Deficits In Pediatric Patients Research Articles

Lower extremity inter-joint coupling angles and variability during gait in pediatric hypermobility spectrum disorder

Complex musculoskeletal complications in children with hypermobility spectrum disorder (HSD) include pain, proprioception deficits, and joint instability, which may result in movement dysfunction during walking. However, no studies have explored the inter-joint coordination deficits in children with HSD. The purpose of this study was to determine the lower extremity inter-joint coupling angles, patterns, and variability during walking in children with HSD compared to children without HSD (non-HSD). Ankle, knee, and hip kinematics during the stance phase of walking in 18 children with HSD and 18 children without HSD were measured using three-dimensional motion analysis. Coupling angles, patterns, and variability of hip-knee, hip-ankle, and knee-ankle were quantified in the sagittal, frontal, and transverse planes using vector coding techniques. Statistical modeling of coupling angles on sine and cosine scales and bootstrapped standard errors were used to compare coupling angles between HSD and non-HSD groups. Permutational multivariate analysis of variance and statistical non-parametric mapping two-sample t-tests were used to compare the coupling patterns and variability between HSD and non-HSD groups, respectively. Our results indicated that coupling angles, patterns, and variability were not significantly different between the groups. These findings suggest that lower extremity inter-joint coordination and its variability during walking might not be a promising area for further research or intervention in children with HSD. Further research could use other biomechanical methods to investigate coordination deficits in pediatric patients with HSD, and how aging and disease progression are associated with coordination deficits in individuals with HSD.

Evidence of vestibular dysfunction in children with enlarged vestibular aqueduct

ObjectiveTo investigate the occurrence and characteristics of balance and vestibular deficits in pediatric patients with enlarged vestibular aqueduct (EVA). Materials and methodsRetrospective review of 53 children with EVA who underwent a comprehensive vestibular evaluation in our pediatric balance and vestibular program. Laboratory testing included videonystagmography (VNG), rotary chair, video head impulse testing (vHIT), vestibular evoked myogenic potential (VEMP), subjective visual vertical (SVV) and Sensory Organization Test (SOT) in posturography. ResultsThe mean age of these children, 31 girls and 22 boys, was 7.1 years (SD = 4.8). Among these 53 children, 16 had unilateral EVA (7 on the left side and 9 on the right side) and 37 had bilateral EVA, in which genetic testing confirmed 5 cases of Pendred syndrome. Abnormal testing results were found in 58% (11/19) on SOT, 67% (32/48) on rotary chair, 55% (48/88 of ears) on VEMP, 30% (8/27) on vHIT, 39% (7/18) on SVV, and 8% (4/53) on VNG. ConclusionsVestibular dysfunction may be a common finding in children with EVA. Clinicians who provide medical care for children with EVA need to be familiar with signs of potential balance and vestibular impairments. Although performing vestibular evaluation on young children with EVA can be difficult, objective testing is important in order to identify any potential vestibular deficit in these pediatric patients so that proper vestibular rehabilitation and balance retraining can be provided.

Balance and Vestibular Deficits in Pediatric Patients with Autism Spectrum Disorder: An Underappreciated Clinical Aspect.

Children with autism spectrum disorder (ASD) not only have communication and social difficulties, but also exhibit poor balance and motor control ability, which frequently affect daily activities. Effective balance and motor control rely on the integration of somatosensory, visual, and vestibular inputs. Although reports of balance dysfunction in ASD have been documented, comprehensive studies of balance and vestibular function in children with ASD are scarce. In this study, we retrospectively reviewed 36 pediatric patients diagnosed with ASD who underwent balance/vestibular laboratory testing in our speciality clinic. Results from sensory organization test (SOT) or modified clinical test for sensory integration of balance (mCTSIB) found that out of 15 patients, 80% had abnormal findings. Of the children who successfully completed each vestibular test, abnormal responses were observed in 12 (80%) sensory organization tests, 5 (24%) vestibular evoked myogenic potential (VEMP), 22 (66%) videonystagmography (VNG), and 11 (32%) sinusoidal rotary chair tests. These results indicate that balance and vestibular testing may be of diagnostic value for clinicians and providers as an aid in early detection, intervention, and the development of appropriate management and therapies for this patient population. Increased awareness of this topic is warranted to promote better clinical management of this special group of patients and improve their quality of life.

