effect of biofilm accumulation on IP signals. To ensure the controlled formation of biofilm we created an “artificial” alginate gel biofilm, which was introduced into an experimental column at varying amounts. The IP response was measured throughout. Our initial results showed that the IP method is sensitive to biofilm accumulation, after a certain point, and could potentially be used to monitor such microbial structures remotely. Further research with real biofilms under more natural conditions is needed to validate our results.
Introduction: “More Trouble than They Are Worth”
Authors: Perl Jeffrey M. , Griffiths Paul J. , Evans G. R. , Davis Clark
Published: December 2008

This essay, which is the editor's introduction to part 1 of a multipart symposium on quietism, also constitutes his call for symposium papers. The symposium is meant be comprehensive. It is described as political and broadly cultural as well as religious, and in religious terms is said to cover not only the Catholic and Protestant quietisms (most properly so called) of the late seventeenth and eighteenth centuries, but also the proto-quietisms of the medieval Western church and reputedly quietist aspects of the Gnostic, Eastern Orthodox, early Hasidic, Shi'ite, Jain and other Indic, Taoist, and Zen religious traditions. This introduction emphasizes the secular approaches, mostly antipolitical or postphilosophical, that wear the adjective “quietist” metaphorically, including the postmodern currents that Martha Nussbaum has named “hip quietism” and the “minimalist” philosophical version developed by Wittgenstein and some of his successors, notably Richard Rorty. This introduction concludes with attention to Rorty's late essay “Naturalism and Quietism,” then with a dedication of the entire symposium to Rorty's memory.
SOX9 Directly Binds CREB as a Novel Synergism With the PKA Pathway in BMP-2–Induced Osteochondrogenic Differentiation
Authors: Zhao Li , Li Gang , Zhou Guang-Qian
Published: December 2008

SOX9 acts as a master transcription factor in osteochondrogenesis, and the phosphorylation by protein kinase A (PKA) has been shown to increase its DNA binding and transactivation activity. The PKA pathway is involved in the complex downstream signaling underlying the BMP-2–mediated osteochondrogenesis. This study therefore aimed at further analyzing the possible cross-talk between the SOX9 and the PKA regulation on the background of BMP-2 stimulation. It was first shown that the removal of the residues serine 64 and 211 of SOX9 diminished, but did not completely deplete, its stimulatory effect on the expression of both osteo- and chondrogenic markers. PKA activators and inhibitors increased and decreased the action of wildtype and mutated SOX9, respectively. Interestingly, the interplay of the SOX9 action with the PKA pathway was further shown to occur through direct physical association between SOX9 and CREB, a prototypical PKA downstream transcription factor. Moreover, the binding was shown to be an active biological event happening on BMP-2 stimulation. The C-terminal domain of SOX9 and amino acid residue serine at position 133 of CREB were identified to be involved in the interaction. The action of SOX9 was enhanced by overexpressing CREB. These results suggest that PKA signaling synergizes with SOX9 at the nuclear and cytoplasmic levels to promote BMP-2–induced osteochondrogenic differentiation.
BMD in Population-Based Adult Women Is Associated With Socioeconomic Status
Authors: Brennan Sharon L , Henry Margaret J , Wluka Anita E , Nicholson Geoffrey C , Kotowicz Mark A , Williams Joanne W , Pasco Julie A
Published: December 2008

