Pediatrics Commons^™

Epilepsy Research In Africa: A Scoping Review By The Ilae Pediatric Commission Research Advocacy Task Force, Pauline Samia, Jane Hassell, Jessica Hudson, Azim Ahmed, Jasmit Shah, Charles Hammond, Edward Kija, Stéphane Auvin, Jo Wilmshurst

Paediatrics and Child Health, East Africa

Objective: Despite the high prevalence of epilepsy in Africa, evaluation of epi- lepsy research trends on the continent is lacking. Without establishing effective research, improvement in care for people with epilepsy cannot be effectively strategized or targeted.

Methods: A scoping review of the peer-reviewed literature on epilepsy from Africa (1989–2019) was conducted. The aim was to understand from this what areas are well researched versus underresearched based on published epilepsy topics.

Results: A total of 1227 publications were identified and assessed. A significant increase in publications occurred over the 30 years assessed. African author lead- ership was …

Use Of Cgm In Monitoring Neonatal Diabetes, Emily Metzinger, Kelsee Halpin

Posters

Introduction Although continuous glucose monitoring (CGM) has increased in patients with Type 1 diabetes, its use in the neonatal population has remained limited. Neonates, particularly preterm infants, are at risk of impaired glucose control. Several studies have looked at the use of these devices in improving care for premature infants without diabetes. We describe a case of a very low birth infant with neonatal diabetes secondary to a GATA-6 mutation. Clinical Case This is a late preterm infant with IUGR, truncus arteriosus, and neonatal diabetes. Exome genetic panel showed a mutation in GATA-6. Her neonatal diabetes was challenging to control …

Use Of Physiologically-Based Pharmacokinetic Modeling To Inform Dosing Of The Opioid Analgesics Fentanyl And Methadone In Children With Obesity, Jacqueline G. Gerhart, Fernando O. Carreño, Jennifer L. Ford, Andrea N. Edginton, Eliana M. Perrin, Kevin M. Watt, William J. Muller, Andrew M. Atz, Amira Al-Uzri, Paula Delmore, Daniel Gonzalez, Catherine Sherwin

Pediatrics Faculty Publications

Obesity is an increasingly alarming public health threat, with nearly 20% of children classified as obese in the United States today. Children with obesity are commonly prescribed the opioids fentanyl and methadone, and accurate dosing is critical to reducing the risk of serious adverse events associated with overexposure. However, pharmacokinetic studies in children with obesity are challenging to conduct, so there is limited information to guide fentanyl and methadone dosing in these children. To address this clinical knowledge gap, physiologically-based pharmacokinetic models of fentanyl and methadone were developed in adults and scaled to children with and without obesity to explore …

Evaluation Of Sibel’S Advanced Neonatal Epidermal (Anne) Wireless Continuous Physiological Monitor In Nairobi, Kenya, Jesse Coleman, Amy Sarah Ginsburg, William Macharia, Roseline Ochieng, Dorothy Chomba, Guohai Zhou, Dustin Dunsmuir, Shuai Xu, J. Mark Ansermino

Paediatrics and Child Health, East Africa

Background: Neonatal multiparameter continuous physiological monitoring (MCPM) technologies assist with early detection of preventable and treatable causes of neonatal mortality. Evaluating accuracy of novel MCPM technologies is critical for their appropriate use and adoption.

Methods: We prospectively compared the accuracy of Sibel’s Advanced Neonatal Epidermal (ANNE) technology with Masimo’s Rad-97 pulse CO-oximeter with capnography and Spengler’s Tempo Easy reference technologies during four evaluation rounds. We compared accuracy of heart rate (HR), respiratory rate (RR), oxygen saturation (SpO₂), and skin temperature using Bland-Altman plots and root-mean-square deviation analyses (RMSD). Sibel’s ANNE algorithms were optimized between each round. …

Detection Of Enterotoxin Gene Cluster In Staphylococcus Epidermidis Recovered From Neonatal Liver Abscess, Bishara J. Freij, Barbara E. Robinson-Dunn, Patrick M. Schlievert

Posters

• Neonatal pyogenic liver abscesses are rare • Potential routes of hepatic infection include: portal vein, biliary ducts, hepatic artery during sepsis, or direct spread from infected contiguous structures • Risk factors may include bloodstream infection, abdominal surgery, umbilical vein catheterization, total parenteral nutrition, necrotizing enterocolitis, and/or immune deficiencies • More common in premature infants at present • Variety of etiologic agents for neonatal liver abscess: Staphylococcus aureus (methicillin-susceptible and resistant), coagulase-negative staphylococci (CONS), streptococci, Escherichia coli, Klebsiella, Pseudomonas, Enterobacter, Serratia, and Candida • Umbilical stump and catheter exit site hub often colonized with CONS • CONS colonize nearly all …

Vampirism For The Microbiologist, Joudeh B. Freij, Bishara J. Freij

Posters

Vampirism has been a means to explain the cause of communicable diseases throughout the centuries.

