Pediatrics Commons^™

Response Assessment In Pediatric Craniopharyngioma: Recommendations From The Response Assessment In Pediatric Neuro-Oncology (Rapno) Working Group, Lindsey M Hoffman, Camilo Jaimes, Kshitij Mankad, David M Mirsky, Benita Tamrazi, Christopher L Tinkle, Cassie Kline, Aparna Ramasubramanian, Fatema Malbari, Ross Mangum, Holly Lindsay, Vincent Horne, David J Daniels, Sameer Keole, David R Grosshans, Tina Young Poussaint, Roger Packer, Sergio Cavalheiro, Brigitte Bison, Todd C Hankinson, Hermann L Müller, Ute Bartels, Katherine E Warren, Murali Chintagumpala

Faculty, Staff and Student Publications

BACKGROUND: Craniopharyngioma is a histologically benign tumor of the suprasellar region for which survival is excellent but quality of life is often poor secondary to functional deficits from tumor and treatment. Standard therapy consists of maximal safe resection with or without radiation therapy. Few prospective trials have been performed, and response assessment has not been standardized.

METHODS: The Response Assessment in Pediatric Neuro-Oncology (RAPNO) committee devised consensus guidelines to assess craniopharyngioma response prospectively.

RESULTS: Magnetic resonance imaging is the recommended radiologic modality for baseline and follow-up assessments. Radiologic response is defined by 2-dimensional measurements of both solid and cystic tumor …

Rare Case Of Nasal Vault Oncocytic Cystadenoma, Aarti Agarwal, Kathleen Mcclain, Karen Banker, Kudakwashe Chikwava, Udayan K. Shah

Department of Otolaryngology - Head and Neck Surgery Faculty Papers

Background: Oncocytic Cystadenomas are a rare benign pathology often found to arise from the salivary glands, reported more commonly in minor salivary glands, but even more rarely in major salivary glands and the larynx. This is the first known report of a nasal vault oncocytic cystadenoma in a pediatric patient.

Methods: Case Report and Literature Review.

Case presentation: A 10-year-old female presented with a mass involving the left nasal vestibule that caused nasal obstruction and a visible external abnormality. MRI imaging revealed a cystic lesion in the anterior nasal cavity that was rim enhancing with a bright T2 signal that …

N-Of-1 Trials Vs Usual Care In Children With Hypertension: A Pilot Randomized Clinical Trial, Joyce P Samuel, Cynthia S Bell, Joshua A Samuels, Celin Rajan, Adrienne K Walton, Charles Green, Jon E Tyson

Faculty, Staff and Student Publications

BACKGROUND: Blood pressure (BP) is often inadequately controlled in children treated for hypertension, and personalized (n-of-1) trials show promise for tailoring treatment choices. We assessed whether patients whose treatment choices are informed by an n-of-1 trial have improved BP control compared to usual care.

METHODS: A randomized clinical trial was conducted in a pediatric hypertension clinic in Houston from April 2018 to September 2020. Hypertensive adolescents and young adults 10-22 years old were randomized 1:1 to a strategy of n-of-1 trial using ambulatory BP monitoring to inform treatment choice or usual care, with treatment selected by physician preference. The primary …

Early Antibiotics And Risk For Necrotizing Enterocolitis In Premature Infants: A Narrative Review., Alain Cuna, Michael J. Morowitz, Venkatesh Sampath

Manuscripts, Articles, Book Chapters and Other Papers

While prompt initiation of antibiotics at birth due to concerns for early onset sepsis is common, it often leads to many preterm infants being exposed to treatment despite negative blood cultures. Such exposure to early antibiotics can impact the developing gut microbiome putting infants at increased risk of several diseases. Necrotizing enterocolitis (NEC), a devastating inflammatory bowel disease that affects preterm infants, is among the most widely studied neonatal disease that has been linked to early antibiotics. While some studies have demonstrated an increased risk of NEC, other studies have demonstrated seemingly contrary findings of decreased NEC with early antibiotics. …