With few exceptions, an inverse relationship exists between social disadvantage and disease. However, there are conflicting data for the relationship between socioeconomic status (SES) and BMD. The aim of this study was to assess the association between SES and lifestyle exposures in relation to BMD. In a cross-sectional study conducted using 1494 randomly selected population-based adult women, we assessed the association between SES and lifestyle exposures in relation to BMD. BMD was measured at multiple anatomical sites by DXA. SES was determined by cross-referencing residential addresses with Australian Bureau of Statistics 1996 census data for the study region and categorized in quintiles. Lifestyle variables were collected by self-report. Regression models used to assess the relationship between SES and BMD were adjusted for age, height, weight, dietary calcium, smoking, alcohol consumption, physical activity, hormone therapy, and calcium/vitamin D supplements. Unadjusted BMD differed across SES quintiles (p < 0.05). At each skeletal site and SES index, a consistent peak in adjusted BMD was observed in the mid-quintiles. Differences in adjusted BMD were observed between SES quintiles 1 and 4 (3–7%) and between quintiles 5 and 4 (2–7%). At the spine, the maximum difference was observed (7.5%). In a subset of women, serum 25(OH)D explained a proportion of the association between SES and BMD (difference remained up to 4.2%). Observed differences in BMD across SES quintiles, consistent across both SES indices, suggest that low BMD may be evident for both the most disadvantaged and most advantaged.
Genome-Wide Analysis of Genes Related to Kidney Stone Formation and Elimination in the Calcium Oxalate Nephrolithiasis Model Mouse: Detection of Stone-Preventive Factors and Involvement of Macrophage Activity
Authors: Okada Atsushi , Yasui Takahiro , Hamamoto Shuzo , Hirose Masahito , Kubota Yasue , Itoh Yasunori , Tozawa Keiichi , Hayashi Yutaro , Kohri Kenjiro
Published: December 2008

We previously established a mouse kidney stone formation model and showed that mice have a higher tolerance to stone formation than rats. Furthermore, we showed that the generated calcium oxalate crystal deposits could be eliminated after several days. This study investigated the transcriptome of stone formation and elimination in the mouse kidney based on gene selection using a microarray technique. Eight-week-old male C57BL/6N mice were administered 80 mg/kg glyoxylate for 15 days, and kidney calcium oxalate crystal depositions had increased by day 6; thereafter, depositions decreased gradually and had almost disappeared by day 15. On microarray analysis, mRNA expression in the crystal-formed kidneys showed the significant expression of 18,064 genes. Thirty-one, 21, and 25 genes showed at least a 2-fold increased expression during the experimental course (days 3–15), stone formation phase-specific (days 3–6), and stone elimination phase-specific (days 9–15) stages, respectively. Among these genes, those related to chemotaxis and monocyte/macrophage activation were identified. Gene ontology analysis to identify overexpressed genes highlighted categories related to inflammation, immune reactions and the complement activation pathway. Quantitative PCR of 17 previously reported stone-related genes with a significant expression on microarray analysis showed significantly increased chemokines, stone matrix proteins, and their receptors; the significant decrease of several types of transporters and superoxide dismutase; and the persistently high expression of Tamm-Horsfall protein throughout the experiment. In conclusion, inflammation and immune reactivity through macrophage migration are involved in stone formation and elimination in mouse kidneys.
Subtrochanteric and Diaphyseal Femur Fractures in Patients Treated With Alendronate: A Register-Based National Cohort Study
Authors: Abrahamsen Bo , Eiken Pia , Eastell Richard
Published: December 2008

Alendronate (aln) is a potent bisphosphonate with a prolonged duration of action. Recent reports have found long-term aln use to be common in patients with subtrochanteric or proximal diaphyseal femur fracture, raising concerns that these fractures could be a consequence of excessive suppression of bone turnover. Two national observational register-based studies were performed: (1) cross-sectional study (N = 11,944) comparing age distribution, exposure, and trauma mechanisms between different types of proximal femur fractures and (2) matched cohort study in patients with prior nonhip fractures (N = 5187 + 10,374), testing the hypothesis that the increase in the risk of subsequent atypical femur fractures exceeded the increase in typical hip fractures. We also sought evidence of a dose-response relationship, where high adherence to or long-term use of aln led to more atypical femur fractures. We found that 7% of patients with atypical fractures were aln exposed, and the same was found for typical hip fractures. In the cohort study, the HR for subtrochanteric/diaphyseal fracture with aln was 1.46 (0.91–2.35, p = 0·12) compared with 1.45 (1.21–1.74, p < 0·001) for hip fracture after adjustment for comorbidity and co-medications. The risk was reduced by high adherence, and the ratio between hip and subtrochanteric/diaphyseal femur fractures was identical in aln-treated patients and the control cohort even in the limited number of patients who received long-term treatment. Subtrochanteric/diaphyseal femur fractures share the epidemiology and treatment response of classical hip fractures and are best classified as osteoporotic fractures.
Localization of the Cis-Enhancer Element for Mouse Type X Collagen Expression in Hypertrophic Chondrocytes In Vivo
Authors: Zheng Qiping , Keller Bettina , Zhou Guang , Napierala Dobrawa , Chen Yuqing , Zabel Bernhard , Parker Andrew E , Lee Brendan
Published: December 2008

