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Shriners Hospitals for Children Medical Center - Lexington

Hospital / health systemLexington, Kentucky, United States

Research output, citation impact, and the most-cited recent papers from Shriners Hospitals for Children Medical Center - Lexington (United States). Aggregated across the NobleBlocks index of 300M+ scholarly works.

Total works
418
Citations
15.8K
h-index
63
i10-index
393
Also known as
Shriners Hospitals for Children Medical Center - Lexington

Top-cited papers from Shriners Hospitals for Children Medical Center - Lexington

Outcome tools used for ambulatory children with cerebral palsy: responsiveness and minimum clinically important differences
Donna Oeffinger, Anita Bagley, Selwyn O. Rogers, George E. Gorton +4 more
2008· Developmental Medicine & Child Neurology315doi:10.1111/j.1469-8749.2008.03150.x

This prospective longitudinal multicenter study of ambulatory children with cerebral palsy (CP) examined changes in outcome tool score over time, tool responsiveness, and used a systematic method for defining minimum clinically important differences (MCIDs). Three hundred and eighty-one participants with CP (Gross Motor Function Classification System [GMFCS] Levels I-III; age range 4-18y, mean age 11y [SD 4y 4mo]; 265 diplegia, 116 hemiplegia; 230 males, 151 females). At baseline and follow-up at least 1 year later, Functional Assessment Questionnaire, Gross Motor Function Measure, Pediatric Quality of Life Inventory, Pediatric Outcomes Data Collection Instrument, Pediatric Functional Independence Measure, temporal-spatial gait parameters, and oxygen cost were collected. Adjusted standardized response means determined tool responsiveness for nonsurgical (n=292) and surgical (n=87) groups at GMFCS Levels I to III. Most scores reaching medium or large effect sizes were for GMFCS Level III. Nonsurgical group change scores were used to calculate MCID thresholds for ambulatory children with CP. These values were verified by examining participants who changed GMFCS levels. Tools measuring function were responsive when a change large enough to cause a change in GMFCS level occurred. MCID thresholds assess change in study populations over time, and serve as the basis for designing prospective intervention studies.

Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies: II. The childhood myositis assessment scale (CMAS): a quantitative tool for the evaluation of muscle function
Daniel J. Lovell, Carol B. Lindsley, Robert M. Rennebohm, S. Ballinger +4 more
1999· Arthritis & Rheumatism240doi:10.1002/1529-0131(199910)42:10<2213::aid-anr25>3.0.co;2-8

OBJECTIVE: To develop, validate, and determine the measurement characteristics of a quantitative tool for assessing the severity of muscle involvement in children with idiopathic inflammatory myopathies. METHODS: The Childhood Myositis Assessment Scale (CMAS) was developed from 2 existing observational functional assessment tools to assess muscle function in the areas of strength and endurance across a wide range of ability and ages. The 14 ordinal items included were chosen to assess primarily axial and proximal muscle groups and are ranked with standard performance and scoring methods. Following the development of the CMAS, a training video and written instructions were developed and reviewed by the physicians participating in this study. Subsequently, utilizing a randomized block design, 12 physicians independently scored 10 children (9 with dermatomyositis, 1 with polymyositis; ages 4-15 years) twice in one day (morning and afternoon) on the CMAS. A pediatric physical therapist performed quantitative manual muscle strength testing (MMT) twice on each child (morning and afternoon), including the neck, trunk, and proximal and distal extremity muscle groups. RESULTS: The CMAS has a potential range of 0-51, with higher scores indicating greater muscle strength and endurance. The observed mean for the 10 patients was 36.4 (median 44, SD 14.1, observed range 5-51). The total score for the CMAS correlated with the physician's global assessment (by visual analog scale) of disease activity, the MMT score, serum creatine kinase level, and the Juvenile Arthritis Functional Assessment Report score. The score on the CMAS was not correlated with patient age. Interrater reliability (Kendall's coefficient of concordance) ranged from 0.77 to 1.0 for individual items (all P < 0.001), and overall, it was 0.95 (P < 0.001). Intrarater reliability for the individual physicians was measured by correlation of the CMAS scores for each patient on 2 separate evaluations and ranged from 0.97 to 0.99, with an overall correlation for all physicians of 0.98 (all P < 0.001). CONCLUSION: The CMAS demonstrated an acceptable range of observed scores, excellent convergent validity, and excellent inter- and intrarater reliability. The CMAS is validated to quantitatively assess muscle function in the areas of strength and endurance in children with idiopathic inflammatory myopathies. It can be used in routine clinical care as well as therapeutic trials.

