Childrens Hospital Los Angeles Research Articles

OTHR-09. A RETROSPECTIVE COHORT STUDY EXAMINING RADIOLOGIC AND CLINICAL CHARACTERISTICS OF CHILDREN AND ADOLESCENTS WITH ATYPICAL/COMPLEX PINEAL CYSTS

Abstract INTRODUCTION The significance of atypical or complex pineal cysts (CPC) on magnetic resonance imaging (MRI) is often unclear, and the appropriate management has not been well defined. We conducted a retrospective cohort study of children and adolescents (0-18 years of age) with complex pineal cysts noted on brain MRIs obtained at Children’s Hospital Los Angeles (CHLA) prior to 05/31/2022. METHODS We retrospectively reviewed electronic medical records at CHLA to identify patients <18 years of age who were diagnosed with CPC upon MRI between January 1, 2010 and May 31, 2022. CPC were defined as cysts that are multi-cystic, enhancing, ≥1 cm in size, have a solid component, or exhibit restricted diffusion. Patients were identified by an automated Boolean search of a radiology report database; individual reports were then reviewed for eligibility. Patients with normal pineal glands or simple (<1 cm) pineal cysts were excluded. Electronic medical records were evaluated for patient chief complaint, initial clinical and radiographic symptoms, tumor markers (beta-human chorionic gonadotropin and alpha-fetoprotein), and final diagnosis. RESULTS We identified a cohort of 175 patients; 106 (61%) were female. Mean age at initial MRI was 8.36 years of age (SD 5.36). Tumor markers were measured in 52 patients (30%). The most common presenting symptoms for patients were headache (34%), seizures (26%), nausea/vomiting (19%), weakness/fatigue (17%), and dizziness (12%). Additional presenting symptoms included ataxia, sensory changes, and anxiety. Pineal malignancies were identified in 4 patients (2.2%), which consisted of a pituitary pure germinoma, a pineal region parenchymal tumor, and two pineoblastomas. The remaining 98% of patients (171 patients) either had a benign pineal cyst or were categorized as unknown. CONCLUSION Most CPC are not associated with malignant neoplasms and represent incidental findings with scant clinical significance.

30: SOCIAL VULNERABILITY INDEX AND CHILD OPPORTUNITY INDEX HIGH IN ASTHMA BUT NOT LINKED TO PICU STAY

Introduction: Racial and socioeconomic disparities in pediatric asthma prevalence, severity, and use of primary and emergency care have been well studied. However, understanding of the impact of these disparities on risk of hospitalization in the pediatric intensive care unit (PICU) is limited. We used composite measures of socioeconomic risk factors through the Centers for Disease Control and Prevention (CDC) Social Vulnerability Index (SVI), and the Child Opportunity Index (COI) 2.0 to compare patients admitted for status asthmaticus to the PICU or pediatric ward at Children’s Hospital Los Angeles (CHLA). We hypothesized patients with a high SVI and low COI are at higher risk for PICU level care. Methods: Following IRB approval, patients were identified using ICD-10 codes for asthma. The outcome of interest was need for PICU versus ward care. Patients were included as requiring PICU level of care if admitted to the PICU during their stay. Patient registered street address was geocoded and linked to US Census-American Community Survey 2019 Data (5-Year) at the census tract level. Bivariate analysis with the patient’s SVI, COI, hospital length of stay (LOS), and PICU admission were conducted. Statistical analysis used SAS, STATA, and ArcGIS Pro. Results: From [October 2015 – May 2022], there were 4,766 admissions matched to addresses from 3,617 distinct patients of which 626 (13.1%) required PICU admission. Overall median SVI for all patients was 0.86 with no difference between groups for SVI: median (1,3 IQR) PICU 0.86 (0.67, 0.93) Ward 0.86 (0.69, 0.93) p=0.56; or for COI: PICU 27 (9, 50) Ward 24 (10, 50) p=0.82. Hospital LOS was significantly longer for those requiring PICU admission: PICU 3 (3, 5) Ward 2 (2, 3) (days) p< 0.001. Conclusions: We found that children admitted to CHLA for asthma had an elevated SVI overall reflecting a patient population among the lowest socioeconomic status in the United States. The median 0.86 SVI score indicates a population that is more socially vulnerable than all but 14% of the US population. Similarly, the COI score demonstrated a population at risk. Yet, there was no difference between groups for the SVI or COI scores indicating we care for children at increased socioeconomic risk, but this did not increase PICU use for asthma admissions.

