Children's National Medical Center Research Articles

High Hydroxyurea Usage in Sickle Cell Anemia Regardless of Patient Demographic

Introduction/Background: Hydroxyurea is highly effective in sickle cell disease, yet it is still underutilized. The Pediatric Sickle Cell Program of Northern Virginia recently published a 10-year analysis of uniform hydroxyurea (HU) implementation starting in infancy for all patients with sickle cell disease of severe genotype (SCA), demonstrating rise in HU usage from 32.5% baseline rate to 79.6% after two years, and to > 90% after 8 years. 1 Other HU utilization studies reporting lower rates largely use Medicaid data, and lower socioeconomics is often cited as a barrier to HU utilization. The Northern Virginia study reported on all patients with SCA regardless of insurance coverage, thus raising the question whether the high HU usage rate achieved in this program was influenced by a high proportion of non-Medicaid patients or other unusual demographic features. Therefore, we analyzed 10 years of HU usage data by insurance, ethnicity, and age. Methods: HUdata extracted from a longitudinal REDCap database and electronic medical records (EMR) with IRB approval (Children's National Medical Center IRB, Pro00002745, Pro00013374) previously reported 1 for five 2-year intervals are additionally analyzed for insurance, ethnicity, and age, with new data for 2019-2021. HU-eligibility includes SCA patients age ≥ 9 months and not chronically transfused. HU users (on HU) are patients demonstrating Hgb F rise and expected hemogram changes in response to HU, monitored every 2-3 months; HU non-users (not on HU) are patients who refuse HU or are grossly non-adherent as evidenced by lack of changes in Hgb F and hemogram. Pharmacy dispense records available in Epic EMR are used as corroborative information. Results: 1. Medicaid patients comprised 39 - 46% and non-Medicaid patients comprised 54 - 61% of all patients over the five 2-year periods. African-American (AA) and African patients comprised 85% of all patients. Medicaid coverage ranged from 24 - 39% for AA patients and 38 - 48% for African patients. (African is defined as born in Africa or having parents born in Africa who immigrated to the US.) 2. Comparing HU usage by insurance over the five 2-year periods, HU users increased from 72% to 91% for Medicaid patients and from 83% to 95% for non-Medicaid patients. Conversely, HU non-users decreased from 28% to 9% for Medicaid patients and from 17% to 5% for non-Medicaid patients. No statistical difference was detected in HU usage by Medicaid versus non-Medicaid insurance in any interval (p = 0.13 to 0.91) (Table 1). 3. HU usage rates in AA and African patients were not statistically different (p = 0.07 to 0.32), except in one interval (2013-15) (p = 0.006) (Table 1). 4. Comparison of HU usage in two age groups, 9 months-12 years and ≥ 13 years, showed that while HU non-acceptance/non-adherence tended to be higher in the age ≥ 13 group, no statistical significance was reached in any interval (p = 0.13 to 0.97) (Table 1). 5. The uniform HU implementation program targeted for fetal hemoglobin (Hgb F) 30% or higher by frequent HU dose optimization and close adherence monitoring. Hgb F > 20% was considered good adherence, and Hgb F 10-20% reflected partial adherence. Partially adherent patients did not distribute differently between Medicaid and non-Medicaid insurance (p = 0.07 to 0.86) but were predominantly age > 13 (p = 0.0005 to 0.001) (Table 2). Conclusion: 1. HU users and HU non-users did not disproportionately distribute to any demographic group. 2. Uniform HU implementation was effective in both Medicaid and non-Medicaid populations. 3. Ethnicity and age were not significant factors in HU uptake or acceptance, but decreased HU adherence is more frequent in teenagers. 4. HU can be effectively implemented for all SCA patients across demographic groups. Medicaid insurance, as a proxy for socioeconomic level, is not a barrier to high HU usage. Reference: 1. Phan V, Park JA, Dulman R, Lewis A, Briere N, Notarangelo B, Yang E. Ten-year longitudinal analysis of hydroxyurea implementation in a pediatric sickle cell program. Eur J Haematol. 2022 Nov;109(5):465-473. doi: 10.1111/ejh.13827. Epub 2022 Sep 2. PMID: 35811388.

Association between Acute Pain Scores in Children with Sickle Cell Disease and Emergency Department Disposition and Return Visit Rates