Treatment of Sialorrhea With Glycopyrrolate
Richard J. Mier, Steven Bachrach, Ryan C. Lakin, Tara Barker +2 more
2000· Archives of Pediatrics and Adolescent Medicine194doi:10.1001/archpedi.154.12.1214

OBJECTIVE: To determine the safety and efficacy of glycopyrrolate in the treatment of developmentally disabled children with sialorrhea. DESIGN: Placebo-controlled, double-blind, crossover dose-ranging study. SETTING: Outpatient facilities in 2 pediatric hospitals. PATIENTS: Thirty-nine children with both developmental disabilities and excessive and bothersome sialorrhea. MAIN OUTCOME MEASURES: Parent and investigator evaluation of change in sialorrhea and adverse effects. RESULTS: Glycopyrrolate in doses of 0.10 mg/kg per dose is effective at controlling sialorrhea. Even at low doses, 20% of children may exhibit adverse effects severe enough to require discontinuation. CONCLUSIONS: Glycopyrrolate is effective in the control of excessive sialorrhea in children with developmental disabilities. Approximately 20% of children given glycopyrrolate may experience substantial adverse effects, enough to require discontinuation of medication. Arch Pediatr Adolesc Med. 2000;154:1214-1218.

Effects of Caffeine on Intermittent Hypoxia in Infants Born Prematurely
Lawrence Rhein, Nicole R. Dobson, Robert A. Darnall, Michael J. Corwin +4 more
2014· JAMA Pediatrics171doi:10.1001/jamapediatrics.2013.4371

IMPORTANCE: Preterm infants have immature respiratory control and resulting intermittent hypoxia (IH). The extent of IH after stopping routine caffeine treatment and the potential for reducing IH with extended caffeine treatment are unknown. OBJECTIVES: To determine (1) the frequency of IH in premature infants after discontinuation of routine caffeine treatment and (2) whether extending caffeine treatment to 40 weeks' postmenstrual age (PMA) reduces IH. DESIGN, SETTING, AND PARTICIPANTS: A prospective randomized clinical study was conducted at 16 neonatal intensive care units in the United States, with an 18-month enrollment period. Preterm infants (<32 weeks' gestation) previously treated with caffeine were randomized to extended caffeine treatment or usual care (controls) at a PMA of at least 34 weeks but less than 37 weeks. Continuous pulse oximeter recordings were obtained through 40 weeks' PMA. Oximeter data were analyzed by persons masked to patient group. INTERVENTION: Continued treatment with caffeine. MAIN OUTCOMES AND MEASURES: Number of IH events and seconds with less than 90% hemoglobin oxygen saturation (Sao2) per hour of recording. RESULTS: Our analysis included 95 preterm infants. In control infants, the mean (SD) time at less than 90% Sao2 at 35 and 36 weeks' PMA was 106.3 (89.0) and 100.1 (114.6) s/h, respectively. The number of IH events decreased significantly from 35 to 39 weeks' PMA (P = .01). Extended caffeine treatment reduced the mean time at less than 90% Sao2 by 47% (95% CI, -65% to -20%) to 50.9 (48.1) s/h at 35 weeks and by 45% (95% CI, -74% to -17%) to 49.5 (52.1) s/h at 36 weeks. CONCLUSIONS AND RELEVANCE: Substantial IH persists after discontinuation of routine caffeine treatment and progressively decreases with increasing PMA. Extended caffeine treatment decreases IH in premature infants. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT01875159.