VP Shunt With Recurrent Malfunction in Two Pediatric Patients: Is the Hydrocephalus Truly Idiopathic?

VP Shunt With Recurrent Malfunction in Two Pediatric Patients: Is the Hydrocephalus Truly Idiopathic?

Factors Associated With Delay in Cleft Surgery at a Tertiary Children's Hospital in a Major US Metropolitan City.

To identify factors associated with late cleft repair at a US tertiary children's hospital. Retrospective study of children with CL/P using Children's Hospital Los Angeles (CHLA) records. US tertiary children's hospital. Patients undergoing primary CL or CP repair at CHLA from 2009 to 2018. Proportion of children who had delayed primary CL repair or CP repair using CHLA and American Cleft Palate-Craniofacial Association (ACPA) guidelines and factors associated with late surgery. In total, 805 patients-503 (62.5%) who had CL repair, 302 (37.5%) CP repair-were included. Using CHLA protocol, 14.3% of patients seeking CL repair had delayed surgery. Delay was significantly associated with female gender, non-Hispanic ethnicity, Spanish primary language, government insurance, bilateral cleft, cleft lip and palate (CLP), and syndromic diagnosis. Using ACPA guidelines, 5.4% had delayed surgery. Female gender and syndromic diagnosis were significantly associated with delay and remained significant after adjustment for confounders in multivariate models. For CP repair, 60.3% of patients had delayed surgery using CHLA protocol. Cleft lip and palate diagnosis, complete cleft, syndromic diagnosis, and longer travel distance were significantly associated with delay. Using ACPA guidelines, 28.5% had delayed surgery; however, significant association with patient variables was not consistently observed. Delay in cleft surgery occurs most often for patients seeking CP repair and is associated with female gender, non-Hispanic ethnicity, Spanish language, government insurance, and bilateral CL, CLP, or syndromic diagnoses. Initiatives should aim to optimize cleft surgery delivery for these subpopulations.

2241-PUB: The Effectiveness of Individualized Exercise Programs in a Multidisciplinary Pediatric Clinic for Patients with Type 2 Diabetes

Background/Purpose: The American Diabetes Association projects the incidence of type 2 diabetes (T2DM) among pediatric patients will increase to 49% by 2050. Physical activity and exercise modifications are primary recommendations in controlling diabetes. The purpose of this study is to describe the effects of a physical therapist (PT) prescribed personalized fitness plan to improve cardiovascular fitness in adolescents with T2DM. Methods: Data was collected from patients treated at a multidisciplinary T2DM clinic at Children’s Hospital Los Angeles (CHLA). Physical therapy assessments were done when the patient visited the PT in clinic and included post-exercise heart rate, resting heart rate, rating of perceived exertion, and VO2 max. Multiple regression analysis was used to assess associations between change in fitness and covariates: age, sex, time since diagnosis, and time between first and last PT assessment. Results: Fifty two patients who had at least 2 PT assessments at the T2DM clinic from April 2016 to October 2018 were included in the study. Patients with higher initial post-exercise heart rate experienced the greatest benefit from an individualized exercise program. Patients with lower initial VO2 max scores also experienced the greatest increase in VO2 max over time. There was no significant association with resting heart rate and rating of perceived exertion in regards to age, sex, time since diabetes diagnosis, and time between first and last PT assessment. Conclusion: Prescribing a personalized fitness plan can improve fitness measures in patients with the lowest baseline fitness levels. Implications: Maintaining a healthy and active lifestyle can be difficult for youth with T2DM especially if they lived a sedentary lifestyle prior to diagnosis. Health care providers and educators must consider the age and interests of the patient and family when creating a personalized fitness program. Disclosure L.B. Europa: None. L.N. Buennagel: None. Funding Good Hope Medical Foundation

Transition to Practice: Onboarding Components for Establishing and Sustaining Healthy Work Environments.

Transition to Practice: Onboarding Components for Establishing and Sustaining Healthy Work Environments.

Staffing Challenges Today.

Staffing Challenges Today.