Introduction Reducing pain through prompt administration of pain medication in the emergency department (ED) is a primary driver of national guidelines for sickle cell disease (SCD), yet there are few data on how pain scores and changes in pain scores are associated with both the disposition at the initial visit and the likelihood of a return visit. In this study, we analyzed acute pain scores from ED visits for children with uncomplicated SCD pain crisis to determine the relationships between pain scores and disposition (admission or discharge) or occurrence of a return ED visit. Objectives For children with uncomplicated SCD pain crises, we aimed to determine (1) the association between initial pain score, last pain score, change in pain scores and the disposition of index ED visits, and (2) the association between these pain score values, number of parenteral opioid doses in the ED, whether an opioid prescription was provided at discharge, and an ED return visit within 14 days. Methods This is a multicenter retrospective cohort study of acute pain scores of ED visits by children for uncomplicated SCD pain crises. It was conducted using electronic health care data from the Pediatric Emergency Care Applied Research Network (PECARN) Registry across 7 sites (Lurie Children's, Children's Hospital of Philadelphia, Cincinnati Children's Hospital, Children's National Medical Center, Children's Colorado, Children's Wisconsin, and Nationwide Children's). Index ED visits were defined as uncomplicated pain crises with no preceding ED visits within 14 days between 01/2017 - 11/2021. We collected the following ED pain scores: initial, last, and change in scores (initial minus last) as measured by a numeric rating scale (0-10). For analyses related to disposition, we modeled the relationship between the three pain scores and hospital disposition adjusting for age, sex and uncomplicated pain crisis visit admission rate. For analyses related to return visits, we utilized the same predictor variables and added both the number of parenteral opioid doses administered in the ED and whether or not a prescription for an opioid was given at discharge. For all analyses, adjustment for site was made using generalized estimating equations with an exchangeable correlation structure to account for clustering within site. Modified Poisson regression was used to identify associations. SAS software version 9.4 was used for all statistical analyses. Results A total of 4,985 index visits for uncomplicated SCD pain crisis were included; 52% resulted in hospitalization. Overall, 39% of the children were <12 years old, 54% were female, and 96% were Black. Eligible index ED visits per site varied from 157 to 1855. The overall ED median (IQR) initial pain score was 8.0 (6.0-10).The median last pain score was 5.0 (2.0-8.0); median change in ED pain score was 2.0 (0.0-5.0). In univariate analyses, higher initial and last pain scores and a smaller change in pain scores were all associated with higher hospitalization rates. Adjusted rate ratios (95% CI) from multivariable models showed last pain score was the best predictor of admission 1.16 (1.12, 1.19), with change in pain score no longer significant 0.98 (0.96, 1.00). Examining percent of children hospitalized by last pain score shows over 80% of children with a last pain score of 7 or higher were hospitalized, compared to less than 40% if 4 or lower (Figure 1). 29% of the 2,377 visits that were originally discharged to home had a return visit within 14 days. None of the included pain scores from the initial visit (initial, last, or change) were associated with having a return visit within 14 days. However, both an increased number of ED opioid doses and failure to receive a prescription for an opioid at discharge were associated with increased return visits. Figure 2 shows the ED opioid doses and prescription for opioid combination and their respective return visit rates, with a high of 36% for ≥ 3 doses of ED opioid doses and no prescription and a low of 22% if 1 opioid given and a prescription at discharge. Conclusions: We found that for a child with SCD presenting to the ED with an uncomplicated pain crisis, the last ED pain score was a better predictor of hospitalization than initial or change in pain score. None of those pain scores predicted a return visit after discharge; however, fewer opioid doses in the ED and receipt of an opioid prescription at discharge were associated with fewer return visits.

Videofluoroscopic swallow study and fiberoptic endoscopic evaluation of swallow, which is superior?

The LaryngoscopeEarly View Triological Society Best Practice Videofluoroscopic swallow study and fiberoptic endoscopic evaluation of swallow, which is superior? CM Lawlor, CM Lawlor orcid.org/0000-0003-0636-6233 Division of Otolaryngology, Children's National Medical Center, Washington, DC, USASearch for more papers by this authorSukgi S. Choi, Corresponding Author Sukgi S. Choi [email protected] orcid.org/0000-0002-5448-1546 Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital. Harvard Medical School, Boston, Massachusetts, USA Send correspondence to Sukgi S. Choi, MD, Department of Otolaryngology, Boston Children's Hospital, 300 Longwood Ave BCH3129, Boston, MA 02115, USA. Email: [email protected]Search for more papers by this author CM Lawlor, CM Lawlor orcid.org/0000-0003-0636-6233 Division of Otolaryngology, Children's National Medical Center, Washington, DC, USASearch for more papers by this authorSukgi S. Choi, Corresponding Author Sukgi S. Choi [email protected] orcid.org/0000-0002-5448-1546 Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital. Harvard Medical School, Boston, Massachusetts, USA Send correspondence to Sukgi S. Choi, MD, Department of Otolaryngology, Boston Children's Hospital, 300 Longwood Ave BCH3129, Boston, MA 02115, USA. Email: [email protected]Search for more papers by this author First published: 18 May 2023 https://doi.org/10.1002/lary.30755 The authors have no funding, financial relationships, or conflicts of interest to disclose. Editor's Note: This Manuscript was accepted for publication on May 08, 2023. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation

Introduction to fertility preservation in pediatric patients with cancer: A report from the Children's Oncology Group.

Pediatric Blood & CancerEarly View e30235 HIGHLIGHT Introduction to fertility preservation in pediatric patients with cancer: A report from the Children's Oncology Group Jennifer M Levine, Corresponding Author Jennifer M Levine [email protected] orcid.org/0000-0003-1417-0169 Pediatric Oncology, Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, District of Columbia, USA Correspondence Jennifer M Levine, 111 Michigan Ave, NW, 4th Floor, Pediatric Oncology, Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC 20010, USA. Email: [email protected]Search for more papers by this author Jennifer M Levine, Corresponding Author Jennifer M Levine [email protected] orcid.org/0000-0003-1417-0169 Pediatric Oncology, Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, District of Columbia, USA Correspondence Jennifer M Levine, 111 Michigan Ave, NW, 4th Floor, Pediatric Oncology, Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC 20010, USA. Email: [email protected]Search for more papers by this author First published: 20 February 2023 https://doi.org/10.1002/pbc.30235Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issuee30235 RelatedInformation

Triological Society Best Practice: Repair of Type 1 Laryngeal Cleft.

