Pediatrics Commons^™

Adaptive, Behavioral, And Emotional Outcomes Following Postoperative Pediatric Cerebellar Mutism Syndrome In Survivors Treated For Medulloblastoma, Kimberly P Raghubar, Andrew M Heitzer, Fatema Malbari, Jason Gill, Roy V Sillitoe, Livia Merrill, Johanna Escalante, M Fatih Okcu, Guillermo Aldave, Avner Meoded, Stephen Kralik, Kimberly Davis, Marina Ma, Emily A H Warren, Mark D Mccurdy, Howard L Weiner, William Whitehead, Michael E Scheurer, Lisa Rodriguez, Amy Daigle, Murali Chintagumpala, Lisa S Kahalley

Faculty, Staff and Students Publications

OBJECTIVE: Patients who experience postoperative pediatric cerebellar mutism syndrome (CMS) during treatment for medulloblastoma have long-term deficits in neurocognitive functioning; however, the consequences on functional or adaptive outcomes are unknown. The purpose of the present study was to compare adaptive, behavioral, and emotional functioning between survivors with and those without a history of CMS.

METHODS: The authors examined outcomes in 45 survivors (15 with CMS and 30 without CMS). Comprehensive neuropsychological evaluations, which included parent-report measures of adaptive, behavioral, and emotional functioning, were completed at a median of 2.90 years following craniospinal irradiation.

RESULTS: Adaptive functioning was significantly worse in …

Catheter-Based Fetal Cardiac Interventions, Betul Yilmaz Furtun, Shaine Alaine Morris

Faculty, Staff and Students Publications

Fetal cardiac intervention (FCI) is an emerging and rapidly advancing group of interventions designed to improve outcomes for fetuses with cardiovascular disease. Currently, FCI is comprised of pharmacologic therapies (e.g., trans-placental antiarrhythmics for fetal arrhythmia), open surgical procedures (e.g., surgical resection of pericardial teratoma), and catheter-based procedures (e.g., fetal aortic valvuloplasty for aortic stenosis). This review focuses on the rationale, criteria for inclusion, technical details, and current outcomes of the three most frequently performed catheter-based FCI procedures: (1) aortic valvuloplasty for critical aortic stenosis (AS) associated with evolving hypoplastic left heart syndrome (HLHS), (2) atrial septal intervention for HLHS with …

Feasibility Of Fit24, A Digital Diabetes Prevention Program For Hispanic Adolescents: Qualitative Evaluation Study, Erica G Soltero, Salma M Musaad, Teresia M O'Connor, Debbe Thompson, Keith Norris, Bettina M Beech

Faculty, Staff and Students Publications

BACKGROUND: Digital health interventions are promising for reaching and engaging high-risk youth in disease prevention opportunities; however, few digital prevention interventions have been developed for Hispanic youth, limiting our knowledge of these strategies among this population.

OBJECTIVE: This study qualitatively assessed the feasibility and acceptability of Fit24, a 12-week goal-setting intervention that uses a Fitbit watch (Fitbit Inc) and theoretically grounded SMS text messages to promote physical activity and sleep among Hispanic adolescents (aged between 14 and 16 years) with obesity.

METHODS: After completing the intervention, a subsample of youth (N=15) participated in an in-depth interview. We categorized the themes …

Seasonality, Clinical Characteristics, And Outcomes Of Respiratory Syncytial Virus Disease By Subtype Among Children Aged <5 Years: New Vaccine Surveillance Network, United States, 2016-2020, Ariana P Toepfer, Justin Z Amarin, Andrew J Spieker, Laura S Stewart, Mary Allen Staat, Elizabeth P Schlaudecker, Geoffrey A Weinberg, Peter G Szilagyi, Janet A Englund, Eileen J Klein, Marian G Michaels, John V Williams, Rangaraj Selvarangan, Christopher J Harrison, Joana Y Lively, Pedro A Piedra, Vasanthi Avadhanula, Brian Rha, James Chappell, Meredith Mcmorrow, Heidi Moline, Natasha B Halasa

Faculty, Staff and Students Publications

BACKGROUND: Respiratory syncytial virus (RSV) is a leading cause of acute respiratory illnesses in children. RSV can be broadly categorized into 2 major subtypes: A and B. RSV subtypes have been known to cocirculate with variability in different regions of the world. Clinical associations with viral subtype have been studied among children with conflicting findings such that no conclusive relationships between RSV subtype and severity have been established.