Emerging Therapeutic Strategies For Diffuse Intrinsic Pontine Glioma: A Systematic Review, Shahrukh Farrukh, Shagufta Habib, Amna Rafaqat, Zouina Sarfraz, Azza Sarfraz, Muzna Sarfraz, Karla Robles-Velasco, Miguel Felix, Ivan Cherrez-Ojeda

Department of Paediatrics and Child Health

Background: Of all central nervous systems tumors, 10-20% are located in the brainstem; diffuse intrinsic pontine glioma (DIPG) is diagnosed in 80% of them. With over five decades of clinical trial testing, there are no established therapeutic options for DIPG. This research article aims to collate recent clinical trial data and provide a landscape for the most promising therapies that have emerged in the past five years.
Methods: PubMed/MEDLINE, Web of Science, Scopus, and Cochrane were systematically searched using the following keywords: Diffuse intrinsic pontine glioma, Pontine, Glioma, Treatment, Therapy, Therapeutics, curative, and/or Management. Both adult and pediatric patients with …

Micrornas And Gene Regulatory Networks Related To Cleft Lip And Palate, Chihiro Iwaya, Akiko Suzuki, Junichi Iwata

Faculty, Staff and Student Publications

Cleft lip and palate is one of the most common congenital birth defects and has a complex etiology. Either genetic or environmental factors, or both, are involved at various degrees, and the type and severity of clefts vary. One of the longstanding questions is how environmental factors lead to craniofacial developmental anomalies. Recent studies highlight non-coding RNAs as potential epigenetic regulators in cleft lip and palate. In this review, we will discuss microRNAs, a type of small non-coding RNAs that can simultaneously regulate expression of many downstream target genes, as a causative mechanism of cleft lip and palate in humans …

The Clinical And Genetic Spectrum Of Autosomal-Recessive Tor1a-Related Disorders, Afshin Saffari, Tracy Lau, Homa Tajsharghi, Ehsan Ghayoor Karimiani, Ariana Kariminejad, Stephanie Efthymiou, Giovanni Zifarelli, Tipu Sultan, Mehran Beiraghi Toosi, Shahnaz Ibrahim

Department of Paediatrics and Child Health

In the field of rare diseases, progress in molecular diagnostics led to the recognition that variants linked to autosomal-dominant neurodegenerative diseases of later onset can, in the context of biallelic inheritance, cause devastating neurodevelopmental disorders and infantile or childhood-onset neurodegeneration. TOR1A-associated arthrogryposis multiplex congenita 5 (AMC5) is a rare neurodevelopmental disorder arising from biallelic variants in TOR1A, a gene that in the heterozygous state is associated to torsion dystonia-1 (DYT1 or DYT-TOR1A), an early-onset dystonia with reduced penetrance. While 15 individuals with TOR1A-AMC5 have been reported (less than 10 in detail), a systematic investigation of the full disease-associated spectrum has …

Tobacco Exposure In Adults And Children With Proteinuric Glomerulopathies: A Neptune Cohort Study., Linda Wang, Bayle Smith-Salzberg, Kevin Ec Meyers, Dorey A. Glenn, Katherine R. Tuttle, Vimal K. Derebail, Tammy M. Brady, Keisha Gibson, Abigail R. Smith, Michelle M. O'Shaughnessy, Tarak Srivastava, Gentzon Hall, Jarcy Zee, Markus Bitzer, Christine B. Sethna

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Tobacco exposure has been recognized as a risk factor for cardiovascular disease (CVD) and progression of kidney disease. Patients with proteinuric glomerulopathies are at increased risk for cardiovascular morbidity and mortality. Multiple studies have linked tobacco exposure to CVD and chronic kidney disease, but the relationships between smoking and proteinuric glomerulopathies in adults and children have not been previously explored.