Immunotherapy for osteosarcoma: Where do we go from here?
Mary Frances Wedekind, Lars M. Wagner, Timothy P. Cripe
2018· Pediatric Blood & Cancer158doi:10.1002/pbc.27227

Osteosarcoma is the most common bone tumor in children and young adults, with few advances in survival and treatment, especially for metastatic disease, in the last 30 years. Recently, immunotherapy has begun to show promise in various adult cancers, but the utility of this approach for osteosarcoma remains relatively unexplored. In this review, we outline the mechanisms and status of immunotherapies currently in clinical trials as well as future therapies on the horizon, and discuss their potential application for osteosarcoma.

A PAX1 enhancer locus is associated with susceptibility to idiopathic scoliosis in females
Swarkar Sharma, Douglas Londoño, Walter L. Eckalbar, Xiaochong Gao +4 more
2015· Nature Communications156doi:10.1038/ncomms7452

Idiopathic scoliosis (IS) is a common paediatric musculoskeletal disease that displays a strong female bias. By performing a genome-wide association study (GWAS) of 3,102 individuals, we identify significant associations with 20p11.22 SNPs for females (P=6.89 × 10(-9)) but not males (P=0.71). This association with IS is also found in independent female cohorts from the United States of America and Japan (overall P=2.15 × 10(-10), OR=1.30 (rs6137473)). Unexpectedly, the 20p11.22 IS risk alleles were previously associated with protection from early-onset alopecia, another sexually dimorphic condition. The 174-kb associated locus is distal to PAX1, which encodes paired box 1, a transcription factor involved in spine development. We identify a sequence in the associated locus with enhancer activity in zebrafish somitic muscle and spinal cord, an activity that is abolished by IS-associated SNPs. We thus identify a sexually dimorphic IS susceptibility locus, and propose the first functionally defined candidate mutations in an enhancer that may regulate expression in specific spinal cells.

Natural History of Untreated Chronic Slipped Capital Femoral Epiphysis
Brian T. Carney, Stuart L. Weinstein
1996· Clinical Orthopaedics and Related Research151doi:10.1097/00003086-199601000-00006

From 1915 to 1952, 31 hips in 28 patients with slipped capital femoral epiphysis were observed without interventional treatment at the University of Iowa Department of Orthopaedic Surgery. A slip was considered chronic if the symptoms had been present for 3 weeks or more. The severity of the slip was determined by radiographs. Assessment included Iowa Hip Rating and grading of radiographic degenerative changes. The mean duration of patient followup from the onset of symptoms was 41 years. The mean patient age was 54 years old at review, and 13 years old at onset of symptoms. Degenerative arthritis developed in hips with displaced slipped capital femoral epiphysis. Untreated slipped capital femoral epiphysis can progress to a severe degree. The natural history of chronic slipped capital femoral epiphysis is favorable provided that displacement is minimal and remains so.

Social and Community Participation of Children and Youth With Cerebral Palsy Is Associated With Age and Gross Motor Function Classification
Robert J. Palisano, Lin-Ju Kang, Lisa A. Chiarello, Margo Orlin +2 more
2009· Physical Therapy123doi:10.2522/ptj.20090162

BACKGROUND: Through social and community participation, children and youth with cerebral palsy (CP) form friendships, gain knowledge, learn skills, express creativity, and determine meaning and purpose in life. OBJECTIVE: The purposes of this study were: (1) to determine whether social and community participation of children and youth with CP differ based on age, sex, and gross motor function, and (2) to identify the types of activities in which social and community participation are highest. DESIGN AND METHODS: A prospective cross-sectional analytic design was used. The participants were a sample of convenience of 291 children (6-12 years of age) and 209 youth (13-21 years of age) with CP (55.4% males, 44.6% females) receiving services from 7 children's hospitals. Participants completed the Children's Assessment of Participation and Enjoyment (CAPE) by structured interview. Gross Motor Function Classification System (GMFCS) level was determined by the researchers. RESULTS: Youth did a higher percentage of activities with friends and others and outside the home than children. Children and youth in level I did a higher percentage of activities with friends and others compared with children and youth in levels II and III and in levels IV and V. Children and youth in level I and in levels IV and V did a higher percentage of activities outside the home than children and youth in levels II and III. Differences were not found between females and males. The percentage of activities done with friends and others and outside the home was highest for physical and skill-based activities. LIMITATIONS: Findings cannot be attributed only to GMFCS level. CONCLUSIONS: The ability to walk without restrictions is desirable for social and community participation. For children and youth with CP who have limitations in mobility, physical therapists have roles as consultants for accessibility, activity accommodations, and assistive technology and as advocates for inclusive environments.