The LaryngoscopeEarly View Triological Society Best Practice Triological Society Best Practice: Repair of Type 1 Laryngeal Cleft Claire M. Lawlor MD, Claire M. Lawlor MD orcid.org/0000-0003-0636-6233 Division of Otolaryngology, Children's National Medical Center, Washington, DC, USASearch for more papers by this authorSukgi S. Choi MD, MBA, Corresponding Author Sukgi S. Choi MD, MBA [email protected] orcid.org/0000-0002-5448-1546 Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, USA Send correspondence to Sukgi S. Choi, Department of Otolaryngology, Boston Children's Hospital, 300 Longwood Ave, BCH3129, Boston, MA 02115, USA. Email: [email protected]Search for more papers by this author Claire M. Lawlor MD, Claire M. Lawlor MD orcid.org/0000-0003-0636-6233 Division of Otolaryngology, Children's National Medical Center, Washington, DC, USASearch for more papers by this authorSukgi S. Choi MD, MBA, Corresponding Author Sukgi S. Choi MD, MBA [email protected] orcid.org/0000-0002-5448-1546 Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, USA Send correspondence to Sukgi S. Choi, Department of Otolaryngology, Boston Children's Hospital, 300 Longwood Ave, BCH3129, Boston, MA 02115, USA. Email: [email protected]Search for more papers by this author First published: 09 February 2023 https://doi.org/10.1002/lary.30612 Editor's Note: This Manuscript was accepted for publication on January 26, 2023. The authors have no funding, financial relationships, or conflicts of interest to disclose. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation

Characteristics and outcomes of glomerulonephritis with membranoproliferative pattern in children.

BackgroundMembranoproliferative glomerulonephritis (MPGN) is a rare histopathologic pattern of glomerular injury with limited studies in pediatric patients. Characteristics and outcomes of children with MPGN have also remained to be further explored.MethodsWe retrospectively reviewed the clinicopathological features, genetic findings, treatments and outcomes in 17 pediatric patients pathologically diagnosed with MPGN from 2007 to 2020 in the Children’s National Medical Center in China.ResultsMedian age at disease onset was 9.9 years (IQR, 5.6–11.9 years). Most of the patients (12/17) had nephrotic range of proteinuria, and nephritic-nephrotic syndrome was the most common clinical presentation (35.2%). Secondary causes were identified in eight patients including hepatitis B virus (HBV) infection (n=4), methylmalonic acidemia (MMA, n=2), rheumatoid arthritis (RA, n=1) and Aymé-Gripp Syndrome (n=1). The nine patients with primary MPGN were further identified as immune-complex mediated MPGN (n=8), and unclassifiable MPGN (U-MGPN, n=1). Genetic analyses identified pathogenic variants of MMACHC gene in two cases of MMA and established the diagnosis for Aymé-Gripp syndrome in one case with a de novo variant of MAF gene. Comparing study between the complete or partial remission group (n=8) and non-response group (n=9) showed a significant difference in the timing of renal biopsy (P<0.05). Normal renal function was preserved in ten patients at the last follow-up. Two patients developed into end-stage renal disease (ESRD).ConclusionsChildren with MPGN pattern present heterogenous clinical features. Genetic detection helps to explore underlying causes of MPGN. Early identification of the primary or secondary causes of MPGN in children is vital.

Sistrunk vs modified Sistrunk procedures: Does procedure type matter?

BackgroundThyroglossal Duct Cyst (TDC) is the most common congenital neck mass in children and is surgically managed with a Sistrunk procedure. Some surgeons perform a modified Sistrunk (mSis), involving the dissection of the fistula beyond the hyoid bone without coring out the foramen cecum at the base of the tongue. We aim to evaluate surgical outcomes of children undergoing Sistrunk (Sis) or modified Sistrunk (mSis) procedures for TDC at an academic pediatric institution. Materials and methodsWe conducted a retrospective chart review of the Children's National Medical Center database from 2004 to 2014. Basic demographic information, preoperative characteristics, postoperative complications, and recurrence were extracted for children diagnosed with TDC. We estimated descriptive statistics using Kruskal-Wallis tests and Pearson's chi-square for continuous and categorical values. Results157 patients that underwent TDC excision were identified. Sistrunk (Sis) was performed in 52 cases (33%) and modified Sistrunk (mSis) performed in 105 (67%) cases. 84 (54%) were female and the mean age at surgery was 5.4 years (SD=4.5). Overall recurrence was detected in 8 cases (5.1%) and did not differ significantly by procedure type [2 (4%) in Sis and 6 (6%) in mSis, p = 0.616]. Post-operative complications did not differ significantly between Sis and mSis procedure: swelling [6 (12%) and 18 (17%), p = 0.481]; seroma [5 (10%) and 10 (10%), p = 1.00]; surgical site infection [3 (6%) and 8 (8%), p = 0.752]; or post-excision incision and drainage [3 (6%) and 9 (9%), p = 0.752] (respectively). ConclusionsOur findings reveal no statistical difference in recurrence rates between Sis and mSis with no risk factors for recurrence identified. Furthermore, there was no difference in post-operative complications between the groups. Both surgical procedures were associated with few complications and low recurrence.