The type X collagen gene (Col10a1) is a specific molecular marker of hypertrophic chondrocytes during endochondral bone formation. Mutations in human COL10A1 and altered chondrocyte hypertrophy have been associated with multiple skeletal disorders. However, until recently, the cis-enhancer element that specifies Col10a1 expression in hypertrophic chondrocytes in vivo has remained unidentified. Previously, we and others have shown that the Col10a1 distal promoter (−4.4 to −3.8 kb) may harbor a critical enhancer that mediates its tissue specificity in transgenic mice studies. Here, we report further localization of the cis-enhancer element within this Col10a1 distal promoter by using a similar transgenic mouse approach. We identify a 150-bp Col10a1 promoter element (−4296 to −4147 bp) that is sufficient to direct its tissue-specific expression in vivo. In silico analysis identified several putative transcription factor binding sites including two potential activator protein-1 (AP-1) sites within its 5′- and 3′-ends (−4276 to −4243 and −4166 to −4152 bp), respectively. Interestingly, transgenic mice using a reporter construct deleted for these two AP-1 elements still showed tissue-specific reporter activity. EMSAs using oligonucleotide probes derived from this region and MCT cell nuclear extracts identified DNA/protein complexes that were enriched from cells stimulated to hypertrophy. Moreover, these elements mediated increased reporter activity on transfection into MCT cells. These data define a 90-bp cis-enhancer required for tissue-specific Col10a1 expression in vivo and putative DNA/protein complexes that contribute to the regulation of chondrocyte hypertrophy. This work will enable us to identify candidate transcription factors essential both for skeletal development and for the pathogenesis of skeletal disorders.
Bone Structure at the Distal Radius During Adolescent Growth
Authors: Kirmani Salman , Christen David , van Lenthe G Harry , Fischer Philip R , Bouxsein Mary L , McCready Louise K , Melton L Joseph , Riggs B Lawrence , Amin Shreyasee , Müller Ralph , Khosla Sundeep
Published: December 2008

The incidence of distal forearm fractures peaks during the adolescent growth spurt, but the structural basis for this is unclear. Thus, we studied healthy 6- to 21-yr-old girls (n = 66) and boys (n = 61) using high-resolution pQCT (voxel size, 82 μm) at the distal radius. Subjects were classified into five groups by bone-age: group I (prepuberty, 6–8 yr), group II (early puberty, 9–11 yr), group III (midpuberty, 12–14 yr), group IV (late puberty, 15–17 yr), and group V (postpuberty, 18–21 yr). Compared with group I, trabecular parameters (bone volume fraction, trabecular number, and thickness) did not change in girls but increased in boys from late puberty onward. Cortical thickness and density decreased from pre- to midpuberty in girls but were unchanged in boys, before rising to higher levels at the end of puberty in both sexes. Total bone strength, assessed using microfinite element models, increased linearly across bone age groups in both sexes, with boys showing greater bone strength than girls after midpuberty. The proportion of load borne by cortical bone, and the ratio of cortical to trabecular bone volume, decreased transiently during mid- to late puberty in both sexes, with apparent cortical porosity peaking during this time. This mirrors the incidence of distal forearm fractures in prior studies. We conclude that regional deficits in cortical bone may underlie the adolescent peak in forearm fractures. Whether these deficits are more severe in children who sustain forearm fractures or persist into later life warrants further study.
Using Telemedicine to Improve the Care Delivered to Sexually Abused Children in Rural, Underserved Hospitals
Authors: MacLeod Kristen J. , Marcin James P. , Boyle Cathy , Miyamoto Sheridan , Dimand Robert J. , Rogers Kristen K.
Published: December 2008

OBJECTIVE. We used live telemedicine consultations to assist remote providers in the examination of sexually assaulted children presenting to rural, underserved hospitals. We hypothesized that telemedicine would increase the ability of the rural provider to perform a complete and accurate sexual assault examination.