METHODS: Data from the Nephrotic Syndrome Study Network (NEPTUNE), a multi-center prospective observational study of participants with proteinuric glomerulopathies, was analyzed. 371 adults and 192 children enrolled in NEPTUNE were included in the analysis. Self-reported tobacco exposure was classified …

Bnt162b2 Mrna Vaccination Against Coronavirus Disease 2019 Is Associated With A Decreased Likelihood Of Multisystem Inflammatory Syndrome In Children Aged 5-18 Years-United States, July 2021 - April 2022., Laura D. Zambrano, Margaret M. Newhams, Samantha M. Olson, Natasha B. Halasa, Ashley M. Price, Amber O. Orzel, Cameron C. Young, Julie A. Boom, Leila C. Sahni, Aline B. Maddux, Katherine E. Bline, Satoshi Kamidani, Keiko M. Tarquinio, Kathleen Chiotos, Jennifer E. Schuster, Melissa L. Cullimore, Sabrina M. Heidemann, Charlotte V. Hobbs, Ryan A. Nofziger, Pia S. Pannaraj, Melissa A. Cameron, Tracie C. Walker, Stephanie P. Schwartz, Kelly N. Michelson, Bria M. Coates, Heidi R. Flori, Elizabeth H. Mack, Laura Smallcomb, Shira J. Gertz, Samina S. Bhumbra, Tamara T. Bradford, Emily R. Levy, Michele Kong, Katherine Irby, Natalie Z. Cvijanovich, Matt S. Zinter, Cindy Bowens, Hillary Crandall, Janet R. Hume, Manish M. Patel, Angela P. Campbell, Adrienne G. Randolph, Overcoming Covid-19 Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C), linked to antecedent severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, is associated with considerable morbidity. Prevention of SARS-CoV-2 infection or coronavirus disease 2019 (COVID-19) by vaccination might also decrease MIS-C likelihood.

METHODS: In a multicenter, case-control, public health investigation of children ages 5-18 years hospitalized from 1 July 2021 to 7 April 2022, we compared the odds of being fully vaccinated (2 doses of BNT162b2 vaccine ≥28 days before hospital admission) between MIS-C case-patients and hospital-based controls who tested negative for SARS-CoV-2. These associations were examined by age group, timing of vaccination, …

Life-Threatening Complications Of Influenza Vs Coronavirus Disease 2019 (Covid-19) In Us Children, Natasha B Halasa, Andrew J Spieker, Cameron C Young, Samantha M Olson, Margaret M Newhams, Justin Z Amarin, Kristin L Moffitt, Mari M Nakamura, Emily R Levy, Vijaya L Soma, Rana Talj, Scott L Weiss, Julie C Fitzgerald, Elizabeth H Mack, Aline B Maddux, Jennifer E Schuster, Bria M Coates, Mark W Hall, Stephanie P Schwartz, Adam J Schwarz, Michele Kong, Philip C Spinella, Laura L Loftis, Gwenn E Mclaughlin, Charlotte V Hobbs, Courtney M Rowan, Melania M Bembea, Ryan A Nofziger, Christopher J Babbitt, Cindy Bowens, Heidi R Flori, Shira J Gertz, Matt S Zinter, John S Giuliano, Janet R Hume, Natalie Z Cvijanovich, Aalok R Singh, Hillary A Crandall, Neal J Thomas, Melissa L Cullimore, Manish M Patel, Adrienne G Randolph, Pediatric Intensive Care Influenza, Overcoming Covid-19 Investigators

Faculty, Staff and Student Publications

Background: Clinical differences between critical illness from influenza infection vs coronavirus disease 2019 (COVID-19) have not been well characterized in pediatric patients.

Methods: We compared demographics, clinical characteristics, and outcomes of US children (aged 8 months to 17 years) admitted to the intensive care or high-acuity unit with influenza or COVID-19. Using mixed-effects models, we assessed the odds of death or requiring life support for influenza vs COVID-19 after adjustment for age, sex, race and Hispanic origin, and underlying conditions including obesity.