Single Oral Or Single Intramuscular Dose Of Vitamin D In The Treatment Of Vitamin D Deficiency In Children At Aga Khan University Hospital, Nairobi: A Randomized Controlled Trial, Winfred Mwikali Ndaka

Master of Medicine in Paediatrics and Child Health

Background: Vitamin D deficiency is a global health concern and plays an important role in bone health and many chronic non-communicable diseases. Vitamin D deficiency in children is associated with short stature, underdevelopment, respiratory infections and many other features that increase the risk of childhood complications. The prevalence of vitamin D deficiency in Kenya among children is high. Treatment with daily oral vitamin D is associated with poor compliance. While a single high intramuscular dose vitamin D offers an alternative strategy, it is however, associated with injection site pain and its absorption is dependent on the patient’s peripheral circulation. Various …

Genomic Answers For Children: Dynamic Analyses Of >1000 Pediatric Rare Disease Genomes., Ana S A Cohen, Emily G. Farrow, Ahmed Abdelmoity, Joseph Alaimo, Shivarajan Manickavasagam Amudhavalli, John Anderson, Lalit R. Bansal, Lauren E. Bartik, Primo Baybayan, Bradley Belden, Courtney D. Berrios, Rebecca L. Biswell, Pawel Buczkowicz, Orion Buske, Shreyasee Chakraborty, Warren A. Cheung, Keith A. Coffman, Ashley M. Cooper, Laura A. Cross, Tom Curran, Thuy Tien T. Dang, Mary M. Elfrink, Kendra Engleman, Erin Day Fecske, Cynthia Fieser, Keely M. Fitzgerald, Emily Fleming, Randi N. Gadea, Jennifer L. Gannon, Rose N. Gelineau-Morel, Margaret Gibson, Jeffrey Goldstein, Elin Grundberg, Kelsee Halpin, Brian S. Harvey, Bryce Heese, Wendy Hein, Suzanne M. Herd, Susan Starling Hughes, Mohammed Ilyas, Jill Jacobson, Janda L. Jenkins, Shao Jiang, Jeffrey J. Johnston, Kathryn Keeler, Jonas Korlach, Jennifer Kussman, Christine Lambert, Caitlin E. Lawson, Jean-Baptist Lepichon, J Steven Leeder, Vicki C. Little, Daniel A. Louiselle, Michael Lypka, Brittany D. Mcdonald, Neil Miller, Ann Modrcin, Annapoorna Nair, Shelby H. Neal, Christopher M. Oermann, Donna M. Pacicca, Kailash Pawar, Nyshele L. Posey, Nigel Price, Laura M B Puckett, Julio Quezada, Nikita Raje, William J. Rowell, Eric T. Rush, Venkatesh Sampath, Carol J. Saunders, Caitlin Schwager, Richard M. Schwend, Elizabeth Shaffer, Craig Smail, Sarah E. Soden, Meghan Strenk, Bonnie Sullivan, Brooke Sweeney, Jade B. Tam-Williams, Adam Walter, Holly Welsh, Aaron M. Wenger, Laurel K. Willig, Yun Yan, Scott T. Younger, Dihong Zhou, Tricia N. Zion, Isabelle Thiffault, Tomi Pastinen

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: This study aimed to provide comprehensive diagnostic and candidate analyses in a pediatric rare disease cohort through the Genomic Answers for Kids program.

METHODS: Extensive analyses of 960 families with suspected genetic disorders included short-read exome sequencing and short-read genome sequencing (srGS); PacBio HiFi long-read genome sequencing (HiFi-GS); variant calling for single nucleotide variants (SNV), structural variant (SV), and repeat variants; and machine-learning variant prioritization. Structured phenotypes, prioritized variants, and pedigrees were stored in PhenoTips database, with data sharing through controlled access the database of Genotypes and Phenotypes.

RESULTS: Diagnostic rates ranged from 11% in patients with prior negative …

Hyperinsulinemic Hypoglycemia And Growth Hormone Deficiency Secondary To 20p11 Deletion, Erica Wee, Kavitha Dileepan, Sarah Tsai, Emily Paprocki

Posters

No abstract provided.

Hemoglobin A1c Increase In Youth At Risk For Developing Type 2 Diabetes Mellitus During The Covid-19 Pandemic, Erica Wee, Ashley Sherman, Mark A. Clements, Kelsee Halpin, Yun Yan

Posters

No abstract provided.