MultiDimensional ClinOmics for Precision Therapy of Children and Adolescent Young Adults with Relapsed and Refractory Cancer: A Report from the Center for Cancer Research
Wendy Chang, Andrew S. Brohl, Rajesh Patidar, Sivasish Sindiri +4 more
2016· Clinical Cancer Research121doi:10.1158/1078-0432.ccr-15-2717

PURPOSE: We undertook a multidimensional clinical genomics study of children and adolescent young adults with relapsed and refractory cancers to determine the feasibility of genome-guided precision therapy. EXPERIMENTAL DESIGN: Patients with non-central nervous system solid tumors underwent a combination of whole exome sequencing (WES), whole transcriptome sequencing (WTS), and high-density single-nucleotide polymorphism array analysis of the tumor, with WES of matched germline DNA. Clinically actionable alterations were identified as a reportable germline mutation, a diagnosis change, or a somatic event (including a single nucleotide variant, an indel, an amplification, a deletion, or a fusion gene), which could be targeted with drugs in existing clinical trials or with FDA-approved drugs. RESULTS: Fifty-nine patients in 20 diagnostic categories were enrolled from 2010 to 2014. Ages ranged from 7 months to 25 years old. Seventy-three percent of the patients had prior chemotherapy, and the tumors from these patients with relapsed or refractory cancers had a higher mutational burden than that reported in the literature. Thirty patients (51% of total) had clinically actionable mutations, of which 24 (41%) had a mutation that was currently targetable in a clinical trial setting, 4 patients (7%) had a change in diagnosis, and 7 patients (12%) had a reportable germline mutation. CONCLUSIONS: We found a remarkably high number of clinically actionable mutations in 51% of the patients, and 12% with significant germline mutations. We demonstrated the clinical feasibility of next-generation sequencing in a diverse population of relapsed and refractory pediatric solid tumors. Clin Cancer Res; 22(15); 3810-20. ©2016 AACR.

Determinants of intensity of participation in leisure and recreational activities by children with cerebral palsy
Robert J. Palisano, Lisa A. Chiarello, Margo Orlin, Donna Oeffinger +4 more
2010· Developmental Medicine & Child Neurology114doi:10.1111/j.1469-8749.2010.03819.x

AIM: To test a model of child, family, and service determinants of intensity of participation in leisure and recreational activities by children with cerebral palsy (CP). METHOD: Participants were 288 children with CP, age range 6 to 12 years (mean 9y 8mo, SD 2y), and their parents from seven children's hospitals. The sample comprised 166 (57.6%) males and 122 (42.4%) females, and between 40 (13.9%) and 74 (25.7%) children in each of the five levels of the Gross Motor Function Classification System. Children completed the Children's Assessment of Participation and Enjoyment by interview. Parents completed the Pediatric Outcomes Data Collection Instrument, Family Environment Scale, Coping Inventory, Measure of Processes of Care, and two questionnaires. Structural equation modeling was used to test the model. RESULTS: Fit statistics indicated a good model fit. The model explains 32% of the variance in intensity of participation. Path coefficients (p ≤ 0.05) indicate that higher gross motor function, higher enjoyment, more effective adaptive behavior, younger age, and higher family activity orientation are associated with higher intensity of participation. The path between services and participation was not significant. INTERPRETATION: Intensity of participation of children with CP is influenced by multiple child and family determinants. Children's gross motor function and behavior in life situations are important for participation; knowledge of activities the child and family enjoy has implications for opportunities for participation. Professionals are encouraged to address priorities for leisure and recreation identified by children with CP and their families.