Results: Children with influenza (n = 179) were younger than those with COVID-19 (n = 381; median, 5.2 …

Pulse Oximetry: Why Oxygen Saturation Is Still Not A Part Of Standard Pediatric Guidelines In Low-And-Middle-Income Countries (Lmics), Maheen Sheikh, Huzaifa Ahmad, Romesa Ibrahim, Muhammad Imran Nisar, Fyezah Jehan

Department of Paediatrics and Child Health

Background: With the high frequency of acute respiratory infections in children worldwide, particularly so in low-resource countries, the development of effective diagnostic support is crucial. While pulse oximetry has been found to be an acceptable method of hypoxemia detection, improving clinical decision making and efficient referral, many healthcare set ups in low- and middle-income countries have not been able to implement pulse oximetry into their practice.
Main body: A review of past pulse oximetry implementation attempts in low- and middle-income countries proposes the barriers and potential solutions for complete integration in the healthcare systems. The addition of pulse oximetry into …

Speech/Language Impairment Or Specific Learning Disability? Examining The Usage Of Educational Categories, Lisa Archibald, Wendy C. Georgan, Tiffany Hogan

Data and Test Instruments

No abstract provided.

Child Opportunity Index And Hospital Utilization In Children With Traumatic Brain Injury Admitted To The Picu, Monica M. Gray, Kurt C. Stange, Elaine A. Borawski, Steven L. Shein, Katherine N. Slain

Faculty Scholarship

IMPORTANCE: The need to understand how Community-based disparities impact morbidity and mortality in pediatric critical illness, such as traumatic brain injury. Test the hypothesis that ZIP code-based disparities in hospital utilization, including length of stay (LOS) and hospital costs, exist in a cohort of children with traumatic brain injury (TBI) admitted to a PICU using the Child Opportunity Index (COI). DESIGN: Multicenter retrospective cohort study. SETTING: Pediatric Health Information System (PHIS) database. PATIENTS: Children 0-18 years old admitted to a PHIS hospital with a diagnosis of TBI from January 2016 to December 2020 requiring PICU care. To identify the most …

Prevalence Of Non-Alcoholic Fatty Liver Disease In Overweight And Obese Children Seeking Ambulatory Healthcare In Nairobi, Kenya, Anne Njeri Mburu, Ahmed Laving, William Macharia, Joyce Sande

Paediatrics and Child Health, East Africa

Background While linked to obesity and associated with an increased cardiovascular morbidity, non-alcoholic fatty liver disease (NAFLD) is an often-asymptomatic cause of chronic liver disease in children. Early detection provides opportunity for interventions to curb progression. Childhood obesity is on the rise in low/middle-income countries, but cause-specific mortality data associated with liver disease are scanty. Establishing the prevalence of NAFLD in overweight and obese Kenyan children would guide in public health policies aimed at early screening and intervention.

Objectives To investigate prevalence of NAFLD in overweight and obese children aged 6–18 years using liver ultrasonography.

Methodology This was a cross-sectional …

Late Onset Paradoxical Effect Of Zolpidem After Anoxic Brain Injury: A Pediatric Case Report, Amanda Lindenberg, Sathya Vadivelu

Posters

Case Diagnosis Acquired brain injury (ABI) is a common cause of disability. It is defined as an insult to the brain that subsequently results in impairments of communication, cognition, sensorimotor function and behavior. Anoxic brain injuries can be particularly disabling. A common cause of a anoxic injury in children is cardiopulmonary arrest. The revised coma recovery scale (CRS-R) is a standardized assessment for level of arousal after brain injury. Case Description A 16 year old healthy boy was admitted after an unwitnessed pulseless cardiopulmonary arrest requiring 3 defibrillations and 2mg of epinephrine. His cardiac and genetic workup was negative. His …

Parent Satisfaction With Same Day Discharge After Laparoscopic Appendectomy For Non Perforated Appendicitis, Nelimar Cruz-Centeno, James Fraser, Shai Stewart Md, Derek Marlor, Rebecca M. Rentea, Pablo Aguayo, David Juang, Richard J. Hendrickson, Charles L. Snyder, Bhargava Mullapudi, Shawn D. St.Peter, Jason D. Fraser, Tolulope A. Oyetunji