METHODS: During 2016-2020, children aged/guardian interviews, chart reviews, and collection of midturbinate nasal plus/minus throat swabs for RSV (RSV-A, RSV-B, and untyped) using reverse transcription polymerase chain reaction.

RESULTS: Among 6398 RSV-positive …

Parallel Use Of Low-Complexity Automated Nucleic Acid Amplification Tests And Lateral Flow Urine Lipoarabinomannan Assays To Detect Tuberculosis Disease In Adults And Adolescents Living With Hiv, Stephanie Bjerrum, Bada Yang, Johanna Åhsberg, Ruvandhi R Nathavitharana, Laura Olbrich, Devan Jaganath, Alexander W Kay, Andreas Lundh, Maunank Shah

Faculty, Staff and Students Publications

This is a protocol for a Cochrane Review (diagnostic). The objectives are as follows: To compare the diagnostic accuracy of parallel use of lateral flow urine lipoarabinomannan on urine and low-complexity automated nucleic acid amplification tests on respiratory samples versus each test alone for detection of tuberculosis disease in adults and adolescents living with HIV who present with presumptive tuberculosis. Secondary objectives To investigate the following sources of heterogeneity: clinical setting; signs and symptoms of tuberculosis; screening positivity for tuberculosis disease by chest x-ray, C-reactive protein elevation, and molecular World Health Organization-recommended rapid diagnostics; seriously ill; advanced HIV; and CD4 …

Parallel Use Of Low-Complexity Automated Nucleic Acid Amplification Tests On Respiratory Samples And Stool With Or Without Lateral Flow Lipoarabinomannan Assays To Detect Pulmonary Tuberculosis Disease In Children, Laura Olbrich, Alexander W Kay, Stephanie Bjerrum, Bada Yang, Johanna Åhsberg, Ruvandhi R Nathavitharana, Andreas Lundh, Maunank Shah, Devan Jaganath

Faculty, Staff and Students Publications

This is a protocol for a Cochrane Review (diagnostic). The objectives are as follows: To compare the diagnostic accuracy of the parallel use of low-complexity automated nucleic acid amplification tests on respiratory and stool specimens in children and lateral flow urine lipoarabinomannan amongst children with HIV versus each assay alone for detecting pulmonary tuberculosis. Secondary objectives To compare the diagnostic accuracy of low-complexity automated nucleic acid amplification tests on respiratory and stool specimens in combination versus each sample type alone. To investigate the following sources of heterogeneity: clinical setting, signs and symptoms of pulmonary tuberculosis disease, screening positivity by chest …

Special Anatomy Series Imaging Inner Ear Structures With High-Frequency Ultrasound: Application To Physical Rehabilitation Space Medicine, Jeffrey Strakowski, Han Zhang, Millard Reschke, Faye Y Chiou-Tan

Faculty, Staff and Students Publications

OBJECTIVE: The objective of this paper is to document the feasibility of image acquisition, image optimization, and sonographic appearance of the exposed anatomic windows of cadaveric inner ear dissection for purposes of potential future clinical evaluation as part of the developing area of physical and rehabilitation space medicine.

METHODS: Cadaveric dissection of the inner ear was conducted with the goal of exposing areas relevant to vestibular balance. Middle and inner ear structures of 3 human cadavers were imaged with multiple broadband transducers, including emphasis with higher frequency transducers.

RESULTS: The images were best optimized with 17 MHz and 22 MHz …

Herbal And Dietary Supplement Induced Liver Injury Leading To Hepatitis-Associated Severe Aplastic Anemia: A Case Report, Rebecca Mercedes, Sanjiv Harpavat, Paula M Hertel, Ghadir Sasa, Susan Kirk, Kalyani Patel, Krupa R Mysore

Faculty, Staff and Students Publications

Herbal and dietary supplements (HDS) are a common etiology of drug induced liver injury and, specifically, Herbalife® supplements have been implicated. Hepatitis associated aplastic anemia (HAAA) is a rare and potentially fatal complication after acute hepatitis characterized by pancytopenia. While there have been rare cases of HDS leading to HAAA, no cases of Herbalife® induced liver injury leading to HAAA have been reported from this specific HDS. We report a unique case of severe aplastic anemia developing after sub-fulminant liver failure associated with chronic HDS use. This case illustrates the importance of warning the public about HDS as their use …