Practice-Based Care Coordination: A Medical Home Essential
Jeanne W. McAllister, Elizabeth Presler, W. Carl Cooley
2007· PEDIATRICS107doi:10.1542/peds.2006-1684

Families who raise children and youth with special health care needs deserve a medical home. They expect a team approach to health care, with coordination across multiple services and settings. Children, youth, and families benefit from the organization of critical information into written care summaries and action plans. If primary care pediatricians, family physicians, and internists are to achieve optimal health care quality and improvement of existing health care delivery, care coordination will be an essential contributing process to their team approach. Several national health policy recommendations identify care coordination as a cross-cutting intervention to fill the gap between what exists and what is needed in health care today. A practice-based care-coordination model, including a definition and vision for care, a framework of structures and processes, and a position description with specific competencies, is needed. Improvement methodology provides an effective means for health care teams to implement and evaluate practice-based care coordination within their medical home. The improvement approach and model must be flexibly applied to have utility across diverse health care organizations. A medical home team approach, with fully developed practice-based care-coordination services, will enhance health and cost outcomes for children, youth, and families and heighten the professional satisfaction of those delivering health care.

Gross Motor Function Classification System and outcome tools for assessing ambulatory cerebral palsy: a multicenter study
Donna Oeffinger, Chester Tylkowski, Mary Kay Rayens, R F Davis +4 more
2004· Developmental Medicine & Child Neurology105doi:10.1017/s0012162204000519

The relationships between different levels of severity of ambulatory cerebral palsy, defined by the Gross Motor Function Classification System (GMFCS), and several pediatric outcome instruments were examined. Data from the Gross Motor Function Measure (GMFM), Pediatric Orthopaedic Data Collection Instrument (PODCI), temporal-spatial gait parameters, and oxygen cost were collected from six sites. The sample size for each assessment tool ranged from 226 to 1047 participants. There were significant differences among GMFCS levels I, II, and III for many of the outcome tools assessed in this study. Strong correlations were seen between GMFCS level and each of the GMFM sections D and E scores, the PODCI measures of Transfer and Mobility, and Sports and Physical Function, Gait Velocity, and Oxygen Cost. Correlations among tools demonstrated that the GMFM sections D and E scores correlated with the largest number of other tools. Logistic regression showed GMFM section E score to be a significant predictor of GMFCS level. GMFM section E score can be used to predict GMFCS level relatively accurately (76.6%). Study data indicate that the assessed outcome tools can distinguish between children with different GMFCS levels. This study establishes justification for using the GMFCS as a classification system in clinical studies.

Deformities of the forearm in patients who have multiple cartilaginous exostosis.
Ronald C. Burgess, Harold E. Cates
1993· Journal of Bone and Joint Surgery103doi:10.2106/00004623-199301000-00003

Thirty-five patients who had multiple cartilaginous exostosis and deformities of the wrist and forearm were evaluated to determine the natural history and prognostic factors for the deformities. Radial and ulnar shortening were correlated linearly (r2 = 0.86), showing that both bones of the forearm were symmetrically involved. The angular growth abnormality of the distal aspect of the radius was correlated with radial shortening (r2 = 0.53) and ulnar shortening (r2 = 0.61), but it had no correlation with negative variance of the ulnar (r2 = 0.30). Carpal slip was an independent factor, with no correlation with any of the factors studied. The ulnar tether theory for deformities of the wrist in patients who have multiple cartilaginous exostosis was not substantiated by this study.