Reference Values of Serum MMP-7 and a Novel Measurement Approach Using Dried Blood Spot: A Diagnostic Test for Biliary Atresia

Background: Matrix metalloproteinase-7 (MMP-7) holds great promise as a diagnostic marker for biliary atresia (BA). This study aimed to establish reference values of serum MMP-7 and explore a novel measurement approach using dried blood spot (DBS). Methods: This was a single-center diagnostic test. Serum and DBS MMP-7 concentrations were measured using an ELISA kit. Intraoperative cholangiography and subsequent histological examinations were used to confirm BA diagnoses. Findings: 176 infants without hepatobiliary diseases were enrolled in the control group. The median serum MMP-7 concentration was 7.53 ng/mL (IQR: 5.58, 10.26) compared with 13.82 ng/mL (IQR: 8.10, 19.80) in the neonatal subgroup. 568 cholestatic infants were enrolled, including 318 patients in the retrospective cohort and 250 in the prospective cohort. Using a cutoff value of 18 ng/mL, the area under the ROC curve (AUC) for the retrospective cohort was 0.967 (95% confidence interval [CI]: 0.947–0.988), with a sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 93.0%, 93.8%, 94.7%, and 91.9%, respectively. The model was prospectively validated and achieved a diagnostic sensitivity of 94.8% (146/154; 95%CI: 89.7%–97.6%), specificity of 87.0% (80/92; 95%CI: 77.9%–92.8%), PPV of 92.4% (146/158; 95%CI: 86.8%–95.8%), and NPV of 90.9% (80/88; 95%CI: 82.3%–95.7%). 136 samples were tested via both DBS and serum. Linear regression showed that DBS MMP-7 levels correlated well with serum results (R = 0.942, P < 0.001). Interpretation: MMP-7 levels can precisely diagnose BA. A reference range and cutoff value were established, which were higher in neonates. DBS could be an alternative approach to measuring MMP-7. Clinical Trial Registration Details: Prospectively registered at http://www.chictr.org.cn/ (ChiCTR2000032983). Funding Information: This study received financial support from Clinical Research Plan of SHDC (no. SHDC2020CR2009A), Shanghai Municipal Key Clinical Specialty (no. shslczdzk05703), National Natural Science Foundation of China (no. 81770519, no. 81771633, no. 81873545 and no. 81974059), The Science Foundation of Shanghai (no. 18411969100 and no. 19ZR1406600), and Children's National Medical Center (no. EK1125180104, no. EKYY20180204, EK112520180211 and no. EK112520180310). Declaration of Interests: Jiale Deng, Dake Yang, Weiwei Xiang, Xianghui Zhang and Zhuo Fang work for WuXi Diagnostics. All authors declared no other conflicts of interest. Ethics Approval Statement: This study was reviewed and approved by the Ethics Committee of Children’s Hospital of Fudan University (No.: 2020-296). This work was performed in compliance with the Declaration of Helsinki and other relevant regulations. Informed consent was obtained from the guardian of each participant before enrollment.

Central breathing abnormalities in children with trisomy 21: Effect of age, sex, and concomitant OSA.

Trisomy 21 (TS21) is a condition with a high risk for sleep apnea. In the pediatric population, the risk also includes central breathing disorders. The aim of this study was to define the clinical and polysomnographic characteristics of central apnea in infants, children, and adolescents with TS21. Retrospective review of baseline polysomnograms (PSGs) in children with TS21 in the sleep center at Children's National Medical Center in Washington DC. We included a total of 158 infants, children, and adolescents (0-18 years) with TS21 in this study. The median age was 4.82 years and 62% were male. The primary findings of the study are that (1) 12% of all pediatric subjects with TS21 included had a central apnea index (CAI) > 2/h; (2) the proportion of TS21 individuals with central breathing abnormalities progressively decreased with age being common in young individuals (≤2 years of age) but rare after 10 years of age; (3) additional sleep breathing disturbances (e.g., OSA and/or hypoxemia) are often present in children with TS21 and central apnea; and (4) the prevalence of central breathing abnormalities in TS21 is influenced by sex, being more likely to persist beyond early childhood (>2 years of age) in females than in males. Central breathing abnormalities are common in TS21 among young children (≤2 years of age) and in females older than 2 years of age. Central apnea is often associated with concomitant obstructive sleep apnea and/or hypoxemia in children with TS21.

Gamma-Glutamyl Transpeptidase Has Different Efficacy on Biliary Atresia Diagnosis in Different Hospital Patient Groups - An Application of Machine Learning Approach

Background: Gamma-glutamyl transpeptidase (GGT) is the most widely used biomarker in the early diagnosis of biliary atresia (BA), but its diagnostic efficacy is questionable in different sub-populations. We aim to identify subgroups that are defined by specific variables with cut-offs and can significantly affect the diagnostic efficacy of GGT for detecting BA. Methods: Clinical data from 1273 infants with neonatal obstructive jaundice (NOJ) diagnosed between January 2012 and December 2017 at the Children’s Hospital of Fudan University were collected, reviewed, and analyzed. Random forest-based Virtual Twins method was used to identify potential subgroups . Findings: Hemoglobin (HGB) and fasting gallbladder filling were selected as defining variables. The diagnostic efficacy of GGT was significantly better (AUC=0.855) for patients with hemoglobin (HGB)≤105 g/L and a gallbladder that was not or poorly filled. Diagnostic efficacy was worst in the subgroup defined by HGB>105 g/L (AUC=0.722). The inclusion of interaction terms between GGT and the subgroups in a logistic regression model significantly improved (p=0.002) prediction performance. Interpretation: This study provides evidence that the diagnostic efficacy of GGT can differ significantly across different subgroups. Therefore, a GGT diagnostic result should be interpreted cautiously when patients belong to subgroups with low diagnostic efficacy. The development of a prediction model and/or clinical diagnostic pathway for early detection of BA should also account for the heterogeneous diagnostic efficacy of GGT. Funding Statement: This study received financial support from Shanghai Key Disciplines (no.2017ZZ02022), National Natural Science Foundation of China (no. 81770519, no. 81771633, no. 81873545 and no. 81974059), The Science Foundation of Shanghai (no. 14411969800, no. 16411952200, no. 18411969100 and 19ZR1406600), and Children's National Medical Center (no. EK1125180104, no. EKYY20180204, EK112520180211 and no. EK112520180310). Declaration of Interests: The authors have no conflict of interest to disclose. Ethics Approval Statement: The study was approved by the Ethics Committee of the Children’s Hospital of Fudan University. Written informed consent was obtained from the legal guardians of all patients before the enrollment of the study.