Relevance of intraoperative motor evoked potentials and D-wave monitoring for the resection of intramedullary spinal cord tumors in children.

The purpose of this study was to evaluate the reliability of motor evoked potentials (MEP), somatosensory evoked potentials (SSEP), and D-wave monitoring as predictors of postoperative neurological deficits in pediatric patients undergoing resection of intramedullary spinal cord tumors (IMSCTs). Additionally, we aimed to determine whether alerts in the intraoperative neurophysiological monitoring (IONM) influenced the extent of resection (EOR). A retrospective analysis of the pediatric patients who underwent resection of IMSCT between March 2010 and April 2021 with an IONM guidance was performed. IONM alerts were recorded and correlated with patients' clinical status at discharge. Twenty three pediatric patients were included. MEP and SSEP were successfully elicited in all patients, while D-wave monitoring was feasible for 14 of them (60.9%). Significant IONM alerts occurred in 6 individuals (26.1%) with monitorable MEP and SSEP and 2 patients with monitorable D-waves (14.3%). The sensitivity, specificity, positive predictive value, and negative predictive value accounted for 100%, 81.8%, 20%, 100% for MEP, 100%, 92.3%, 50%, 100% for D-wave, and 50%, 81%, 20%, 94.44% for SSEP, respectively. Both MEP (p < 0.001) and D-wave monitoring (p < 0.001) accurately predicted postoperative motor deficits, while SSEP failed to provide significant accuracy regarding sensory deficits (p = 0.491). Gross-total tumor resection was performed in 29.4% of patients without IONM alerts and 33.3% of patients with IONM alerts, indicating that IONM alerts did not limit the EOR (p = 0.0857). MEP and D-wave monitoring can be perceived as reliable IONM modalities in pediatric IMSCTs surgery. Caution is needed with the implementation of SSEP to guide surgical decisions.

Children with cavernous malformations of the central nervous system.

Cavernous malformations (CM) of the central nervous system (CNS) are a rare pathology in the pediatric population that may present with an acute onset of severe neurological symptoms. The aim of our study was to evaluate the clinical presentation, family history, genetic background, radiological features, treatment and outcome of children with CM. This observational cohort study included all children with CM of the CNS diagnosed in the period 2000-2020at University Children's Hospital in Ljubljana, Slovenia. Whole exome sequencing was utilized. We identified a cohort of 20 children with CM (mean age 9.3 years, range: 10 days-18.4 years). In our cohort, 16 patients were symptomatic and 4 were asymptomatic; 7 patients had a solitary lesion, and 13 had multiple lesions. Children with multiple lesions become symptomatic at an earlier age compared with children with solitary lesions. We identified five families with familiar cavernous malformation (FCM) syndrome affecting two or more generations; FCM represented 65% of all pediatric cases identified in our study. We confirmed a mutation in FCM associated genes in all but one patient with multiple lesions, with the KRIT1 mutation being the most common. Multiple CM lesions and symptomatic brainstem lesions are associated with worse neurological deficits in pediatric patients. Not all cases of multiple lesions can be linked to mutations in KRIT1, CCM2, or PDCD10, which may indicate that there are other as yet unidentified genes associated with FCM.

Survey of Functional Deficits in Pediatric Patients with Osteogenesis Imperfecta to Determine Rehabilitation Needs: A Retrospective Analysis

<h3>Research Objectives</h3> To investigate components of self-described functional deficits in pediatric patients with osteogenesis imperfecta to understand areas of need for rehabilitation in this population. <h3>Design</h3> A cohort of pediatric patients receiving ongoing OI care in outpatient settings or parents were surveyed using pediatric PROMIS scales across multiple domains. Children too young or unable had parents complete a proxy form. Surveys were repeated within one year to assess for consistency. Data were analyzed retrospectively. <h3>Setting</h3> Patients and families were surveyed during in Shriners Hospitals for Children ambulatory settings. This sample was of the Shriners Chicago patients who completed the PROMIS measures. <h3>Participants</h3> Patients with OI of varied phenotypes seeking care at Shriners Chicago able to complete a form in English or Spanish utilizing a provided tablet were surveyed. Initial results for 102 patients were obtained. <h3>Interventions</h3> Not applicable. <h3>Main Outcome Measures</h3> PROMIS pediatric scales for mobility, upper extremity function, pain interference and peer relations. Repeat surveys were conducted within one year. <h3>Results</h3> Mobility was moderate to severely impaired in 16/60 children, 18/30 parent proxy. Twelve children didn't have a mobility score, as they use a wheelchair for primary mobility. Pain interference was not severe in any patients, but moderate in 25/65 (38%) children and 6/32 (19%) parent proxy. Half of children self-reported mild or no pain interference. Peer relations were mildly or not affected in over 88% (90/102). Upper extremity impairment was moderately to severely affected in about half of the respondents (53/102) with higher impairment by parent proxy than child self-report. <h3>Conclusions</h3> OI is typically characterized by bone fragility, however muscle weakness is a common presenting symptom and muscle abnormalities in this population have been widely described. As 45% of children have reported issues in our cohort with gait/mobility issues or primarily use a wheelchair, anticipatory guided approach to rehabilitation and planning in conjunction with bone-modifying treatment, may improve strength and mobility outcomes. Further study across other Shriners centers is planned. This data may serve as a baseline to assess outcomes of developed programs. <h3>Author(s) Disclosures</h3> N/A.