Changes in Brain Oxygenation in Response to Inhaled 100% Oxygen Are Different in Sickle Cell Disease Patients

Introduction: The prevalence of silent stroke increases monotonically with age in SCD patients, with more than 50% developing detectable white matter hyperintensities on MRI by adulthood. We are exploring the use of inhaled oxygen as a contrast agent to try to identify individuals at risk for silent stroke and to better understand how the brain regulates its own oxygen delivery. In this study, we report changes of brain Blood Oxygen Level Dependent (BOLD) signal and frontal Near Infrared Spectroscopy (NIRs) to 5 minutes of inhaled 100% oxygen in patients with sickle cell disease (SCD), non sickle anemia (ACTL), and control subjects.Method: The study was approved by the institutional review board and performed at Children’s Hospital Los Angeles (CHLA); all patients provided informed consent. Patients with previous stroke, pregnancy, and acute chest pain and crisis hospitalization within one month were excluded. 19 SCD patients (age = 20.7 ± 8.8), 12 patients with non sickle anemia syndromes (age = 25.1 ± 6.9) and 20 ethnicity matched control (CTL) subjects (age = 30.5 ± 12.7) were studied. Peripheral oxygen saturation (SpO2%) was measured by MRI compatible pulse oximeter. A five minute BOLD sequence was acquired with the patient breathing room air for 50 seconds, followed by 100% oxygen for 250 seconds. NIRS recordings of oxygenated hemoglobin change (DOHB), deoxygenated hemoglobin change (DDHB) and tissue oxygenation index (TOI) were recorded from the patient’s forehead. Average BOLD signal was from a 6 cm slab in the middle of the brain. CBC, hemoglobin electrophoresis, and markers of hemolysis were also measured.Results: A representative frontal DOHB signal, and the parameters used to characterize its time-course, are shown in Figure 1. DOHB remained stable until oxygen was administered and then increased over several minutes to a maximum. In many patients, the DOHB signal subsequently declined back toward baseline, representing the effects of compensatory cerebral vasoconstriction. The delay time was shorter in anemic subjects because of increased cerebral blood flow, but the rise times were not different. Peak DOHB increase was greater in SCD patients than in ACTL or non anemic control subjects (1.4 mM ± 0.2mM, 0.8 mM ± 0.2 mM and 0.6 mM ± 0.4 mM, respectively, p <0.0001 by ANOVA). There was no difference in the DOHB vasoconstriction response among the three patient groups.DOHB was highly correlated with DDHB and DTOI in all three patient groups. Consequently, both DDHB and DTOI changes were also larger in SCD patients. All three NIRs signal changes were larger in non-transfused SCD patients than in patients on chronic transfusions. Non-transfused SCD patients had more significant increases in SpO2% than chronically transfused subjects and the effect was proportional to hemoglobin F%, suggesting a p50 effect. After controlling for SpO2%, SCD patients still exhibited larger NIRs changes than ACTL or CTL patients, but the differences between transfused and non transfused SCD patients disappeared.Whole brain %BOLD was also correlated with changes in DOHB, DDHB, TOI, and SpO2%. The relationship between DOHB versus %BOLD is illustrated in Figure 2. %BOLD changes were also significantly higher in SCD patients. However, unlike the NIRS changes, whole brain %BOLD differences could entirely be explained by differences in SpO2% with hyperoxia.Conclusion:100% oxygen administration increases brain oxygenation in SCD patients to a greater degree than in control subjects or patients with anemia of other etiologies. Some of the differences can be explained by differences in resting oxygen saturations but disparities between BOLD and NIRs response suggest regional variation of brain oxygenation in response to this challenge. [Display omitted] [Display omitted] DisclosuresWood:Apopharma: Consultancy; AMAG: Consultancy; World Care Clinical: Consultancy; AMAG: Consultancy; Celgene: Consultancy; Apopharma: Consultancy; Biomed Informatics: Consultancy; Celgene: Consultancy; Ionis Pharmaceuticals: Consultancy; Vifor: Consultancy; Vifor: Consultancy; Ionis Pharmaceuticals: Consultancy; Biomed Informatics: Consultancy; World Care Clinical: Consultancy.