Improving Adherence To Clinical Practice Guidelines For Managing Gastric Intestinal Metaplasia Among Gastroenterologists At A Us Academic Institution, Jake Jacob, Valentine Millien, Scott Berger, Ruben Hernaez, Gyanprakash A Ketwaroo, Avegail G Flores, Jason K Hou, Maria E Jarbrink-Sehgal, Natalia I Khalaf, Daniel G Rosen, Hashem B El-Serag, Mimi C Tan

Faculty, Staff and Students Publications

BACKGROUND: Clinical guidelines reserve endoscopic surveillance after a gastric intestinal metaplasia (GIM) diagnosis for high-risk patients. However, it is unclear how closely guidelines are followed in clinical practice. We examined the effectiveness of a standardized protocol for the management of GIM among gastroenterologists at a US hospital.

METHODS: This was a preintervention and postintervention study, which included developing a protocol and education of gastroenterologists on GIM management. For the preintervention study, 50 patients with GIM were randomly selected from a histopathology database at the Houston VA Hospital between January 2016 and December 2019. For the postintervention study, we assessed change …

Longitudinal Natural History Studies Based On Real-World Data In Rare Diseases: Opportunity And A Novel Approach, Laura Ann Adang, Anjana Sevagamoorthy, Omar Sherbini, Jamie L Fraser, Joshua L Bonkowsky, Francesco Gavazzi, Russel D'Aiello, Nicholson B Modesti, Emily Yu, Sylvia Mutua, Emma Kotes, Justine Shults, Ariel Vincent, Lisa T Emrick, Stephanie Keller, Keith P Van Haren, Sarah Woidill, Isabella Barcelos, Amy Pizzino, Johanna L Schmidt, Florian Eichler, Ali Fatemi, Adeline Vanderver

Faculty, Staff and Students Publications

Growing interest in therapeutic development for rare diseases necessitate a systematic approach to the collection and curation of natural history data that can be applied consistently across this group of heterogenous rare diseases. In this study, we discuss the challenges facing natural history studies for leukodystrophies and detail a novel standardized approach to creating a longitudinal natural history study using existing medical records.

Prospective studies are uniquely challenging for rare diseases. Delays in diagnosis and overall rarity limit the timely collection of natural history data. When feasible, prospective studies are often cross-sectional rather than longitudinal and are unlikely to capture …

Immunotherapy Withdrawal By Step-Down To Mesalamine In Pediatric Patients With Ulcerative Colitis, Reka Szigeti, Richard Kellermayer

Faculty, Staff and Students Publications

OBJECTIVE: Parents and pediatric patients with ulcerative colitis (UC) who progressed to systemic immunotherapy are concerned about lifelong risks from such treatments. There is limited knowledge about withdrawal of such agents and step-down (SD) to enteral 5-aminosalicylic acid (mesalamine) before transitioning to adult care.

METHODS: We studied nine pediatric cases with moderate to severe UC who after a median of 2.18 years of clinical remission on systemic immunotherapy stepped down to oral mesalamine treatment.

RESULTS: Average follow-up time from SD was 3.49 years. Five patients (55.5%) had sustained remission (without any flare noted) after SD during follow-up. Sustained clinical remission …

Exposure To 4,4′-Dde In Visceral Adipose Tissue And Weight Loss In Adolescents From The Teen-Labs Cohort, Bruna Rubbo, Zhenjiang Li, Phum Tachachartvanich, Brittney O Baumert, Hongxu Wang, Shudi Pan, Sarah Rock, Justin R Ryder, Todd Jenkins, Stephanie Sisley, Xiangping Lin, Scott Bartell, Thomas H Inge, Stavra Xanthakos, Brooklynn Mcneil, Anna R Robuck, Michele A La Merrill, Douglas I Walker, David V Conti, Rob Mcconnell, Sandrah P Eckel, Lida Chatzi

Faculty, Staff and Students Publications

Objective: Dichlorodiphenyldichloroethylene (DDE), an obesogen accumulating in adipose tissue, is released into circulation with weight loss, although its impact is underexplored among adolescents. We tested the association using an integrative translational approach of epidemiological analysis among adolescents with obesity and in vitro measures exploring the impact of DDE on adipogenesis via preadipocytes.