MiRNA-124a: a Potential Biomarker for Neurological Deficits Following Cardiac Surgery in Pediatric Patients.

Brain injury is a major source of patient morbidity after cardiac surgery in children. New early accurate biomarkers are needed for the diagnosis of patients at risk for cerebral postoperative damage. Specific circulating miRNAs have been found as suitable biomarkers for many diseases. We tested whether miRNA-124a reflects neurological injury in pediatric patients following heart surgery. Serum samples were obtained from 34 patients before and six hours after heart surgery. MiRNAs-124a was quantified by RQ-PCR. MiRNA-124a levels six hours after heart surgery correlated with the neurological outcome of the patients. In children with neurological deficits, miRNA-124a levels increased while in those with no neurological deficits the levels decreased. MiRNA-124a was able, at six hours after the operation, to identify patients who are at risk for the appearance of neurological deficits. Circulating miRNA-124a is a potential biomarker for the appearance of neurological deficits in pediatric patients following heart surgery. Graphical Abstract.

Os Odontoideum in Children: Treatment Outcomes and Neurological Risk Factors.

Treatment outcomes and risk factors for neurological deficits in pediatric patients with an os odontoideum are unclear. We reviewed the data for 102 children with os odontoideum who were managed at 11 centers between 2000 and 2016 and had a minimum duration of follow-up of 2 years. Thirty-one children had nonoperative treatment, and 71 underwent instrumented posterior cervical spinal arthrodesis for the treatment of C1-C2 instability. Nonoperative treatment consisted of observation (n = 29) or immobilization with a cervical collar (n = 1) or halo body jacket (n = 1). Surgical treatment consisted of atlantoaxial (n = 50) or occipitocervical (n = 21) arthrodesis. One patient also underwent transoral odontoidectomy. Thirty children (29%) presented with neurological deficits, 28 of whom had radiographic atlantoaxial instability (atlantoaxial distance >5 mm) or limited space (≤13 mm) available for the spinal cord (risk ratio, 7.8 [95% confidence interval, 2.0 to 31] compared with children with no radiographic risk factors). The 27 children without neurological deficits or atlantoaxial instability at presentation underwent nonoperative treatment and remained asymptomatic. Of the initial nonoperative cohort, one child developed atlantoaxial instability, and another had a persistent neurological deficit; both children underwent spinal arthrodesis during the study period. One child with cervical instability declined surgery and remained asymptomatic. Spinal fusion occurred in 68 patients in the surgical group by the end of the study period (mean, 3.7 years; range, 2.0 to 11.8 years). Surgical complications occurred in 21 children, including nonunion in 12, new neurological deficits in 4, cerebrospinal fluid leak in 2, symptomatic instrumentation requiring removal 2, and vertebral artery injury in 1. Nine children underwent revision surgery. In the surgical group, Japanese Orthopaedic Association neurological function scores improved significantly from preoperatively to the latest follow-up for the upper extremities (p = 0.026) and lower extremities (p = 0.007). The risk of developing a neurological deficit was strongly associated with atlantoaxial instability and limited space available for the spinal cord in children with os odontoideum. Nonoperative treatment was safe for asymptomatic patients without atlantoaxial instability. Spinal arthrodesis resolved the neurological deficits of children with symptomatic os odontoideum. Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Brain Compression by Encephalo-Myo-Synangiosis is a Risk Factor for Transient Neurological Deficits After Surgical Revascularization in Pediatric Patients with Moyamoya Disease