PATIENTS AND METHODS. Child abuse experts from a university children's hospital provided 24/7 live telemedicine consultations to clinicians at 2 rural, underserved hospitals. Consultations consisted of videoconferencing to assist in the examination and interpretation of findings during live examinations. Consecutive female patients <18 years of age presenting to the 2 participating hospitals were included. We developed and used an instrument to assess the quality of care and the interventions provided via telemedicine as it related to patient history, physical examination, colposcopic and manual manipulation techniques, interpretation of findings, and treatment plans for victims of child sexual abuse.

RESULTS. Data from 42 live telemedicine consultations were analyzed. The mean duration of the consultations was 71 minutes (range: 25–210 minutes). The consultations resulted in changes in interview methods (47%), the use of the multimethod examination technique (86%), and the use of adjunct techniques (40%). There were 9 acute sexual assault telemedicine consults that resulted in changes to the collection of forensic evidence (89%). Rankings of practitioners' skills and the telemedicine consult effectiveness were high, with the majority of cases scoring ≥5 on a 7-point Likert scale.

CONCLUSIONS. The use of telemedicine to assist in the examination of sexually assaulted children presenting to underserved, rural communities results in significant changes in the methods of examination and evidence collection. It is possible that this model of care results in increased quality of care and appropriate forensic evidence collection.
Platelet Transfusion Practices Among Neonatologists in the United States and Canada: Results of a Survey
Authors: Josephson Cassandra D. , Su Leon L. , Christensen Robert D. , Hillyer Christopher D. , Castillejo Marta-Inés , Emory Michele R. , Lin Yulia , Hume Heather , Easley Kirk , Poterjoy Brandon , Sola-Visner Martha
Published: December 2008

OBJECTIVE. In the absence of scientific evidence, current neonatal platelet transfusion practices are based on physicians' preferences, expert advice, or consensus-driven recommendations. We hypothesized that there would be significant diversity in platelet transfusion triggers, product selection, and dosing among neonatologists in the United States and Canada.

METHODS. A Web-based survey on neonatal platelet transfusion practices was distributed to all members of the American Academy of Pediatrics Perinatal Section in the United States and to all physicians listed in the 2005 Canadian Neonatology Directory.

RESULTS. The overall response rate was 37% (1060 of 2875). In the United States, 37% (1007 of 2700) responded, of which 52% practiced at academic centers. Thirty percent (53 of 175) of Canadians responded, of whom 94% practiced at academic centers. As hypothesized, there was significant practice diversity in both countries. The survey also revealed that platelet transfusions are frequently administered to nonbleeding neonates with platelet counts of >50 × 109/L. This practice is particularly prevalent among neonates with specific clinical conditions, including indomethacin treatment, preceding procedures, in the postoperative period, or with intraventricular hemorrhages.

CONCLUSIONS. There is great variability in platelet transfusion practices among US and Canadian neonatologists, suggesting clinical equipoise in many clinical scenarios. Prospective randomized clinical trials to generate evidence-based neonatal platelet transfusion guidelines are needed.
Models of Comprehensive Multidisciplinary Care for Individuals in the United States With Genetic Disorders
Authors: Grosse Scott D. , Schechter Michael S. , Kulkarni Roshni , Lloyd-Puryear Michele A. , Strickland Bonnie , Trevathan Edwin
Published: December 2008