Presentations

Background: Same-day discharge (SDD) after laparoscopic appendectomy for acute non-perforated appendicitis is safe, without an increased rate of postoperative complications, emergency department visits, or re-admissions. We aimed to evaluate caregiver satisfaction with this protocol. Materials and Methods: Patients discharged on the day of laparoscopic appendectomy for non-perforated acute appendicitis were identified between January 2022-August 2022. Surveys to evaluate satisfaction with the protocol were distributed to the caregivers via e-mail or text message 96 hours after discharge. Telephone surveys were conducted if there were no responses to the initial online survey. The surveys assessed comfort with SDD, post-operative pain control adequacy, …

The Efficacy Of Blended Learning In A Pediatric Spine Deformity Management Program In Sub-Saharan Africa, Alaaeldin Ahmad, Abdallah Abushehab, François Waterkeyn, Beverly Cheserem, Massimo Balsano, Christopher Bonfield, Hamisi Shabani, Juma Magogo, Bryson Mcharo, Costansia Bureta

General Surgery, East Africa

Introduction: Our study assessed the efficacy of blended learning, which combines in-person learning and e-learning, in a pediatric scoliosis training program through an international collaborative effort.

Methods: The course comprised two parts: the online portion, where participants reviewed educational materials for 3 weeks and met with faculty once/week for discussion, and the in-person session, where participants reviewed cases in a team-based approach and came to a consensus on treatment strategy, followed by discussion with an international expert. All participants completed a needs assessment (NA) and clinical quiz at three points: before the course, after the online session, and after the …

Successful Va Ecmo For A Pre-B Cell Acute Lymphoblastic Leukemia Patient With Necrotizing Faciitis From Clostridium Septicum, Johanna I. Orrick, Brittany Rouchou, Cara Holton, Jenna Miller

Posters

REPORT: It can be challenging to calculate the risks versus benefits of a potential Extracorporeal Membrane Oxygenation (ECMO) pediatric candidate. A patient with an oncologic comorbidity and an increased potential of needing near-future surgical interventions carries higher ECMO risks. We report a successful VA ECMO run of a newly diagnosed acute lymphoblastic leukemia (ALL) adolescent with Clostridium septicum necrotizing fasciitis. A previously healthy 16-year-old male was diagnosed with pre-B cell ALL after having an ileocolic intussusception and a Clostridium septicum blood infection. He underwent successful surgical intussusception reduction, started induction chemotherapy, and completed a ten-day course of antibiotics. On hospital …

Experiences Of Racism Among Black And African Children With Asthma, Esosa Adah, Rayanna Tucker, Bridgette Jones

Posters

Rationale: The relationship between adverse childhood experiences, toxic stress and asthma risk has been previously described in the literature among adult and pediatric populations. Studies have identified experiences of racism as a chronic stressor associated with asthma morbidity however, most studies have focused primarily on adults or parental perspectives. We initiated a pilot study to characterize described experiences of racism among Black/African American children with persistent asthma and describe some of our initial results. Methods: Children were asked to complete the “Perceptions of Racism in Children and Youth,” a validated questionnaire, measuring perceptions of racism and discrimination. Responses were analyzed …

Hypertrophic Pyloric Stenosis Protocol: A Single Center Study, Nelimar Cruz-Centeno, James A. Fraser Md, Shai Stewart Md, Derek Marlor, Rebecca M. Rentea, Pablo Aguayo, David Juang, Richard J. Hendrickson, Charles L. Snyder, Shawn D. St.Peter, Jason D. Fraser, Tolulope A. Oyetunji

Presentations

Introduction: Initial management of hypertrophic pyloric stenosis (HPS) is correction of electrolyte disturbances with fluid resuscitation. In 2015, our institution implemented a fluid resuscitation protocol based on previous data that focused on minimizing blood draws and allowing immediate ad libitum feeds postoperatively. Here we describe the protocol and subsequent outcomes. Methods: We conducted a single-center retrospective review of patients diagnosed with HPS from 2016-2020. All patients were managed per the protocol outlined in Figure 1. All were given formula or breast milk after the post-anesthesia care unit and discharged home after tolerating three consecutive feeds. Feedings were given every 2-3 …