Editorial introductions

Current Opinion in Psychiatry was launched in 1988. It is one of a successful series of review journals whose unique format is designed to provide a systematic and critical assessment of the literature as presented in the many primary journals. The field of psychiatry is divided into 12 sections that are reviewed once a year. Each section is assigned a Section Editor, a leading authority in the area, who identifies the most important topics at that time. Here we are pleased to introduce the Journal's Section Editors for this issue. SECTION EDITORS Hans W. HoekHans W. HoekDr Hans W. Hoek is Professor of Psychiatry at University Medical Center Groningen, the Netherlands, and has an adjunct position as Professor of Epidemiology at Columbia University, New York, USA. He is based in The Hague where he is Director of the psychiatric residency program and Dean of the Parnassia Academy, part of Parnassia Psychiatric Institute, a large mental health institute with branches throughout the Netherlands. Dr Hoek has a long-term interest in both schizophrenia and eating disorders. In the schizophrenia field he has been involved in studies on schizophrenia spectrum disorders after prenatal exposure to famine and on first episode psychosis among immigrants in The Netherlands, with research ongoing in rural South Africa. Dr Hoek's main publications in the eating disorder field relate to the epidemiology and treatment of eating disorders. Dr Hoek has been chair of the work group that developed the recent Dutch practice guideline for the treatment of eating disorders. Dr Hoek participated in the revision of DSM–5 as member of both the Eating Disorders Work Group and the Impairment and Disability Study Group. He has been consultant for the development of the WHO Disability Assessment Schedule (WHODAS 2.0). He is past president of the Eating Disorders Research Society and was the recipient of the Academy for Eating Disorders Leadership Award in Research 2011. Anna Keski-RahkonenAnna Keski-RahkonenDr Anna Keski-Rahkonen, MD, PhD, MPH is a psychiatrist, an epidemiologist, and a sexologist. She currently works as a Professor of Mental Health at the University of Helsinki in Finland. She is board certified in public health, psychiatry, and medical education. She received her medical training at the University of Helsinki and studied public health at Columbia University in New York, USA. She has published over 70 original articles on various aspects of eating disorders and has received many awards for her teaching and mentoring. She has also written several books on eating disorders for the general audience. She is particularly interested in the epidemiology of eating disorders and body image. Richa BhatiaRicha BhatiaRicha Bhatia, MD is a Child, Adolescent and Adult psychiatrist, dual Board certified in Child, Adolescent and General Psychiatry by the American Board of Psychiatry and Neurology. She serves as Section Editor for the Child and Adolescent Psychiatry issue of Current Opinion in Psychiatry. She has also served as an Associate Editor for the journal, Current Psychiatry, and presently serves as a member of its editorial board. She is a former faculty member in the departments of psychiatry at Harvard Medical School, USA, and Geisel School of Medicine at Dartmouth, USA. She completed her child and adolescent psychiatry fellowship from Children's National Medical Center / George Washington University in Washington, DC, USA. Dr Bhatia has been the recipient of Marian Butterfield Award by the Association of Women Psychiatrists, and has been recognized as a Fellow of the American Psychiatric Association. She is the author of two books on psychiatry: Demystifying Psychiatric Conditions and Treatments and 65 Answers about Psychiatric Conditions. She has been an active member of the Anxiety and Depression Association of America and the American Psychiatric Association. She has chaired and lectured at workshops at various psychiatric conferences. Some of her areas of interest are global mental health, compassion cultivation, and integration of psychotherapy and psychopharmacology into clinical practice. Her expert professional opinions have been quoted in prominent news media such as CNBC, The Guardian, US News and World Report.

COVID-19 Pandemic Restrictions and the Use of Technology for Pediatric Palliative Care in the Acute Care Setting.

The COVID-19 pandemic has caused health care facilities to restrict visitors for patients in all care settings. Most pediatric care facilities have restricted visitation to one parent at a time, unfortunately even if the child is in critical condition or is terminally ill. These situations have necessitated the use of technology such as the Zoom platform to have difficult conversations concerning complex medical decision-making and goals of care. In cases where the child is deemed at immediate end of life, many facilities will allow both parents to be at the bedside, but no other family or friends that may be integral support to the parents or child. These situations have compelled the use of FaceTime, Zoom, or Skype technology to facilitate real-time support at end of life for these young patients and their caregivers. This article presents a case where technologies such as these were utilized to assist a family in goals-of-care discussions and at end of life for an infant in the intensive care unit at a large urban pediatric care facility during the COVID-19 pandemic.

Clinical Utility of Anaerobic and Fungal Blood Cultures in the Pediatric Oncologic Population.