In pediatric patients with moyamoya disease, the pathophysiology of transient neurological deficits and the clinical features of perioperative cerebral blood flow (CBF) changes are unclear. The purpose of this study was to investigate the risk factors of postoperative transient neurological deficits and identify predictors of perioperative CBF changes. This retrospective study included 42 surgical procedures in 28 pediatric patients who underwent surgical revascularization for moyamoya disease, including encephalo-myo-synangiosis (EMS) with or without superficial temporal artery-middle cerebral artery (MCA) anastomosis. Magnetic resonance images and single photon emission computed tomography results were obtained. Brain compression by EMS was also checked in fluid attenuated inversion recovery images. Using single photon emission computed tomography, CBF was measured at each anterior and posterior part of the MCA region. Postoperative transient neurological deficits were observed in 12 (28.6%) out of 42 surgical procedures. Brain compression by EMS was a significant risk for transient neurological deficits (P= 0.009). The postoperative CBF in the anterior region increased in 9 cases (21.4%) and decreased in 10 cases (23.8%); in the posterior region, it increased in 12 cases (28.6%) and decreased in 10 cases (23.8%). Preoperative CBF of the anterior region was significantly related to both perioperative CBF changes in the MCA regions (anterior part, P= 0.004; posterior part, P= 0.025). Brain compression by EMS is a risk factor for postoperative transient neurological deficits in pediatric patients with moyamoya disease, and preoperative CBF of the anterior MCA region could predict perioperative CBF change in the MCA regions.

Magnetic resonance imaging of changes in the brain of children cured of acute lymphoblastic leukemia.

This study was aimed to systematize magnetic resonance imaging (MRI) presentation of toxic leukoencephalopathy, to find the correlation between method of central nervous system (CNS) leukemia prevention and changes on MRI, to find relationship between existence leukoencephalopathy on imaging and neurocognitive deficits in pediatric patients after anti-leukemic therapy. Brain MRI data of 48 children, who underwent a therapy course according to the ALL-MB intermediate risk protocol, was evaluated. In accordance with two arms of this protocol, they received either radiation therapy, or additional intrathecal administration of chemotherapeutic agents as a prevention of CNS leukemia. Also, neurocognitive tests were performed. According to the results of the performed investigation, 10 (50%) out of 20 children, who received cranial irradiation and 18 (66.6%) out of 27 patients, who received only intrathecal therapy demonstrated abnormal brain changes (leukoencephalopathy) according to MRI data. Leukoencephalopathy was mostly presented by diffuse zones and localized predominantly in the frontal and temporal lobes. There was no correlation between method of CNS prevention and the existence of leukoencephalopathy on MRI. The analysis of our data did not show significant differences in brain damage and severity of cognitive impairment depending on the type of prevention of CNS leukemia. Moreover, in this study no statistical correlation was found between leukoencephalopathy on MRI and neurocognitive impairment according to clinical tests data. Further long-term prospective studies and examinations should be performed to assess late neurotoxic effects.

A-39 Predictors of Language Functioning Deficits in Pediatric Patients with Anti-NMDAR Encephalitis

Abstract Objective This study aimed to examine predictors of language functioning in a sample of pediatric patients diagnosed with anti-N-methyl-D-aspartate receptor encephalitis (ANMDARE). Method Patient data was collected through retrospective chart review within a children’s hospital. Pediatric patients (N=16) treated for ANMDARE (12% male; 25% Caucasian, 50% African American, 25% Latino, mean age = 12.3) who completed a neuropsychological evaluation were identified (time since diagnosis M = 177.4;SD = 310.2). Language assessments included Wechsler Abbreviated Scale of Intelligence-II (WASI-II) Vocabulary; Wide Range Achievement Test- 4 (WRAT-4) Word Reading; Delis-Kaplan Executive Function System (D-KEFS) Verbal Fluency. Results Two independent-samples t-tests compared language functioning in patients with normal and abnormal MRI findings. Significant differences were found in vocabulary scores between patients with normal (M = 45.57, SD = 5.50) and abnormal MRIs (M = 36.67, SD = 5.35;t(11) = 2.94,p = 0.013). The magnitude of the differences in means (μd = 8.91,95%CI:2.25-15.56) was medium (η2 = 0.44). A significant difference in semantic fluency scores were also found (Normal MRI; M = 10.67, SD = 2.25 vs abnormal MRI; M = 6.13;SD = 1.23; t(12) = 3.89,p = 0.002). The magnitude of the differences in means (μd = 4.54, 95%CI:2.00- 7.09) was medium (η2 = 0.56). Additional analyses revealed a relationship between seizure presentation and vocabulary scores (t(11) = 2.50;p = 0.029). Conclusions Pediatric patients with abnormal MRIs demonstrate greater deficits in vocabulary and semantic fluency compared to those with normal MRIs. Moreover, patients who presented with seizures were more likely to have deficits in vocabulary. Previous research shows variability in prolonged deficits in language functioning in both children and adults. To the best of our knowledge, this is the first study to compare symptom presentation with language outcomes, thus potentially contributing to the clinical utility of MRI scans.