LG-16RADIOGRAPHIC AND OPHTHALMOLOGIC CHARACTERISTICS OF OPTIC PATHWAY GLIOMA IN CHILDREN WITH NEUROFIBROMATOSIS 1

LG-16. RADIOGRAPHIC AND OPHTHALMOLOGIC CHARACTERISTICS OF OPTIC PATHWAY GLIOMA IN CHILDREN WITH NEUROFIBROMATOSIS 1 Elizabeth Unterbrink1, Benita Tamrazi1,2, Mark Borchert1,2, Ashley Margol1,2, Linda Randolph1,2, Tena Rosser1,2, Girish Dhall1,2, and Nathan Robison1,2; Children’s Hospital Los Angeles, Los Angeles, CA, USA; University of Southern California Keck School of Medicine, Los Angeles, CA, USA OBJECTIVE: To describe the natural history of optic pathway glioma (OPG) in children with neurofibromatosis 1 (NF1). METHODS: The Children’s Hospital Los Angeles (CHLA) institutional review board approved this retrospective study. Using a clinical database, we identified children with NF1 and OPG followed at CHLAwith at least 1 MRI obtained at ,12 years of age from 6/1/1997-5/31/2014. Demographic, treatment, and visual field/ acuity data were extracted. MRIs were reviewed by a neuroradiologist. RESULTS: Of 63 children with NF1 and OPG, 50 had available imaging and were included. Mean age at initial MRI was 4.8 years; mean follow-up was 6.8 years. OPG was apparent on brain MRI in 4/7 children at ,2 years (57%; 95% confidence interval[95%CI] 20-94); 19/23 by 4 years (83%; 95%CI 67-98); 29/31 by 6 years (94%; 95%CI 85-100); and 50/50 by 12 years. Among 13 children (26%) who subsequently received tumor-directed treatment, OPG was MRI-apparent in 5/6 children by 4 years (83%; 95%CI 54-100), 8/9 by 6 years (89%; 95%CI 68-100) and 13/13 by 8 years. Among children with detailed visual outcome data, visual impairment was seen in 6/20 at , 4 years (30%; 95%CI 10-50); and 26/45 by 12 years (58%; 95%CI 43-72). A subset had normalization of vision, with persistent visual abnormalities in only 14/41 children on follow-up (34%; 95%CI 20-49). DISCUSSION: A minority of children without MRI-apparent OPG at initial evaluation eventually developed symptomatic disease. The high incidence of early-onset visual abnormalities highlights the importance of early NF1 diagnosis and ophthalmologic screening. Neuro-Oncology 18:iii78–iii96, 2016. doi:10.1093/neuonc/now075.16 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

FRI0353 Infliximab vs cyclophosphamide in pediatric takayasu’s arteritis

BackgroundPediatric Takayasu’s Arteritis (pTA) is a rare granulomatous vasculitis that affects the aorta and its branches. Though cyclophosphamide (CYC) is often used as first line therapy there are few studies...

Improving Home Management Plan of Care Compliance Rates Through an Electronic Asthma Action Plan

Background. In 2007, the Joint Commission mandated reporting of three children’s asthma care (CAC) measures for hospitalized patients with asthma. The third children’s asthma care measure (CAC-3) focuses on hospital discharge with a comprehensive home management plan of care (HMPC) based on the clinical severity. Objective. To improve CAC-3 compliance and identify what interventions would have the most impact. Methods. This was a retrospective observational study, conducted at the Children’s Hospital Los Angeles (CHLA) between October 2008 and January 2012. A total of 470 patients admitted with a primary diagnosis of asthma were included. Four Plan-Do-Study-Act cycles testing separate interventions were used throughout the study period: clinical care coordinators (CCCs), red clipboard for paper HMPC, electronic HMPC, and hard-stop HMPC. Chi-square and binomial tests compared CHLA’s CAC-3 compliance rates within intervention windows as well as to the national average. Results. Between October 2008 and May 2009, CHLA had a compliance rate of 39%, well below the national average (p = .001). Involvement of CCCs increased the overall compliance to 74% (χ2(1) = 11.59, p < .001). Implementation of an electronic HMPC in October 2010 led to the largest increase in overall compliance (93%) when compared to the previous intervention window (χ2(1) = 4.38, p < .036), as well as the national average (p = .016). Compliance rates remained above 90% for four out of the following five quarters. Conclusions.Involvement of CCCs led to a significant increase in the overall CAC-3 compliance. An electronic HMPC improved rates well above the national average. This provides a framework for other institutions that may or may not utilize an electronic medical record.