Methods: We included 63 participants from the Teen-Longitudinal Assessment of Bariatric Surgery (Teen-LABS) cohort. We assessed 4,4'-DDE in visceral adipose tissue at surgery and BMI and waist circumference at surgery and 0.5, 1, 3, and 5 years after. We conducted longitudinal analysis to estimate the interaction on weight …

Sars-Cov-2 Viral Load In The Nasopharynx At Time Of First Infection Among Unvaccinated Individuals: A Secondary Cross-Protocol Analysis Of 4 Randomized Trials, Leigh H Fisher, Jia Jin Kee, Albert Liu, Claudia M Espinosa, April K Randhawa, James Ludwig, Craig A Magaret, Samuel T Robinson, Peter B Gilbert, Ollivier Hyrien, James G Kublin, Nadine Rouphael, Ann R Falsey, Magdalena E Sobieszczyk, Hana M El Sahly, Beatriz Grinsztejn, Glenda E Gray, Karen L Kotloff, Cynthia L Gay, Brett Leav, Ian Hirsch, Frank Struyf, Lisa M Dunkle, Kathleen M Neuzil, Lawrence Corey, Yunda Huang, Paul A Goepfert, Stephen R Walsh, Lindsey R Baden, Holly Janes, Covid-19 Prevention Network (Covpn)

Faculty, Staff and Students Publications

IMPORTANCE: SARS-CoV-2 viral load (VL) in the nasopharynx is difficult to quantify and standardize across settings, but it may inform transmission potential and disease severity.

OBJECTIVE: To characterize VL at COVID-19 diagnosis among previously uninfected and unvaccinated individuals by evaluating the association of demographic and clinical characteristics, viral variant, and trial with VL, as well as the ability of VL to predict severe disease.

DESIGN, SETTING, AND PARTICIPANTS: This secondary cross-protocol analysis used individual-level data from placebo recipients from 4 harmonized, phase 3 COVID-19 vaccine efficacy trials sponsored by Moderna, AstraZeneca, Janssen, and Novavax. Participants were SARS-CoV-2 negative at baseline …

Gut Development Following Insulin-Like Growth Factor-1 Supplementation To Preterm Pigs, Martin Bo Rasmussen, Kristine Holgersen, Stanislava Pankratova, Ole Bæk, Douglas G Burrin, Thomas Thymann, Per Torp Sangild

Faculty, Staff and Students Publications

BACKGROUND: Reduced insulin-like growth factor-1 (IGF-1) levels may contribute to impaired organ development in preterm infants. Using preterm pigs as a model, we hypothesized that IGF-1 supplementation improves health and gut development during the first three weeks of life.

METHODS: First, clinical and organ endpoints were compared between artificially-reared, cesarean-delivered preterm pigs and vaginally-delivered, sow-reared term pigs at 5, 9 and 19 days. Next, preterm pigs were treated with recombinant human IGF-1 for 19 days (2.25 mg/kg/day, systemically).

RESULTS: Relative to term pigs, preterm pigs had lower body weight, fat, bone contents, relative weights of liver and spleen and a …

Patterns Of Multiple Organ Dysfunction And Renal Recovery In Critically Ill Children And Young Adults Receiving Continuous Renal Replacement Therapy, Sameer Thadani, Dana Fuhrman, Claire Hanson, Hyun Jung Park, Joseph Angelo, Poyyapakkam Srivaths, Katri Typpo, Michael J Bell, Katja M Gist, Joseph Carcillo, Ayse Akcan-Arikan

Faculty, Staff and Students Publications

OBJECTIVES: Acute kidney injury requiring dialysis (AKI-D) commonly occurs in the setting of multiple organ dysfunction syndrome (MODS). Continuous renal replacement therapy (CRRT) is the modality of choice for AKI-D. Mid-term outcomes of pediatric AKI-D supported with CRRT are unknown. We aimed to describe the pattern and impact of organ dysfunction on renal outcomes in critically ill children and young adults with AKI-D.

DESIGN: Retrospective cohort.

SETTING: Two large quarternary care pediatric hospitals.

PATIENTS: Patients 26 y old or younger who received CRRT from 2014 to 2020, excluding patients with chronic kidney disease.

INTERVENTIONS: None.