P026 RELATIONS BETWEEN PHYSICAL ACTIVITY, DIET, AND BODY COMPOSITION IN PEDIATRIC PATIENTS WITH INFLAMMATORY BOWEL DISEASE

The presence of lean mass (LM) deficits in pediatric patients with IBD is well established, and these deficits persist despite achievement of remission and restoration of body mass. LM deficits are associated with negative health outcomes including decreased physical function, metabolic dysregulation, increased risk of infection, and reduction of bone mass. These deficits are multifactorial though largely explained by chronic malnutrition due to enteric nutrient losses, inadequate dietary intake, malabsorption of nutrients, and increased energy needs. In addition to malnutrition, physical activity (PA) may also play a role in the low LM and myopenia noted in youth with IBD. Unfortunately, PA and dietary intake have been relatively understudied in this population. As such, the present cross-sectional pilot study aims to describe relations between PA, dietary intake, and body composition in a sample of pediatric patients with IBD compared to healthy peers. 87 patients aged 8-17 years completed the study (31 Crohn’s Disease (CD), 23 Ulcerative Colitis (UC), and 31 Healthy Controls (HC) completed the study protocol. Assessment measures included: PA (Godin Leisure Time Exercise Questionnaire), 4-Day Food Records, Body Composition and Bone Density (Dual-energy X-ray Absorptiometry; DXA). Medical record reviews were conducted for IBD patients only. Analyses included one-way ANOVAs to evaluate differences between study groups and two-tailed Pearson correlations to evaluate relations between variables of interest across groups. Healthy controls had significantly greater moderate to vigorous physical activity compared to both IBD groups and were significantly younger than and consumed a higher percentage of calories from fat compared to youth with UC. Youth with UC consumed a significantly higher percentage of calories from carbohydrates compared to HC but not CD participants. No other statistically significant differences were found based on the study group. Correlational analyses (N = 87) revealed that being male (r =.-.30), older age (r =.72), higher total energy intake (r =.22), a higher percentage of calories from protein (r =.37), and higher BMI z-score (r =.51) were associated with higher total body LM. While physical activity was not related to LM, it is possible that differences would be noted if activities were coded as resistance versus aerobic exercise as these have been shown to have differential effects on LM. Understanding health behaviors associated with the development of LM, including PA and dietary intake, are key to improving growth and development outcomes in pediatric patients with IBD. The present study suggests the need for further research into PA and macronutrient intake as a means of promoting short and long-term health and well-being for pediatric patients with IBD.

The effect of cerebellar tumor resection on pain perception in pediatric patients.

126 Background: Acute cerebellar infarctions have been shown to lead to affective and cognitive deficits in pediatric patients. However, the consequences of cerebellar insult on pain processing are unknown. Recent evidence not only suggests that the cerebellum plays a role in affective processing, but it also demonstrates increased activity in this area when individuals experience experimental or clinical pain, particularly in the posterior cerebellum. The goal of this study is to evaluate the effect of cerebellar resection of low-grade astrocytomas in children on pain perception. Describing the impact of this treatment may be of particular importance in children, as it may alter sensory development into adulthood. Methods: Twelve pediatric patients treated with surgery only for non-malignant astrocytomas (mean age = 13.78 ± 5.64) and ten age-, gender-, race- and handedness-matched healthy controls (mean age = 14.52 ± 5.96) were evaluated using quantitative sensory testing and magnetic resonance imaging, comprising diffusion tensor imaging, resting state and event-related functional MRI scans. Five of these patients had tumors localized to the posterior cerebellar hemispheres. The psychophysical measures assessed included heat and cold detection thresholds (HDT, CDT), heat and cold pain thresholds (HPT, CPT), and cold pain tolerance using a cold pressor task. Results: Patients with lesions in the posterior cerebellum (n = 5) were shown to have a significantly lower tolerance to the cold pressor test than controls (n = 10). No significant differences were detected for the threshold measures. This suggests increased pain sensitivity in these patients to suprathreshold noxious stimuli compared to controls. Patients with lesions that did not include the posterior cerebellar hemispheres (n = 7) did not show significant differences with controls in terms of cold pain tolerance or thresholds. Conclusions: The differences in cold pain tolerance between patients and controls are presumed to result from damage to the posterior cerebellar hemispheres, potentially interfering with the modulation of pain pathways. Analysis of the preliminary imaging data is underway and recruitment is ongoing. Support: NIH/R21CA185870(EM)