Protocols for Late Maxillary Protraction in Cleft Lip and Palate Patients at Childrens Hospital, Los Angeles

This paper describes the protocols used at Childrens Hospital Los Angeles (CHLA) to protract the maxilla during early adolescence. It is a modification of techniques introduced by Eric Liou with his Alternate Rapid Maxillary Expansion and Constriction (ALT-RAMEC) technique. The main differences between the CHLA protocol and previous maxillary protraction protocols are the age the protraction is attempted, the sutural loosening by alternating weekly expansion with constriction and the use of Class III elastics to support and redirect the protraction by nightly facemask wear. The CHLA protocol entirely depends on patient compliance and must be carefully taught and monitored. In a cooperative patient, the technique can correct a Class III malocclusion that previously would have been treated with LeFort 1 maxillary advancement surgery. Thus, it is not appropriate for patients requiring 2 jaw surgeries to correct mandibular prognathism, occlusal cants or facial asymmetry. The maxillary protraction appears to work by a combination of skeletal advancement, dental compensation and rotation of the occlusal planes. Microscrew/microimplant/temporary anchorage devices have been used with these maxillary protraction protocols to assist in expanding the maxilla, increasing skeletal anchorage during protraction, limiting dental compensations and reducing skeletal relapse.

Improving Outcomes Through Quality Improvement

The nutrition quality improvement (QI) program at the Cystic Fibrosis (CF) Center of Childrens Hospital Los Angeles (CHLA)aimed to increase patient body mass index (BMI) through improved clinician (physician, dietitian, and social worker) adherence to nutrition recommendations set forth by the Cystic Fibrosis Foundation (CFF). The QI program used a combination of strategies intended to increase cognitive cues for desired behaviors and to promote environmental change intended to prompt clinicians to adopt nutrition treatment recommendations. Program analysis involved collection of data on clinician behavior, nutrition treatment plans, and patient BMI. Evaluation involved a pretest—posttest design with multiple observations to determine behavior maintenance throughout the 18-month study period. Results indicate that the program produced changes in clinician behavior as evidenced by increased rates of adherence to nutrition recommendations, including the use of BMI/age as the primary nutrition marker and recommending aggressive nutrition treatment, though with noted attrition by the final data collection period. A clear trend toward improvement in BMI/age was evident with statistical significance at the P &lt; .05 level. The QI program demonstrated that simple behavior cues in combination with training can produce changes in clinician behavior, which in turn affects patient outcomes.

Feasibility of airway pressure release ventilation in infants with near end stage- neonatal bronchopulmonary dysplasia

Background: Ventilated premature infants with severe, advanced bronchopulmonary dysplasia (BPD) often require high mean airway pressure (MAP) and FiO2 to maintain acceptable gas exchange using conventional mechanical ventilation techniques such as synchronized intermittent mandatory ventilation (SIMV). In addition, they are at risk to develop ep isodes of severe respiratory failure (BPD spells). We report our experience with airway pressure release ventilation (APRV), a ventilation modality which allows spontaneous respiration during a relatively long high pressure phase followed by a shorter release period allowing for CO2 removal from the conducting airways. Despite the higher MAP, the unique APRV flow pattern is thought to decrease volutrauma. Objective: To determine the feasibility and safety of APRV in neonates with severe BPD. Design/methods: Approval for this retrospective review was obtained from Childrens Hospital Los Angeles (CHLA) Institutional Review Board. Patients with advanced BPD treated in the Newborn and Infant Critical Care Unit at CHLA that received APRV at the discretion of the attending neonatologist for develo ping severe respiratory failure between May 2004 and June 2005 were identified and their medical records reviewed. Results: Nine patients were placed on APRV using the AVEA ventilator (CareFusion, San Diego, CA). Eight infants were former extremely premature infants with severe BPD and 1 infant was born at term infant with suspected surfactant protein-B defi ciency. Mean gestational and postmenstrual age was 26.7± 4.9 and 50.5± 9.8 weeks, respectively at the time of initiation of APRV. The duration of APRV was 17.5± 11.9 days. On APRV, patients received higher MAP (29 vs. 15 cm H2O; p < 0.001) and lower FiO2 (0.5 vs. 0.3; p = 0.38) compared to SIMV with pressure support, high-frequency oscillatory ventilation or high-frequency jet ventilation. Seven of the 9 patients had clinical improv ement on APRV within 3 days defined by fewer episodes of severe desaturation, variability in FiO2, and vasopressor/inotrope support. In addition, none of th e infants received pharmacological neuromuscular blockade after the initiation of APRV. Despite the higher MAP, patients did not develop air-leaks and remained hemodynamically stable with normal blood pressure and urine output. Conclusions: These preliminary data indicate that infants with severe BPD tolerate the use of APRV without apparent adverse effects. However, it remains unclear whether APRV is indeed safe and beneficial in former very preterm neonates with seve re BDP and episodes of respiratory failure. Randomized controlled studies are required to determine the safety, efficacy and l ong-term morbidities of this mode of ventilation in neonates.