MEASUREMENTS AND MAIN RESULTS: Organ …

Retrospective Analysis Of Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome In Paediatric Patients Undergoing Hematopoietic Cell Transplantation -A Multicentre Study, Dristhi Ragoonanan, Hisham Abdel-Azim, Aditya Sharma, Saleh Bhar, Jennifer Mcarthur, Renee Madden, April Rahrig, Rajinder Bajwa, Jian Wang, Victoria Sun, Mariah Wright, Rebekah Lassiter, Basirat Shoberu, Jitesh Kawedia, Sajad Jawad Khazal, Kris Michael Mahadeo, Pediatric Acute Lung Injury And Sepsis Investigators (Palisi) Network

Faculty, Staff and Students Publications

BACKGROUND: Sinusoidal obstruction syndrome is a potentially fatal complication following hematopoietic cell transplantation, high-intensity chemotherapies and increasingly seen with calicheamicin based leukemia therapies. Paediatric specific European Society for Blood and Marrow Transplantation (pEBMT) diagnostic criteria have demonstrated benefit in single center studies compared to historic criteria. Yet, the extent to which they have been universally implemented remains unclear.

METHODS: We conducted a retrospective multi-centre study to examine the potential impact of the Baltimore, modified Seattle and pEBMT criteria on the incidence, severity, and outcomes of sinusoidal obstruction syndrome among paediatric hematopoietic cell transplantation patients.

FINDINGS: The incidence of sinusoidal obstruction …

Association Of Minor Electrocardiographic (Ecg) Abnormalities With Epilepsy Duration In Children: A Manifestation Of The Epileptic Heart?, Brittnie Bartlett-Lee, Leslie Dervan, Christina Miyake, R Scott Watson, See Wai Chan, Anne E Anderson, Yi-Chen Lai

Faculty, Staff and Students Publications

Purpose: Cardiac abnormalities resulting from chronic epilepsy ("the epileptic heart") constitute a well-recognized comorbidity. However, the association of cardiac alterations with epilepsy duration remains understudied. We sought to evaluate this association using electrocardiogram (ECG).

Methods: We prospectively enrolled children between 1 months and 18 years of age without known cardiac conditions or ion channelopathies during routine clinic visits. ECGs were categorized as abnormal if there were alterations in rhythm; PR, QRS, or corrected QT interval; QRS axis or morphology; ST segment or T wave. An independent association between ECG abnormalities and epilepsy duration was evaluated using multivariable logistic regression modeling. …

The Evidence Is Mounting: Insurance Coverage Disruptions Lead To Worse Outcomes Among Children Diagnosed With Cancer, Philip J Lupo, Christina L Magyar

Faculty, Staff and Students Publications

No abstract provided.

Examining The Relationship Between Anxiety Severity And Autism-Related Challenges During Cognitive Behavioral Therapy For Children With Autism, Madeleine N Fuselier, Andrew G Guzick, Jafar Bakhshaie, Jeffrey J Wood, Philip C Kendall, Connor M Kerns, Brent J Small, Wayne K Goodman, Eric A Storch

Faculty, Staff and Students Publications

PURPOSE: Using data from a randomized clinical trial evaluating cognitive behavioral therapy (CBT) for children with autism and co-occurring anxiety, this study examined the relationship between autism features and anxiety symptoms throughout CBT.

METHODS: Two multilevel mediation analyses were run which examined the mediating role of changes in anxiety for changes in two core features of autism, (a) repetitive and restrictive behaviors (RRBs) and (b) social communication/interaction impairments, between pre- and post-treatment.

RESULTS: Indirect effects between time and autism characteristics were significant for both models, indicating that as anxiety changes, so do RRBs and social communication/interaction as the outcomes respectively. …

Inaccurate Diagnosis Of Diabetes Type In Youth: Prevalence, Characteristics, And Implications, Mustafa Tosur, Xiaofan Huang, Audrey S Inglis, Rebecca Schneider Aguirre, Maria J Redondo

Faculty, Staff and Students Publications

Classifying diabetes at diagnosis is crucial for disease management but increasingly difficult due to overlaps in characteristics between the commonly encountered diabetes types. We evaluated the prevalence and characteristics of youth with diabetes type that was unknown at diagnosis or was revised over time. We studied 2073 youth with new-onset diabetes (median age [IQR] = 11.4 [6.2] years; 50% male; 75% White, 21% Black, 4% other race; overall, 37% Hispanic) and compared youth with unknown versus known diabetes type, per pediatric endocrinologist diagnosis. In a longitudinal subcohort of patients with data for ≥ 3 years post-diabetes diagnosis (n = 1019), …