Gross Motor Function Classification System and outcome tools for assessing ambulatory cerebral palsy: a multicenter study
Donna Oeffinger, Chester Tylkowski, Mary Kay Rayens, R F Davis +4 more
2004· Developmental Medicine & Child Neurology102doi:10.1111/j.1469-8749.2004.tb00491.x

The relationships between different levels of severity of ambulatory cerebral palsy, defined by the Gross Motor Function Classification System (GMFCS), and several pediatric outcome instruments were examined. Data from the Gross Motor Function Measure (GMFM), Pediatric Orthopaedic Data Collection Instrument (PODCI), temporal‐spatial gait parameters, and oxygen cost were collected from six sites. The sample size for each assessment tool ranged from 226 to 1047 participants. There were significant differences among GMFCS levels I, II, and III for many of the outcome tools assessed in this study. Strong correlations were seen between GMFCS level and each of the GMFM sections D and E scores, the PODCI measures of Transfer and Mobility, and Sports and Physical Function, Gait Velocity, and Oxygen Cost. Correlations among tools demonstrated that the GMFM sections D and E scores correlated with the largest number of other tools. Logistic regression showed GMFM section E score to be a significant predictor of GMFCS level. GMFM section E score can be used to predict GMFCS level relatively accurately (76.6%). Study data indicate that the assessed outcome tools can distinguish between children with different GMFCS levels. This study establishes justification for using the GMFCS as a classification system in clinical studies.

Outcome assessments in children with cerebral palsy, Part I: descriptive characteristics of GMFCS Levels I to III
Donna Oeffinger, George E. Gorton, Anita Bagley, Diane E. Nicholson +4 more
2007· Developmental Medicine & Child Neurology100doi:10.1111/j.1469-8749.2007.00172.x

This prospective cross-sectional multicenter study assessed the relationships between Gross Motor Function Classification System (GMFCS) level and scores on outcome tools used in pediatric orthopedics. Five hundred and sixty-two participants with cerebral palsy (CP; 339 males, 223 females; age range 4-18y, mean age 11y 1mo [SD 3y 7mo]; 400 with diplegia, 162 with hemiplegia; GMFCS Levels I-III;) completed the study. The Functional Assessment Questionnaire (FAQ), Gross Motor Function Measure (GMFM) Dimensions D and E, Pediatric Quality of Life Inventory (PedsQL), the Pediatric Outcomes Data Collection Instrument (PODCI), Pediatric Functional Independence Measure (WeeFIM), temporal-spatial gait parameters, and O(2) cost were collected during one session. Descriptive characteristics are reported by GMFCS level clinicians can use for comparison with individual children. Tools with a direct relationship between outcome scores and GMFCS levels were the PODCI Parent and Child Global Function, Transfers & Basic Mobility, and Sports and Physical Function; PODCI Parent Upper Extremity Function; WeeFIM Self-care and Mobility; FAQ Question 1; GMFM Dimensions D and E; GMFM-66; O(2) cost; and temporal-spatial gait parameters. Child report scores differed significantly higher than Parent scores for six of eight PODCI subscales and three of four PedsQL dimensions. Children classified into different GMFCS levels function differently.

A Comparison of Three Methods of Pain Control for Posterior Spinal Fusions in Adolescent Idiopathic Scoliosis
Todd A. Milbrandt, Manuj Singhal, Christin Minter, Anna McClung +4 more
2009· Spine95doi:10.1097/brs.0b013e3181a90ceb