If there is a concern for sepsis in a pediatric patient an aerobic blood culture is obtained. A febrile or ill oncology patient will often be evaluated with aerobic, anaerobic, and fungal blood cultures. There is to our knowledge little evidence to confirm the added benefit of broadly obtaining these cultures. A retrospective analysis of blood cultures from patients in the oncology and hematopoietic stem cell transplant wards at Children's National Medical Center between January 2010 and April 2017. Positive anaerobic and fungal cultures were evaluated for corollary positive aerobic cultures from the same time frame (+/-24 h). Unique isolates were designated as those positive anaerobic and fungal cultures without positive aerobic cultures and evaluated with detailed chart review. A total of 10,950 cultures were evaluated during the study period: 6579 aerobic, 2391 anaerobic cultures, 1980 fungal. In total, 419 positive aerobic, 140 positive anaerobic, and 43 positive fungal cultures were reviewed. Among these, 22 unique anaerobic cultures and 21 unique fungal cultures met case criteria. Only 7 cultures of obligate anaerobes were observed: 4 Clostridial spp., 2 Propionobacterium acnes, and 1 Peptostreptococcus spp. Thirteen of 21 fungal isolates consisted of a single colony and were regarded as probable contaminants. Penicillium, Cladosporium, and dematiaceous molds were in greatest frequency. Potential risk factors for patients with breakthrough isolates were evaluated and described. There is little increased yield from the use fungal and anaerobic blood cultures in addition to aerobic blood cultures in the routine evaluation of pediatric oncology and bone marrow transplant patients. Fungal and anaerobic blood cultures and should be reserved for cases with high clinical suspicion.

Gastric emptying of milk in infants and children up to 5years of age: normative data and influencing factors.

Gastric emptying scintigraphy is widely used in infants and children, but there is a lack of age-specific normative data. The objectives of this retrospective study were: 1) to establish a range of gastric emptying of milk or formula as a surrogate for normal gastric emptying in infants and young children ≤5years of age, and 2) to investigate the effects of patient age, feeding volume, feeding route and gastroesophageal reflux on gastric emptying. The reports of 5,136 gastric emptying studies of children ≤5years of age performed at Children's National Medical Center from January 1990 to August 2012 were reviewed. Demographic data, 1-h and 3-h gastric emptying values and gastroesophageal reflux status of all patients were stored in a database. Using stringent inclusion and exclusion criteria, the studies of patients as similar to healthy children as possible were selected for this study. The study group included 2,273 children (57% male) ages 0-59months (median: 4.6months). The median 1-h gastric emptying was 43% (interquartile range [IQR] 34-54%). The median 3-h gastric emptying was 91% (IQR 79-98%). Sixty-one percent of patients with 1-h gastric emptying value of <50% had 3-h gastric emptying ≥80%. Gastric emptying was significantly faster in children ≤6months as compared with all older age groups. In each age group, the median gastric emptying decreased with increasing feeding volume. Gastric emptying was significantly faster in patients fed via combined nasogastric tube and oral routes as compared with those fed exclusively orally. There was no significant difference in gastric emptying of children with and without gastroesophageal reflux. Although there are statistically significant differences in gastric emptying based on age, volume and route of feeding, the data suggest that overall normal liquid gastric emptying in infants and children ≤5years of age is ≥80% at 3h. One-hour emptying measurements are not reliable for detecting delayed gastric emptying.

Editorial introductions

Current Opinion in Psychiatry was launched in 1988. It is one of a successful series of review journals whose unique format is designed to provide a systematic and critical assessment of the literature as presented in the many primary journals. The field of psychiatry is divided into 12 sections that are reviewed once a year. Each section is assigned a Section Editor, a leading authority in the area, who identifies the most important topics at that time. Here we are pleased to introduce the Journal's Section Editors for this issue. SECTION EDITORS Hans W. HoekHans W. HoekDr Hans W. Hoek is Professor of Psychiatry at University Medical Center Groningen, the Netherlands, and has an adjunct position as Professor of Epidemiology at Columbia University, New York, USA. He is based in The Hague where he is Director of the psychiatric residency program and Dean of the Parnassia Academy, part of Parnassia Psychiatric Institute, a large mental health institute with branches throughout the Netherlands. Dr Hoek has a long-term interest in both schizophrenia and eating disorders. In the schizophrenia field he has been involved in studies on schizophrenia spectrum disorders after prenatal exposure to famine and on first episode psychosis among immigrants in The Netherlands, with research ongoing in rural South Africa. Dr Hoek's main publications in the eating disorder field relate to the epidemiology and treatment of eating disorders. Dr Hoek has been chair of the work group that developed the recent Dutch practice guideline for the treatment of eating disorders. Dr Hoek participated in the revision of DSM–5 as member of both the Eating Disorders Work Group and the Impairment and Disability Study Group. He has been consultant for the development of the WHO Disability Assessment Schedule (WHODAS 2.0). He is past president of the Eating Disorders Research Society and was the recipient of the Academy for Eating Disorders Leadership Award in Research 2011. Anna Keski-RahkonenAnna Keski-RahkonenDr Anna Keski-Rahkonen, MD, PhD, MPH is a psychiatrist, an epidemiologist, and a sexologist. She currently works as a Professor of Mental Health at the University of Helsinki in Finland. She is board certified in public health, psychiatry, and medical education. She received her medical training at the University of Helsinki and studied public health at Columbia University in New York, USA. She has published over 70 original articles on various aspects of eating disorders and has received many awards for her teaching and mentoring. She has also written several books on eating disorders for the general audience. She is particularly interested in the epidemiology of eating disorders and body image. Richa BhatiaRicha BhatiaRicha Bhatia, MD is a Child, Adolescent and Adult psychiatrist, dual Board certified in Child, Adolescent and General Psychiatry by the American Board of Psychiatry and Neurology. She serves as Section Editor for the Child and Adolescent Psychiatry issue of Current Opinion in Psychiatry. She has also served as an Associate Editor for the journal, Current Psychiatry. In addition, she serves on the editorial board of other psychiatry journals such as Culture, Medicine and Psychiatry. She is a former faculty member in the departments of psychiatry at Harvard Medical School, USA, and Geisel School of Medicine at Dartmouth, USA. She completed her child and adolescent psychiatry fellowship from Children's National Medical Center / George Washington University in Washington, DC, USA. Dr Bhatia has been the recipient of Marian Butterfield Award by the Association of Women Psychiatrists, and has been recognized as a Fellow of the American Psychiatric Association. She is the author of two books on psychiatry: Demystifying Psychiatric Conditions and Treatments and 65 Answers about Psychiatric Conditions. She has been an active member of the Anxiety and Depression Association of America and the American Psychiatric Association. She has chaired and lectured at workshops at various psychiatric conferences. Some of her areas of interest are global mental health, compassion cultivation, and integration of psychotherapy and psychopharmacology into clinical practice.