Myocardial Work By Strain Echocardiography: A Predictor Of Exercise Capacity In The Fontan Population, Amulya Buddhavarapu, Suma Goudar, David A. White, Daniel Forsha, Doaa Aly

Posters

INTRODUCTION: Global myocardial work index (GWI) obtained by non-invasive pressure-strain loops is a fast-emerging tool to assess myocardial contractility, independent of loading conditions. Data about the feasibility and utility of this modality in single ventricles are still lacking. AIM: We aimed to explore the relationship between the non-invasive myocardial work indices and exercise parameters in the Fontan population. METHOD: Fontan patients were prospectively evaluated by exercise testing and echocardiography. The systemic ventricle was tracked in 3 orthogonal apical planes for global longitudinal strain (GLS). Pressure-strain loops were generated against brachial blood pressure (surrogate for ventricular systolic pressure in the absence …

Asthma Exacerbation, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Clinical Practice Guidelines)

No abstract provided.

Real-Time Feedback Of Air Quality In Children’S Bedrooms Reduces Exposure To Secondhand Smoke, Vincent Berardi, Bradley N. Collins, Laura M. Glynn, Stephen J. Lepore, E. Melinda Mahabee-Gittens, Karen M. Wilson, Melbourne F. Hovell

Psychology Faculty Articles and Research

Introduction:
Secondhand smoke (SHS) exposure creates health risks for nonsmokers and is especially detrimental to children. This study evaluated whether immediate feedback in response to poor indoor air quality in children’s bedrooms can reduce the potential for SHS exposure, as measured by adherence to a World Health Organization (WHO) indoor air standard.

Methods:
Homes that contained children and an adult who regularly smoked inside (n=298) had an air particle monitor installed in the child’s bedroom. These devices measured the concentration of particulate matter (PM2.5) for approximately three months and, for half of the participants, immediately provided aversive feedback …

Nexmif Pathogenic Variants In Individuals Of Korean, Vietnamese, And Mexican Descent, David F Rodriguez-Buritica

Faculty, Staff and Student Publications

NEXMIF pathogenic variants have been known to produce a wide spectrum of X-linked intellectual disability (ID) in both males and females. Thus far, few individuals from diverse populations have been described with NEXMIF-related disorders. Herein, we report three individuals with NEXMIF pathogenic variants, the first two are the only males of Korean and Vietnamese descent described with this disorder to our knowledge. The last patient is a Hispanic female who harbors the same pathogenic variant as a previously described Caucasian individual, but with differing clinical presentation. These patients present with many classic symptoms of NEXMIF-related disorders including ID, epilepsy, developmental …

Pediatric Nemaline Myopathy: A Systematic Review Using Individual Patient Data, Mary Baylies

Faculty, Staff and Student Publications

Nemaline myopathy is a skeletal muscle disease that affects 1 in 50 000 live births. The objective of this study was to develop a narrative synthesis of the findings of a systematic review of the latest case descriptions of patients with NM. A systematic search of MEDLINE, Embase, CINAHL, Web of Science, and Scopus was performed using Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines using the keywords pediatric, child, NM, nemaline rod, and rod myopathy. Case studies focused on pediatric NM and published in English between January 1, 2010, and December 31, …

Using Artificial Neural Networks To Predict Intra-Abdominal Abscess Risk Post-Appendectomy, Michael L Chang

Faculty, Staff and Student Publications

OBJECTIVE: To determine if artificial neural networks (ANN) could predict the risk of intra-abdominal abscess (IAA) development post-appendectomy.

BACKGROUND: IAA formation occurs in 13.6% to 14.6% of appendicitis cases with "complicated" appendicitis as the most common cause of IAA. There remains inconsistency in describing the severity of appendicitis with variation in treatment with respect to perforated appendicitis.

METHODS: Two "reproducible" ANN with different architectures were developed on demographic, clinical, and surgical information from a retrospective surgical dataset of 1574 patients less than 19 years old classified as either negative (n = 1,328) or positive (n = 246) for IAA post-appendectomy …

Oral Drug Dosing Following Bariatric Surgery: General Concepts And Specific Dosing Advice, Jurjen S. Kingma, Desirée M. T. Burgers, Valerie M. Monpellier, Marinus J. Wiezer, Heleen J. Blussé Van Oud‐Alblas, Janelle D. Vaughns, Catherine M. T. Sherwin, Catherijne A. J. Knibbe

Pediatrics Faculty Publications

Bariatric or weight-loss surgery is a popular option for weight reduction. Depending on the surgical procedure, gastric changes like decreased transit time and volume and increased pH, decreased absorption surface in the small intestine, decreased exposure to bile acids and enterohepatic circulation, and decreased gastrointestinal transit time may be expected. In the years after bariatric surgery, patients will also substantially lose weight. As a result of these changes, the absorption, distribution, metabolism and/or elimination of drugs may be altered. The purpose of this article is to report the general influence of bariatric surgery on oral drug absorption, and to provide …