In Brief Study Design. Retrospective cohort study. Objective. To compare the efficacy of patient-controlled analgesia (PCA) with morphine alone, a single preoperative intrathecal morphine injection and PCA (IT/PCA), and epidural catheter infusion without PCA (EPI) for postoperative pain control after posterior spinal fusion (PSF) and segmental spinal instrumentation (SSI) in adolescent idiopathic scoliosis (AIS). Summary of Background Data. Postoperative pain control after PSF and SSI in AIS can be managed in different ways. EPI provides for a longer period of pain relief but is reliant on the correct placement and maintenance of the catheter in the epidural space. A single preoperative intrathecal morphine injection also provides for long acting analgesia. No direct comparison of these 3 methods of postoperative pain control has been reported. Methods. An IRB-approved retrospective chart review was performed at 2 institutions from 1997 to 2005. The medical record was reviewed to determine pain scores after surgery at multiple time periods. The 3 groups were compared using Student t test and χ2 and significance was defined as P < 0.05. Results. There was no statistical difference in the gender, age, magnitude of curve, or number of levels fused in the IT/PCA (N = 42), PCA (N = 41), or EPI (N = 55) groups. Postoperative pain scores were lowest in the IT/PCA group in the first 8 hours (P < 0.05) but the pain scores in the EPI group were then lower through 24 hours (P < 0.05). Total morphine use (mg/kg) was lower in the IT/PCA group compared with the PCA group at 12 hours and 24 hours (P = 0.0001). Return to solid food ingestion was quickest in the EPI group (2.0 days) followed by the IT/PCA (2.6 days) and PCA alone (3.2 days) (P < 0.002). Respiratory depression and transient neurologic change occurred most frequently in the EPI group (EPI 11/55 pts vs. 1/42 IT/PCA vs. 0/41 PCA P < 0.001). Pruritis was greatest in the epidural group (11/55 P < 0.05). There were no intraoperative somatosensory-evoked potential changes or permanent neurologic injury recorded in any group. Conclusion. An EPI controls postoperative pain for the longest period of time and allows for a quicker return to consumption of solid foods. However, a single preoperative intrathecal morphine injection controls the pain equally for the first 24 hours with less pruritis and with less adverse events thus requiring less nursing and physician intervention after PSF and SSI in AIS. All methods were safe with no neurologic injury recorded. Three different pain management methods were evaluated after posterior spinal fusion and instrumentation; intravenous patient-controlled analgesia, continuous epidural infusion, and a single preoperative intrathecal injection. We observed that the epidural catheter infusion provides analgesia for the longest period of time and allows for a quicker return to consumption of solid foods. However, a single preoperative intrathecal morphine injection controls the pain equally for the first 24 hours with less pruritis and fewer adverse events thus requiring less nursing and physician intervention. All methods were safe with no neurologic injury recorded.

Function and Well-Being in Ambulatory Children With Cerebral Palsy
Marinis Pirpiris, Philip E. Gates, James J. McCarthy, Jacques DʼAstous +4 more
2005· Journal of Pediatric Orthopaedics91doi:10.1097/01.bpo.0000191553.26574.27

The purpose of this study was to determine whether there is a significant association between function and well-being in children with cerebral palsy. To determine this, the authors used validated measures of function (Gillette Functional Assessment Questionnaire, Gross Motor Function Classification System, Gross Motor Function Measure, and walking speed) and correlated them to health-related quality of life (HRQOL) measures (Pediatric Outcomes Data Collection Instrument, Pediatric Quality of Life instrument). In a cross-sectional study of ambulatory children with mild to moderate cerebral palsy aged 10.2 +/- 3.2 years, mild to moderate decreases in function were found when compared with normative data. As the assessment of HRQOL comprises both functional well-being and psychosocial well-being, the authors decided to specify the aspect of well-being to which they were referring. It was found that the child's function was not correlated to psychosocial well-being. The children with mild cerebral palsy had greater effects on their psychosocial well-being than would be predicted by their functional disability. Functional measures were good at predicting the functional well-being but were weak at predicting the psychosocial arm of well-being.

Deactivating Germline Mutations in <i>LEMD3</i> Cause Osteopoikilosis and Buschke-Ollendorff Syndrome, but Not Sporadic Melorheostosis
Steven Mumm, Deborah Wenkert, Xiafang Zhang, William H. McAlister +2 more
2006· Journal of Bone and Mineral Research85doi:10.1359/jbmr.061102