Water, Sanitation, And Hygiene Infrastructure And Resources In Schools In Belize During The Covid-19 Pandemic, 2021-2023, Anh N Ly, Kelsey Mcdavid, Christina Craig, Dian Maheia, Yolanda Gongora, Alexandra Medley, Francis Morey, Russell Manzanero, Gerhaldine Morazan, Allison Lino, Vickie Romero, Rosalva Blanco, Kanako Ishida, Matthew Lozier, Kristy O Murray

Faculty, Staff and Students Publications

Access to water, sanitation, and hygiene (WASH) resources in schools is critical for disease prevention and control, especially during public health emergencies. In Belize, systematic, national data on WASH in schools are needed to inform public health decisions and interventions. From December 2021 to January 2022, a national survey was sent electronically to government and government-aided primary and secondary schools in Belize (N = 308) to gather information on WASH services. From the survey, 12 pilot schools were selected based on the highest self-reported need for WASH resources to participate in additional evaluation and intervention, which included environmental nudges, supplemental …

Islet Autoantibodies As Precision Diagnostic Tools To Characterize Heterogeneity In Type 1 Diabetes: A Systematic Review, Jamie L Felton, Maria J Redondo, Richard A Oram, Cate Speake, S Alice Long, Suna Onengut-Gumuscu, Stephen S Rich, Gabriela S F Monaco, Arianna Harris-Kawano, Dianna Perez, Zeb Saeed, Benjamin Hoag, Rashmi Jain, Carmella Evans-Molina, Linda A Dimeglio, Heba M Ismail, Dana Dabelea, Randi K Johnson, Marzhan Urazbayeva, John M Wentworth, Kurt J Griffin, Emily K Sims, Ada/Easd Pmdi Collaborators

Faculty, Staff and Students Publications

BACKGROUND: Islet autoantibodies form the foundation for type 1 diabetes (T1D) diagnosis and staging, but heterogeneity exists in T1D development and presentation. We hypothesized that autoantibodies can identify heterogeneity before, at, and after T1D diagnosis, and in response to disease-modifying therapies.

METHODS: We systematically reviewed PubMed and EMBASE databases (6/14/2022) assessing 10 years of original research examining relationships between autoantibodies and heterogeneity before, at, after diagnosis, and in response to disease-modifying therapies in individuals at-risk or within 1 year of T1D diagnosis. A critical appraisal checklist tool for cohort studies was modified and used for risk of bias assessment.

RESULTS: …

A Scoping Review Of Artificial Intelligence In Medical Education: Beme Guide No 84, Morris Gordon, Michelle Daniel, Aderonke Ajiboye, Hussein Uraiby, Nicole Y Xu, Rangana Bartlett, Janice Hanson, Mary Haas, Maxwell Spadafore, Ciaran Grafton-Clarke, Rayhan Yousef Gasiea, Colin Michie, Janet Corral, Brian Kwan, Diana Dolmans, Satid Thammasitboon

Faculty, Staff and Students Publications

Background: Artificial Intelligence (AI) is rapidly transforming healthcare, and there is a critical need for a nuanced understanding of how AI is reshaping teaching, learning, and educational practice in medical education. This review aimed to map the literature regarding AI applications in medical education, core areas of findings, potential candidates for formal systematic review and gaps for future research.

Methods: This rapid scoping review, conducted over 16 weeks, employed Arksey and O'Malley's framework and adhered to STORIES and BEME guidelines. A systematic and comprehensive search across PubMed/MEDLINE, EMBASE, and MedEdPublish was conducted without date or language restrictions. Publications included in …

Outcomes Of Extracorporeal Cardiopulmonary Resuscitation For In-Hospital Cardiac Arrest Among Children With Noncardiac Illness Categories, Morgann Loaec, Adam S Himebauch, Ron Reeder, Jessica S Alvey, Jonathan A Race, Lillian Su, Javier J Lasa, Julia C Slovis, Tia T Raymond, Ryan Coleman, Bradley J Barney, Todd J Kilbaugh, Alexis A Topjian, Robert M Sutton, Ryan W Morgan

Faculty, Staff and Students Publications

Objectives: The objective of this study was to determine the association of the use of extracorporeal cardiopulmonary resuscitation (ECPR) with survival to hospital discharge in pediatric patients with a noncardiac illness category. A secondary objective was to report on trends in ECPR usage in this population for 20 years.