Approaches to providing comprehensive coordinated care for individuals with complex diseases include the medical home approach, the chronic care model in primary care, and disease-specific, multidisciplinary specialty clinics. There is uneven availability and utilization of multidisciplinary specialty clinics for different genetic diseases. For 2 disorders (ie, hemophilia and cystic fibrosis), effective national networks of specialty clinics exist and reach large proportions of the target populations. For other disorders, notably, sickle cell disease, fewer such centers are available, centers are less likely to be networked, and centers are used less widely. Models of comanagement are essential for promoting ongoing communication and coordination between primary care and subspecialty services, particularly during the transition from pediatric care to adult care. Evaluation of the effectiveness of different models in improving outcomes for individuals with genetic diseases is essential.
Neurofibromatosis Type 1 Revisited
Authors: Williams Virginia C. , Lucas John , Babcock Michael A. , Gutmann David H. , Korf Bruce , Maria Bernard L.
Published: December 2008

Neurofibromatosis type 1 (NF1) is an autosomal dominant condition with a worldwide incidence of ∼1 per 2500 to 3000 individuals. Caused by a germ-line–inactivating mutation in the NF1 gene on chromosome 17, the disease is associated with increased morbidity and mortality. In the past several years, significant progress has been made in standardizing management of the major clinical features of neurofibromatosis type 1. Moreover, improved understanding of how the neurofibromatosis type 1 protein, neurofibromin, regulates cell growth recently provided insight into the pathogenesis of the disease and has led to the development of new therapies. In this review, we describe the clinical manifestations, recent molecular and genetic findings, and current and developing therapies for managing clinical problems associated with neurofibromatosis type 1.
Safety and Immunogenicity of a Pentavalent Vaccine Compared With Separate Administration of Licensed Equivalent Vaccines in US Infants and Toddlers and Persistence of Antibodies Before a Preschool Booster Dose: A Randomized, Clinical Trial
Authors: Guerra Fernando A. , Blatter Mark M. , Greenberg David P. , Pichichero Michael , Noriega Fernando R. ,   on behalf of the Pentacel Study Group
Published: December 2008

OBJECTIVE. Our goal was to compare the safety and immunogenicity of a combination vaccine (DTaP5-IPV-Hib; Pentacel) with that of its separately administered, US-licensed equivalent vaccines (diphtheria, tetanus, 5-component acellular pertussis vaccine [DTaP5; Daptacel], inactivated poliovirus vaccine [IPV; IPOL], and Haemophilus influenzae type b [Hib] vaccine [ActHIB]), when administered to infants and toddlers concomitantly with other routinely recommended vaccines and to assess antibody persistence from the fourth dose in toddlers to the fifth (preschool) DTaP5 dose.

SUBJECTS AND METHODS. In this randomized, multicenter study, 1939 healthy infants were immunized at 2, 4, and 6 months of age with 1 of 3 lots of DTaP5 coadministered with IPV and Hib vaccines or 1 lot of DTaP5-IPV-Hib combination vaccine. Subsequently, 849 of these study participants were given a fourth dose of DTaP5 and Hib vaccines or a fourth dose of DTaP5-IPV-Hib at 1 to 16 months of age. Safety was monitored throughout the study, and blood specimens were obtained to assess antibody responses.

RESULTS. DTaP5-IPV-Hib elicited similar or fewer solicited injection-site and systemic reactions as compared with the separate administration of US-licensed DTaP5, IPV, and Hib vaccines. Seroresponse and seroprotection rates elicited by DTaP5-IPV-Hib were noninferior to US-licensed equivalent vaccines after the infant series and after the fourth dose. Children immunized with DTaP5-IPV-Hib had higher antibody geometric mean concentrations to pertussis toxoid and filamentous hemagglutinin; children immunized with the separate vaccines had higher responses to pertactin. Hib antibody responses to Hib polysaccharide were nearly identical in the DTaP5-IPV-Hib and separate-vaccine groups. Persistence of antibodies to the fifth (preschool) dose was also similar between groups.