UNLABELLED: Autosomal dominant OPK and BOS feature widespread foci of osteosclerotic trabeculae without or with skin lesions, respectively. Occasionally, a larger area of dense bone in OPK or BOS resembles MEL, a sporadic sclerosing disorder primarily involving cortical bone. Others, finding deactivating germline LEMD3 mutations in OPK or BOS, concluded such defects explain all three conditions. We found germline LEMD3 mutations in OPK and BOS but not in sporadic MEL. INTRODUCTION: In 2004, others discovered that heterozygous, loss-of-function, germline mutations in the LEMD3 gene (LEMD3 or MAN1) cause both osteopoikilosis (OPK) and Buschke-Ollendorff syndrome (BOS). OPK is an autosomal dominant, usually benign, skeletal dysplasia featuring multiple, small, especially metaphyseal, oval or round, dense trabecular foci distributed symmetrically throughout the skeleton. BOS combines OPK with connective tissue nevi comprised of collagen and elastin. In some OPK and BOS families, an individual may have relatively large, asymmetric areas of dense cortical bone interpreted as melorheostosis (MEL). MEL, however, classically refers to a sporadic, troublesome skeletal dysostosis featuring large, asymmetric, "flowing hyperostosis" of long bone cortices often with overlying, constricting soft tissue abnormalities. However, a heterozygous germline mutation in LEMD3 was offered to explain MEL. MATERIALS AND METHODS: We studied 11 unrelated individuals with sclerosing bone disorders where LEMD3 mutation was a potential etiology: familial OPK (1), familial BOS (2), previously reported familial OPK with MEL (1), sporadic MEL (3), sporadic MEL with mixed-sclerosing-bone dystrophy (1), and patients with other unusual sclerosing bone disorders (3). All coding exons and adjacent mRNA splice sites for LEMD3 were amplified by PCR and sequenced using genomic DNA from leukocytes. We did not study lesional tissue from bone or skin. RESULTS: In the OPK family, a heterozygous nonsense mutation (c.1433T>A, p.L478X) was discovered in exon 1. In the two BOS families, a heterozygous nonsense mutation (exon 1, c.1323C>A, p.Y441X) and a heterozygous frame-shift mutation (exon 1, c.332_333insTC) were identified. In the individual with MEL and familial OPK, a heterozygous nonsense mutation (c.1963C>T, p.R655X) was detected in exon 7. However, no LEMD3 mutation was found for any other patient, including all four with sporadic MEL. CONCLUSIONS: We confirm that OPK and BOS individuals, including those with MEL-like lesions, have heterozygous, deactivating, germline LEMD3 mutations. However, MEL remains of unknown etiology.

Clinically prescribed orthoses demonstrate an increase in velocity of gait in children with cerebral palsy: a retrospective study
Hank White, Jennifer Jenkins, William Neace, Chester Tylkowski +1 more
2002· Developmental Medicine & Child Neurology81doi:10.1017/s0012162201001992

The purpose of this study was to determine the effect clinically prescribed ankle-foot orthoses (AFOs) have on the temporal-spatial parameters of gait, as compared with barefoot walking in children with cerebral palsy. A retrospective chart review of data collected between 1995 and 1999 in our motion analysis laboratory was performed. A retrospective chart review of 700 patients revealed 115 patients (mean age 9 years) who had a primary diagnosis of CP (diplegia n=97, hemiplegia n=18). All were wearing clinically prescribed hinged or solid AFOs at the time of undergoing a three dimensional gait analysis. In line with our standard clinical practice, data for both conditions (braced and barefoot walking) were collected the same day by the same examiner. Statistical analyses indicated the temporal and spatial gait parameters of velocity, stride length, step length, and single limb stance were significantly increased (p<0.001) with the use of AFOs versus barefoot walking. Cadence was the only parameter found to not be statistically different.

The results of preoperative halo-gravity traction in children with severe spinal deformity
Tigran Garabekyan, Pooya Hosseinzadeh, Henry J. Iwinski, Ryan D. Muchow +3 more
2013· Journal of Pediatric Orthopaedics B81doi:10.1097/bpb.0b013e32836486b6

Halo-gravity traction has been used preoperatively for patients with severe spinal deformity but there are limited data in the literature on the results and complications. We studied the outcomes of perioperative halo-gravity traction in children with severe spinal deformity. A retrospective study was carried out on patients who were treated at our center. Twenty-one patients were included in the study. Radiographic and pulmonary function parameters showed significant improvement during the course of traction and at the final follow-up. The overall complication rate was 19%, including two patients with pin loosening and two patients with superficial pin-site infections treated with oral antibiotics.