Editorial introductions

Current Opinion in Ophthalmology was launched in 1990. It is one of a successful series of review journals whose unique format is designed to provide a systematic and critical assessment of the literature as presented in the many primary journals. The field of Ophthalmology is divided into nine sections that are reviewed once a year. Each section is assigned a Section Editor, a leading authority in the area, who identifies the most important topics at that time. Here we are pleased to introduce the Journal's Section Editors for this issue. SECTION EDITORS Cynthia AlleyCynthia AlleyDr Cynthia Alley specializes in pediatric ophthalmology and strabismus. She has a special interest in treating adult strabismus. Dr Alley earned her medical degree from the Temple University School of Medicine, and then completed her training in ophthalmology at Temple University Hospital. She went on to complete a fellowship in Pediatric Ophthalmology and Strabismus at Children's National Medical Center in Washington, DC. Dr Alley is a fellow of the American Academy of Ophthalmology, a member of the American Association for Pediatric Ophthalmology and the Costenbader Society, where she serves on the board. Dr Alley is the former Director of Pediatric Ophthalmology at Temple University Hospital, where she also served as Residency Program Director. Currently, she is on staff at Wills Eye Hospital, where she trains ophthalmology residents and medical students. She is also an Assistant Professor of Ophthalmology at The Philadelphia College of Osteopathic Medicine. Dr Alley is actively involved with World Blindness Outreach, a non-profit humanitarian organization dedicated to treating preventable causes of blindness around the world. She has participated in multiple missions in India, Brazil, Nicaragua, Dominican Republic, and Ecuador, and serves on the board of directors. Richard C. AllenRichard C. AllenRichard C. Allen, MD, PhD, FACS is a Professor in the Dept. of Ophthalmology at Baylor College of Medicine in Houston, TX. Dr Allen attended Duke University and graduated magna cum laude and Phi Beta Kappa with a BS in chemistry where he received the Departmental Award in Chemistry. He was accepted into the Medical Scientist Training Program at Baylor College of Medicine where he received his MD and PhD Degrees. His dissertation “Molecular Genetics of X-linked Immunodeficiencies Affecting B Lymphocyte Function” was awarded the Sigma Xi Thesis Excellence Award. Dr Allen completed a residency in ophthalmology at the University of Iowa Hospitals and Clinics. Under the mentorship of Edwin M. Stone, MD, PhD, and Thomas A. Oetting, MD, MS, he completed a fellowship in molecular ophthalmology and comprehensive ophthalmology at the University of Iowa. In 2000, Dr Allen joined the faculty in the Dept. of Ophthalmology at Baylor College of Medicine where he was Chief of the Ophthalmology Service at Ben Taub General Hospital. In 2002, he joined the faculty in the Division of Ophthalmology, Dept. of Surgery at the University of New Mexico School of Medicine. From 2004 to 2006, he completed additional fellowship training in oculoplastic surgery at the University of Iowa under the direction of Jeffrey A. Nerad MD and Keith D. Carter MD, sponsored by the American Society of Ophthalmic Plastic and Reconstructive Surgery (ASOPRS). In 2006, Dr Allen returned to New Mexico where he rejoined the faculty at the University of New Mexico. In 2009, Dr Allen returned to the University of Iowa as an Associate Professor with tenure in the Dept. of Ophthalmology. From 2013–2014, he was a visiting physician at St. Erik's Eye Hospital, Stockholm, Sweden. In 2015, Dr Allen joined the faculty at MD Anderson Cancer Center in Houston, Texas, USA; in 2018, he moved his primary appointment to Baylor College of Medicine where his is a Professor with tenure. Dr Allen is a member of ASOPRS, the Orbital Society, the European Society of Ophthalmic Plastic and Reconstructive Surgery, the North American Skull Base Society, and the American Eye Study Club. His research interests include oncologic and genetic diseases of the eyelids, orbit, and lacrimal system. He sees patients at Alkek Eye Center, Texas Children's Hospital, MD Anderson Cancer Center, and Ben Taub General Hospital. He resides in Houston, TX with his wife Missy, son Augustus, and daughter Astrid.