CONCLUSIONS. DTaP5-IPV-Hib combination vaccine was shown to be immunogenic and well tolerated. No clinically important differences in the safety or immunologic profiles were noted for DTaP5-IPV-Hib versus the separately administered, US-licensed equivalent vaccines. DTaP5-IPV-Hib is a suitable replacement for separately administered DTaP, IPV, and Hib vaccines.
Hyperbilirubinemia and Language Delay in Premature Infants
Authors: Amin Sanjiv B. , Prinzing Diane , Myers Gary
Published: December 2008

OBJECTIVE. Our goal was to evaluate whether language delay at 3 years in premature infants is associated with previous exposure to hyperbilirubinemia during the first 2 weeks after birth.

PATIENTS AND METHODS. We performed a retrospective case-control study of infants admitted to the NICU between January and October 2003. Inclusion criteria included a birth weight of ≤1500 g and follow-up to age 3 years. Exclusion criteria included genetic disorders and hearing loss or recurrent ear infections. Peak total serum bilirubin levels during the first 2 weeks and duration of hyperbilirubinemia (days with total serum bilirubin level at >8 mg/dL) were determined. Infants with language delay and who were receiving speech therapy by 3 years were identified through developmental clinic charts and a tracking program and compared with infants who had normal language development.

RESULTS. A total of 125 infants with birth weight of ≤1500 g were admitted to the NICU between January and October 2003. Fifteen infants died, and 110 were discharged from the hospital. A total of 102 (93%) of 110 infants had follow-up to the age of 3 years. Four infants were excluded (1 genetic disorder, 3 delayed hearing loss or recurrent ear infections). Twenty-four infants had a language delay and received speech therapy, whereas 74 infants had normal language development. There was no significant difference in peak total serum bilirubin level and duration of hyperbilirubinemia between the 2 groups. On logistic regression, only bronchopulmonary dysplasia was associated with language delay.

CONCLUSIONS. Hyperbilirubinemia, defined as peak total serum bilirubin level or duration of elevated bilirubin in days, is not associated with language delay in premature infants. However, this issue deserves investigation, because other measures of bilirubin, such as unbound bilirubin, may be associated with language delay.
Perinatal Outcomes Associated With Preterm Birth at 33 to 36 Weeks’ Gestation: A Population-Based Cohort Study
Authors: Khashu Minesh , Narayanan Manjith , Bhargava Seema , Osiovich Horacio
Published: December 2008

OBJECTIVE. The aim of our population-based study was to compare the mortality and morbidity of late-preterm infants to those born at term. Advancement in the care of extremely preterm infants has led to a shift of focus away from the more mature preterms, who are being managed as “near terms” and treated as “near normal.” Some recent studies have suggested an increased risk of mortality and morbidity in this group compared with infants born at term. However, there are few population-based mortality and morbidity statistics for this cohort, particularly reflecting current practice.

METHODS. Using data from the British Columbia Perinatal Database Registry we analyzed all singleton births between 33 and 40 weeks’ gestation from April 1999 to March 2002 in the province of British Columbia, Canada. We divided this birth cohort into late preterm (33–36 weeks, n = 6381) and term (37–40 weeks, n = 88 867) groups. We compared mortality and morbidity data and associated maternal factors between the 2 groups.

RESULTS. Stillbirth rate and perinatal, neonatal, and infant mortality rates were significantly higher in the late-preterm group. Infants in this group needed resuscitation at birth more frequently than those in the term group. Late-preterm infants had a significantly higher incidence of respiratory morbidity and infection and had a significantly longer duration of hospital stay. Maternal factors that were more common in the late-preterm group included chorioamnionitis, hypertension, diabetes, thrombophilia, prelabor rupture of membranes, primigravida, and teenage pregnancy.

CONCLUSIONS. Our data support recent literature regarding neonatal mortality and morbidity in late-preterm infants and warrants a review of care for this group at the local, national, and global levels. Reorganization of services and increased resource allocation may be needed in most hospitals and community settings to achieve optimization of care for this group of infants.