Intraoperative neurophysiological monitoring during endoscopic endonasal surgery for pediatric skull base tumors

OBJECT The aim of this study was to evaluate the value of intraoperative neurophysiological monitoring (IONM) using electromyography (EMG), brainstem auditory evoked potentials (BAEPs), and somatosensory evoked potentials (SSEPs) to predict and/or prevent postoperative neurological deficits in pediatric patients undergoing endoscopic endonasal surgery (EES) for skull base tumors. METHODS All consecutive pediatric patients with skull base tumors who underwent EES with at least 1 modality of IONM (BAEP, SSEP, and/or EMG) at our institution between 1999 and 2013 were retrospectively reviewed. Staged procedures and repeat procedures were identified and analyzed separately. To evaluate the diagnostic accuracy of significant free-run EMG activity, the prevalence of cranial nerve (CN) deficits and the sensitivity, specificity, and positive and negative predictive values were calculated. RESULTS A total of 129 patients underwent 159 procedures; 6 patients had a total of 9 CN deficits. The incidences of CN deficits based on the total number of nerves monitored in the groups with and without significant free-run EMG activity were 9% and 1.5%, respectively. The incidences of CN deficits in the groups with 1 staged and more than 1 staged EES were 1.5% and 29%, respectively. The sensitivity, specificity, and negative predictive values (with 95% confidence intervals) of significant EMG to detect CN deficits in repeat procedures were 0.55 (0.22-0.84), 0.86 (0.79-0.9), and 0.97 (0.92-0.99), respectively. Two patients had significant changes in their BAEPs that were reversible with an increase in mean arterial pressure. CONCLUSIONS IONM can be applied effectively and reliably during EES in children. EMG monitoring is specific for detecting CN deficits and can be an effective guide for dissecting these procedures. Triggered EMG should be elicited intraoperatively to check the integrity of the CNs during and after tumor resection. Given the anatomical complexity of pediatric EES and the unique challenges encountered, multimodal IONM can be a valuable adjunct to these procedures.

The Cambridge Automated Neuropsychological Testing Automated Battery (CANTAB) Is Feasible and Valuable for the Evaluation of Neurocognitive Deficits in Pediatric Patients with Sickle Cell Disease: Results of a Pilot Study