Advances in Magnetic Resonance Imaging of the Injured Neonatal Brain

&lt;P&gt;In spite of improvement in neonatal medicine, neonatal encephalopathy and resulting neonatal brain injury is a major complication that can cause long-term disability. Multiple neuroscience studies have determined potential mechanisms that underlie the vulnerability of the neonatal brain to multiple insults. In the near future, there may be new therapeutic options for both prevention and treatment of neonatal brain injury. Most pediatric centers currently use cranial ultrasound to evaluate neonatal brain injury. In the past decade, magnetic resonance imaging (MRI) techniques have been shown to be a useful additive imaging tool in the evaluation of neonatal brain injury. Conventional MRI techniques have been shown not only to provide valuable information about the pattern of brain injury in the neonatal period but also to provide information about prognosis and neurodevelopment outcome. There have been recent advances in MRI of neonatal encephalopathy with respect to the development of MR-compatible incubators. These incubators can provide an environment specifically adapted to meet the needs of critically ill preterm and term neonates when undergoing MRI. In addition, imaging resolution and signal-to-noise ratio (SNR) have been improved dramatically with the use of specifically designed neonatal coils. MR diffusion (both diffusion-weighted imaging and diffusion-tensor imaging) and MR spectroscopy are recently developed techniques that are now used routinely. The goals of this article are to provide information about the technological MR advances in the evaluation of the injured neonatal brain and to review clinical examples, which are in the major differential diagnoses of neonatal brain injury including perinatal white matter injury, hypoxic-ischemic brain injury, metabolic disease, and hemorrhage.&lt;/P&gt; &lt;H4&gt;ABOUT THE AUTHORS&lt;/H4&gt; &lt;P&gt;Ashok Panigrahy, MD; and Stefan Blüml, PhD, are with the Department of Radiology, Division of Neuroradiology, Institute for Maternal Fetal Health, Childrens Hospital Los Angeles (CHLA), Keck School of Medicine of USC, Los Angeles, California.&lt;/P&gt; &lt;P&gt;Address correspondence to: Ashok Panigrahy, MD, Childrens Hospital Los Angeles, Department of Radiology, Mail Stop #81, 4650 Sunset Blvd., Los Angeles, CA 90027; or e-mail &lt;a href="mailto:apanigrahy@chla.usc.edu"&gt;apanigrahy@chla.usc.edu&lt;/a&gt;.&lt;/P&gt; &lt;P&gt;Dr. Panigrahy and Dr. Bluml have disclosed no relevant financial relationships.&lt;/P&gt; &lt;P&gt;The authors thank Marvin D. Nelson, MD; Istvan Seri, MD; Floyd Gilles, MD; and the Neonatal Intensive Care Unit (NICU) and Magnetic Resonance Imaging (MRI) staff at CHLA for support and advice. The authors thank Julia Cruz and Madalyn Shrier for editorial support. Grant support is from the Radiological Society of North America (RSNA), Rudi Schulte Research Institute, and National Institutes of Health (NIH) pediatric research loan repayment grant.&lt;/P&gt; &lt;H4&gt;EDUCATIONAL OBJECTIVES&lt;/H4&gt; &lt;OL&gt; &lt;LI&gt;Identify recent technological advances in the evaluation of the injured neonatal brain using magnetic resonance imaging (MRI).&lt;/LI&gt; &lt;LI&gt;Propose clinical examples of neonatal brain injury as they are delineated via MRI.&lt;/LI&gt; &lt;LI&gt;Differentiate the advantages of MRI evaluation of neonatal brain injury over the current standard of ultrasound imaging.&lt;/LI&gt; &lt;/OL&gt;

Onset of Cardiac Iron Loading in Pediatric Patients.