Evaluation of an Abbreviated Emergency Triage Assessment and Treatment (ETAT) Course at Children's National Medical Center (CNMC)

Background: The World Health Organization Emergency Triage Assessment and Treatment guidelines present recommendations for systematic triage and immediate emergency response in low-resource settings. The WHO instructional plan was developed as 7 half-day sessions targeting health workers with limited education. Medical residents in the US would also benefit from learning triage in resource-limited settings. As part of Children's National …

Evaluation of an Abbreviated Emergency Triage Assessment and Treatment (ETAT) Course at Children's National Medical Center (CNMC)

Evaluation of an Abbreviated Emergency Triage Assessment and Treatment (ETAT) Course at Children's National Medical Center (CNMC)

Management of Chiari I malformations: a paradigm in evolution

Despite decades of experience and research, the etiology and management of Chiari I malformations (CM-I) continue to raise more questions than answers. Controversy abounds in every aspect of management, including the indications, timing, and type of surgery, as well as clinical and radiographic outcomes. This review aims to outline past experiences, consolidate current evidence, and recommend directions for the future management of the Chiari I malformation. A review of recent literature on the management of CM-I in pediatric patients is presented, along with our experience in managing 1073 patients who were diagnosed with CM-I over the past two decades (1998-2018) at Children's National Medical Center (CNMC) in Washington DC. The general trend reveals an increase in the diagnosis of CM-I at younger ages with a significant proportion of these being incidental findings (0.5-3.6%) in asymptomatic patients as well as a rise in the number of patients undergoing Chiari posterior fossa decompression surgery (PFD). The type of surgical intervention varies widely. At our institution, 104 (37%) Chiari surgeries were bone-only PFD with/without outer leaf durectomy, whereas 177 (63%) were PFD with duraplasty. We did not find a significant difference in outcomes between the PFD and PFDD groups (p = 0.59). An analysis of failures revealed a significant difference between patients who underwent tonsillar coagulation versus those whose tonsils were not manipulated (p = 0.02). While the optimal surgical intervention continues to remain elusive, there is a shift away from intradural techniques in favor of a simple, extradural approach (including dural delamination) in pediatric patients due to high rates of clinical and radiographic success, along with a lower complication rate. The efficacy, safety, and necessity of tonsillar manipulation continue to be heavily contested, as evidence increasingly supports the efficacy and safety of less tonsillar manipulation, including our own experience.

Assessment of ST2 for Risk of Death Following Graft-versus-Host Disease in the Pediatric and Adult Age Groups

Background: To address the need for establishing prognostic markers of Non-Relapse Mortality (NRM) and acute graft-versus-hostdisease (aGVHD) after allogeneic hematopoietic cell transplantation (HCT) in the pediatric age group, we conducted a prospective study (NCT02194439). Methods: 415 patients at six U.S. centers including 170 children ≤10 years were accrued from 2013 to 2018. Four biomarkers [stimulation-2 (ST2), interleukin-6, regenerating-islet-derived-3-alpha (REG3α) and tumornecrosis-factor-receptor-1 (TNFR1)] were assessed in the plasma pre-HCT (day -7), and at days +7, +14, +21 post-HCT. We performed landmark analyses for NRM and grade II-IV aGVHD, dichotomizing the cohort at ≤10 years and using each biomarker median as a cutoff for high and low risk groups. The multivariable analyses included the significant covariates. Findings: The NRM was not different among children ≤10 years compared to subjects >10 years. Landmark analyses using post-HCT assays showed that ST2 (>26 ng/mL), TNFR1 (>3441 pg/mL), and REG3α (>25 ng/mL) are associated with NRM in both children 10 years [HR (CI): ST2: 2.60 (1.15-5.86), p=0.021; TNFR1: 2.09 (0.96-4.58), p=0.06; REG3α: 2.57 (1.19-5.55), p=0.016]. When pre-HCT biomarkers were included, ST2 remained significant in both cohorts. After adjustment for significant covariates (race/ethnicity, malignant disease, graft, GVHD prophylaxis), ST2 remained associated with NRM only in recipients ≤10 years [HR(CI): 4.82 (1.89-14.66), p=0.0056]. ST2 was also associated with grade II-IV aGVHD in both ≤10 years and >10 years groups (p=0.0059, and p<0.0001). Interpretation: Assays of ST2, TNFR1, REG3α in the first 3 weeks following HCT have prognostic value for NRM and aGVHD in both children and adults. ST2 prior to HCT is a prognostic biomarker for NRM and severe aGVHD in children ≤10 years allowing for additional stratification. Trial Registration: Clinicaltrial.gov under NCT02194439. Funding Statement: The Eunice Kennedy Shriver National Institute of Child Health and Human Development of Health from the National Institutes of Health R01HD074587, the National Cancer Institute R01CA168814, the Leukemia and Lymphoma Society (grant 1293-15), and the Lilly Physician Scientist Initiative Award. Declaration of Interests: S.P. is an inventor on a patent on “Methods of detection of graft-versus-host disease” (US- 13/573,766). K.R.C. is on the advisory board of Jazz pharmaceuticals. All other authors declare no competing interests. Ethics Approval Statement: This study was approved by the respective Institutional Review Boards at six adult and pediatric centers: Children's National Medical Center, Texas Children's Hospital, Fred Hutchinson Cancer Research Center, Boston Children’s/Dana Farber Cancer Institute, Johns Hopkins, and Indiana University. Informed consent was obtained from all patients or their legal guardians.