Actions Against Racism: Bringing Together Trauma-Informed Practices, Social-Emotional Learning, And Racial Socialization To Combat Racism In Schools, Danielle R Hatchimonji, Kira Branch, Danika Perry, Tia N Barnes

Department of Pediatrics Faculty Papers

Racism is a social determinant of health with dire consequences for the health, education, and mental health of students of color. Thus, there is an urgent need to develop and test evidence-based strategies to combat racism in schools. In response to this need, our team has developed a multi-tiered school-based intervention to build capacity for combatting racism in educators, students, and families. The "Actions Against Racism" intervention synthesizes three evidence-based practices: trauma-informed practices, social-emotional learning (SEL), and racial socialization. The multiple tiers of intervention aim to cultivate skills for combatting individual and structural racism in educators, families, and students across …

Detoxification Role Of Metabolic Glutathione S-Transferase (Gst) Genes In Blood Lead Concentrations Of Jamaican Children With And Without Autism Spectrum Disorder, Jan Bressler, Sydonnie Shakspeare-Pellington, Katherine A Loveland

Faculty, Staff and Student Publications

Glutathione S-transferases (GST) are involved in the detoxification of exogenous chemicals including lead (Pb). Using data from 344 pairs of autism spectrum disorder (ASD) cases and age- and sex-matched typically developing (TD) controls (2−8 years old) from Jamaica, we investigated the interaction between three GST genes and ASD status as determinants of blood Pb concentrations (BPbCs). We found that ASD cases had lower geometric mean BPbCs than TD children (1.74 vs. 2.27 µg/dL, p < 0.01). Using a co-dominant genetic model, ASD cases with the Ile/Val genotype for the GSTP1 Ile105Val polymorphism had lower GM BPbCs than TD controls, after adjusting for a known interaction between GSTP1 and GSTT1, child’s parish, socioeconomic status, consumption of lettuce, fried plantains, and canned fish (Ile/Val: 1.78 vs. 2.13 µg/dL, p = 0.03). Similarly, among carriers of the I/I or I/D (I*) genotype for GSTT1 and GSTM1, ASD cases had lower adjusted GM BPbCs than TD controls (GSTT1 I*: 1.61 vs. 1.91 µg/dL, p = 0.01; GSTM1 I*: 1.71 vs. 2.04 µg/dL, p = 0.01). Our findings suggest that genetic polymorphisms in GST genes may influence detoxification of Pb by the enzymes they encode in Jamaican children with and without ASD.

Cross-Reactive Immunity Against The Sars-Cov-2 Omicron Variant Is Low In Pediatric Patients With Prior Covid-19 Or Mis-C., Juanjie Tang, Tanya Novak, Julian Hecker, Gabrielle Grubbs, Fatema Tuz Zahra, Lorenza Bellusci, Sara Pourhashemi, Janet Chou, Kristin Moffitt, Natasha B. Halasa, Stephanie P. Schwartz, Tracie C. Walker, Keiko M. Tarquinio, Matt S. Zinter, Mary A. Staat, Shira J. Gertz, Natalie Z. Cvijanovich, Jennifer E. Schuster, Laura L. Loftis, Bria M. Coates, Elizabeth H. Mack, Katherine Irby, Julie C. Fitzgerald, Courtney M. Rowan, Michele Kong, Heidi R. Flori, Aline B. Maddux, Steven L. Shein, Hillary Crandall, Janet R. Hume, Charlotte V. Hobbs, Adriana H. Tremoulet, Chisato Shimizu, Jane C. Burns, Sabrina R. Chen, Hye Kyung Moon, Christoph Lange, Adrienne G. Randolph, Surender Khurana

Manuscripts, Articles, Book Chapters and Other Papers

Neutralization capacity of antibodies against Omicron after a prior SARS-CoV-2 infection in children and adolescents is not well studied. Therefore, we evaluated virus-neutralizing capacity against SARS-CoV-2 Alpha, Beta, Gamma, Delta and Omicron variants by age-stratified analyses (5 years of age. As expected, convalescent pediatric COVID-19 and MIS-C cohorts demonstrate higher neutralization titers than hospitalized acute COVID-19 patients. Overall, children and adolescents show some loss of cross-neutralization against all variants, with the most pronounced loss against Omicron. In contrast to SARS-CoV-2 infection, children vaccinated twice demonstrated higher titers against Alpha, Beta, Gamma, Delta and Omicron. These findings can influence transmission, re-infection …