Patients with thalassemia major develop life threatening cardiac complications in their teens and twenties from iron overload. Cardiac MRI allows us to diagnosis preclinical cardiac iron deposition, but it is not known at what age T2* screening should be initiated. Historical data and pilot MRI studies suggest that patients must reach a critical transfusional exposure prior to cardiac iron uptake. This study was a two-institution characterization of the prevalence of cardiac iron overload in 77 pediatric patients.Methods: Study was performed at the Ospedale Regionale Microcitemie in Cagliari and the Childrens Hospital Los Angeles (CHLA). Retrospective review of medical records was authorized by the IRB for pediatric patients undergoing cardiac MRI prior to July of 2007. Subjects at both institutions had assessment of cardiac T2* and cardiac function using validated techniques on a 1.5 T General Electric CVi scanner; patients at CHLA also underwent MRI-based liver iron measurements. Complete transfusional iron burden was measured in the Italian patients. Serial MRI data was recorded in 30 patients, but associations between cardiac T2* and age were determined using linear and logistic regression using only results from the first patient MRI scan.Results: Patient ages ranged from 8.0 to 18 years of age at Cagliari (n=36) and 2.5 to 17.9 years of age at CHLA (n=41), reflecting the use of MRI to monitor liver iron in patients < 8 years of age. Patients were moderately iron loaded with hepatic iron concentrations of 12.7 ± 9.8 (CHLA) and ferritin values of 2329 ± 1162 (Cagliari). Median cardiac T2* was 30.2 and ranged from 3.4 to 72.8. Cardiac T2* and its reciprocal were uncorrelated with liver iron (CHLA) and ferritin levels (Cagliari). Figure 1 demonstrates the decrease in cardiac T2* with increasing chronologic age. Serial data are connected by lines. Although linear regression was weakly positive (r2 = 0.06, p = 0.03), the relationship was fundamentally nonlinear. No patient below the age of 9.5 years of age demonstrated an abnormal cardiac T2* (< 20 ms) while 36% of patients between the ages of 15–18 years had detectable cardiac iron. Figure 2 demonstrates the logistic regression curve modeling the prevalence of detectable cardiac iron as a function of age (r2= 0.13, p< 0.002). Chronologic age was highly correlated (r2 = 0.88) with both transfusional iron burden and with duration of transfusion therapy, making it impossible to separate the relative importance of these three variables.Conclusion: Thalassemia major patients did not accumulate cardiac iron until early in their second decade of life. Consequently, cardiac iron monitoring can be safely deferred until children are able undergo MRI examination without sedation. [Display omitted] [Display omitted]

Interview with a Quality Leader: Wilbert H. Mason on Pediatric Healthcare Quality

: Wilbert H. Mason, MD MPH, is professor of clinical pediatrics at the Keck School of Medicine of the University of Southern California and a member of the research faculty of the Saban Research Institute of Childrens Hospital Los Angeles (CHLA). His specialty is pediatric infectious disease, and his research interest includes pediatric health outcomes, epidemiology, and etiology of Kawasaki disease, on which he has numerous publications. He attended medical school at the University of California-Irvine and obtained an MPH in epidemiology from the University of California-Los Angeles. He is a member of the American Academy of Pediatrics and the Infectious Disease Society of America. He has twice received the Philip E. Rothman Memorial Award for Outstanding Teaching at CHLA as well as the Victor E. Stork Award and the Affiliates and Associates Award. Currently he is leading a research project on patient safety in pediatrics funded by the Agency for Healthcare Research and Quality. He provides leadership to the hospital and faculty in quality and performance improvement through his role as medical director for quality systems management. Activities in the areas of medication safety, clinical guideline development, and cost effectiveness have contributed greatly to the quality of clinical care and efficiency at CHLA.