Abstract 4839 Background:As children with sickle cell disease (SCD) are at significant risk for neurocognitive complications, an automated and objective measure of neurocognitive functioning would address several challenges facing both clinical and research progress in SCD including longitudinal monitoring of deficits, cross-site comparability of neurocognitive tests in multicenter trials, and limited access to pediatric neuropsychologists. The Cambridge Automated Neuropsychological Testing Automated Battery (CANTAB) is a well-validated computerized test with significant normative data in individuals age 4 to 80 that has been used to monitor disease progression and treatment response in children and adults with a range of disorders but has not been used previously in SCD. Hypothesis:We hypothesize that the CANTAB system is a useful and viable tool for the neurocognitive evaluation of pediatric patients with SCD. We expect that the CANTAB testing will be well tolerated by SCD patients and parents, easy to administer in our comprehensive clinic, and generate valid results that correlate with both medical and psychological outcomes. Methods:7 CANTAB tests which assess attention, executive function and memory were run on pediatric SCD patients during scheduled clinic visits. Parents completed the child behavior checklist (CBCL) which generates t-scores for children on internalizing, externalizing scales as well as DSM-oriented scales of affective, anxiety, pervasive developmental, attention and oppositional scales. Medical data including SCD genotype, average hemoglobin (hgb), hematocrit (hct), reticulocyte count (rct), lactate dehydrogenase and hospital utilization records (ER visits, # hospital visits in the last year) was collected. Demographic information and a total pain burden assessment were also collected. Results:11 children with HbSS SCD were enrolled in the pilot study (table 1). All patients successfully completed the CANTAB testing without difficulty. Hgb and rct were associated with strategy score on spatial working memory and the latency score on the motor screening task. Hgb and rct also correlated with internalizing, externalizing, and total symptoms scores on the CBCL (table 2). Specifically lower hgb and higher rct were associated with increased CBCL scores. A regression model incorporating average hgb and total internalizing scores with spatial working memory as the dependent variable revealed a significant interaction between internalizing scores and hgb and a significant model p=.01 and r2 of 0.89 offering preliminary support for a multi-level model incorporating disease and child specific factors (table 2).Table 1N=11Age (sd)HgbHctRctER visitsPainCBCL totalSWM strategySOC mean moves8.5(4.0)8.8 (.59)26.3(2.2)9.7(5.9)3.4(4.6)6(3.6)50.7 (12.5)19.5 (1.5)2.46 (.57)Table 2Lab valueInternalizing-CBCLExternalizing CBCLTotal CBCLSwm strategyMot latencyHgbP=.01P=.02P=.0019P=.01P=.12HctP=.01P=.03P=.002P=.01P=.06RctP=.06P=.08P=.07P=.09P=.04The total pain burden score correlated error making in several tests including the delayed match to sample test (p=.01), spatial working memory test (p=.06), and Stockings of Cambridge task (p=.0038). The pain burden score was not associated with performance or latency on these tests indicating that pain burden may have a specific association with error making. Pain burden also correlated with the somatic measure on the CBCL (p=.01) indicating cross validation between the two measures. Conclusion:This pilot study demonstrates the feasibility and value of the CANTAB system in evaluating neurocognitive deficits in pediatric patients with SCD. These results can be assessed longitudinally following medical interventions. Furthermore, results indicate a multi-level model that includes medical factors, child specific factors, and demographics may be a more appropriate model to utilize in determining the etiology of neurocognitive deficits in SCD. Ongoing studies with an increased sample size will examine the association of neurocognitive function with SCD genotype, MRI, transcranial doppler studies, and family stress.SWM: Spatial working memory SOC: Stockings of Cambridge MOT: Motor Screening Test Disclosures:No relevant conflicts of interest to declare.

Bone mineral density deficits in pediatric patients treated for sarcoma

Children treated for sarcoma are at risk of treatment-associated deficits in bone mineral density (BMD). We investigated the severity of risk factors for BMD deficits in this patient population. Using signed-rank test and logistic regression analysis, we retrospectively analyzed the relation of treatment variables and other potential risk factors to BMD (using quantitative computed tomography (QCT)) of 99 patients treated for pediatric sarcoma who had completed therapy at least 1 year previously. The study group (38% rhabdomyosarcoma (RMS), 25% osteosarcoma (OS), 24% Ewing-family tumors, and 12% non-rhabdomyosarcoma soft-tissue sarcoma (NRSTS)) represented 22% of the sarcoma survivors treated between 1982 and 2003 who remained in follow-up at St. Jude. These patients underwent QCT between July 1, 1997 and February 5, 2003. Their median age was 8.7 years (range, 0.2-21.3 years) at diagnosis and 17.4 years (range, 3.3-30.2 years) at the time of BMD measurement; 58% were male and 82% Caucasian. Median BMD Z-score was -0.75 (range, -3.33-3.02), and median BMD was 168.0 mg/cc (range, 89.2-264.8 mg/cc). Risk of BMD deficit increased significantly with younger age at diagnosis (P = 0.044) and higher cumulative cyclophosphamide dose (P = 0.007). Patients with lower extremity primary disease had a significantly lower risk of BMD deficits than others. We found no association between BMD and body habitus, primary disease, lifestyle factors, or endocrinopathy. A significant subset of sarcoma survivors are at risk of BMD deficits warranting prospective study of BMD to verify our results and refine risk factors contributing to BMD deficits.

186. Comparison of neuropsychological deficits in pediatric patients with childhood-onset schizophrenia and “multidimensionally impaired syndrome”

186. Comparison of neuropsychological deficits in pediatric patients with childhood-onset schizophrenia and “multidimensionally impaired syndrome”