Predictors of fluid resuscitation in pediatric trauma patients

Advanced Trauma Life Support (ATLS) is accepted as the standard for the first hours of trauma care. However, ATLS is designed primarily for adults. In children, vascular access can be difficult and time-consuming. Due to the differences in the epidemiology of children suffering traumatic injury, they may not require aggressive fluid resuscitation. The objective of the study was to establish predictors of fluid resuscitation, and to determine whether all pediatric Level I Trauma victims require two intravenous catheters. Medical charts of all patients aged < 18 years meeting Level I Trauma criteria who presented to Childrens Hospital Los Angeles (CHLA) between January 1 and December 31, 1999 were retrospectively reviewed. There were 152 patients reviewed with a median age of 6 years (range 4 months to 17 years); 64% were boys. The mechanism of injury was motor vehicle crash 49%, fall 37%, crush 8%, gunshot 5%, and knife 1%. Injuries included closed head 88%, penetrating abdomen/chest 6%, and other 6%. Vital signs over time showed no change in 59%, got better in 34%, and got worse in 7%. Fluid resuscitation included no bolus in 70%, 1 bolus in 20%, 2 boluses in 7%, > 2 boluses in 3%. The ICU admitted 23%, 12% were intubated, survival was 95%, and 59% received a prehospital i.v. The i.v. #1 site: antecubital 51%, hand 41%, foot 5%, femoral 1%. The i.v. #2 site: hand 30%, antecubital 20%, foot 2%, none 48%. T test showed no statistically significant differences in fluid resuscitation or second i.v. placement based on the mechanism of injury. T test for unequal variances showed a statistically significant difference in means with p < 0.001 for second i.v. placement as compared with only i.v. fluid amount, age, and Injury Severity Score (ISS). Revised Trauma Score was the only predictor of worsening of vital signs (logistic regression [LR], p < 0.001). Age was the only predictor of second i.v. placement (LR, p < 0.03). ISS was the only predictor of a bolus being given (LR, p < 0.01). In our study, blunt trauma occurred in 90% of children, with 10% requiring > 1 fluid bolus. ISS was the only predictor of the need for fluid resuscitation and is not likely to be helpful in the clinical setting. In our population, nearly 50% had no second i.v. This preliminary review of the nature of pediatric trauma suggests that ATLS guidelines may not always be appropriate for the management of pediatric trauma.

Mood and Behavioral Dysfunction With Opsoclonus-Myoclonus Ataxia

Opsoclonus-myoclonus ataxia syndrome is a paraneoplastic syndrome of cerebellar damage associated with neuroblastoma. The authors assessed psychiatric symptoms of opsoclonus-myoclonus ataxia syndrome in 17 children, who were 16 months to 12(1/2) years of age. Psychiatric symptoms examined included disruptive behavior, affective dysregulation, irritability, impulsivity, cognitive impairment, and poor attention.

Image-matching as a medical diagnostic support tool (DST) for brain diseases in children

Imaging-matching is an important research area in imaging informatics. We have developed and evaluated a novel diagnostic support tool (DST) based on medical image matching using MR brain images. The approach consists of two steps, database generation and image matching. The database contains pre-diagnosed MR brain images. As the images are added to the database, they are registered to the 3D Talairach coordinate system. In addition, regions of interests (ROI) are generated, and image-processing techniques are used to extract relevant image parameters related to the brain and diseases from the ROIs and from the entire MR image. The second step is to retrieve relevant information from the database by performing image matching. In this step, the physician first submits a query image. The DST computes the similarity between the query image and each of the images in the database, and then presents the most similar images to the user. Since the database contains pre-diagnosed images, the retrieved cases tend to contain relevant diagnostic information. To evaluate the usefulness of the DST in a clinical setting, pediatric brain diseases were used. The database contains 2500 pediatric patients between ages 0 and 18 with brain Magnetic Resonance (MR) images of known brain lesions. A testbed was established at the Childrens Hospital Los Angeles (CHLA) for acquiring MR images from the PACS server of patients with known lesions. These images were matched against those in the DST pediatric brain MR database. An expert pediatric neuroradiologist evaluated the matched results. We found that in most cases, the image-matching method was able to quickly retrieve images with relevant diagnostic content. The evaluation method and results are given.