Design: Retrospective multicenter cohort study.

Setting: Hospitals contributing data to the American Heart Association's Get With The Guidelines-Resuscitation registry between 2000 and 2021.

Patients: Children (< 18 yr) with noncardiac illness category who received greater than or equal to 30 minutes of cardiopulmonary resuscitation (CPR) for in-hospital cardiac arrest.

Interventions: None.

Measurements and main results: Propensity score weighting balanced ECPR and conventional CPR (CCPR) groups on hospital and patient characteristics. Multivariable logistic regression incorporating …

Neurocritical Care And Neuromonitoring Considerations In Acute Pediatric Spinal Cord Injury, Ajay X Thomas, Jennifer C Erklauer

Faculty, Staff and Students Publications

Management of pediatric spinal cord injury (SCI) is an essential skill for all pediatric neurocritical care physicians. In this review, we focus on the evaluation and management of pediatric SCI, highlight a novel framework for the monitoring of such patients in the intensive care unit (ICU), and introduce advancements in critical care techniques in monitoring and management. The initial evaluation and characterization of SCI is crucial for improving outcomes as well as prognostication. While physical examination and imaging are the main stays of the work-up, we propose the use of somatosensory evoked potentials (SSEPs) and transcranial magnetic stimulation (TMS) for …

The Impact Of Cognitive Behavioral Therapy On Sleep Problems In Autistic Children With Co-Occurring Anxiety, Holly K Harris, Minjee Kook, Peter Boedeker, Andrew G Gusick, Ariel M Lyons-Warren, Robin P Goin-Kochel, Chaya Murali, Leandra N Berry, Eric A Storch

Faculty, Staff and Students Publications

Purpose: This study seeks to examine the relationship between anxiety-symptom severity and sleep behaviors in autistic children receiving cognitive behavioral therapy (CBT).

Methods: We conducted a secondary-data analysis from a sample of 93 autistic youth, 4 to 14 years, participating in 24 weeks of CBT. Clinicians completed the Pediatric Anxiety Rating Scale (PARS) and parents completed the Children's Sleep Habits Questionnaire, Abbreviated/Short Form (CSHQ-SF) at baseline, mid-treatment, post-treatment and 3 months post-treatment. Mediation analysis evaluated the role of anxiety symptoms in mediating the effect of time in treatment on sleep.

Results: There was a negative association between time in treatment …

Sensory Experiences Questionnaire Unravels Differences In Sensory Profiles Between Mecp2-Related Disorders, Bernhard Suter, Davut Pehlivan, Muharrem Ak, Holly K Harris, Ariel M Lyons-Warren

Faculty, Staff and Students Publications

The methyl CpG-binding protein-2 (MECP2) gene is located on the Xq28 region. Loss of function mutations or increased copies of MECP2 result in Rett syndrome (RTT) and MECP2 duplication syndrome (MDS), respectively. Individuals with both disorders exhibit overlapping autism symptoms, yet few studies have dissected the differences between these gene dosage sensitive disorders. Further, research examining sensory processing patterns in persons with RTT and MDS is largely absent. Thus, the goal of this study was to analyze and compare sensory processing patterns in persons with RTT and MDS. Towards this goal, caregivers of 50 female individuals with RTT and 122 …

Relevance Of Lymphocyte Proliferation To Pha In Severe Combined Immunodeficiency (Scid) And T Cell Lymphopenia, Roshini S Abraham, Amrita Basu, Jennifer R Heimall, Elizabeth Dunn, Alison Yip, Malika Kapadia, Neena Kapoor, Lisa Forbes Satter, Rebecca Buckley, Richard O'Reilly, Geoffrey D E Cuvelier, Sharat Chandra, Jeffrey Bednarski, Sonali Chaudhury, Theodore B Moore, Hilary Haines, Blachy J Dávila Saldaña, Deepakbabu Chellapandian, Ahmad Rayes, Karin Chen, Emi Caywood, Shanmuganathan Chandrakasan, Mark Thomas Vander Lugt, Christen Ebens, Pierre Teira, Evan Shereck, Holly Miller, Victor Aquino, Hesham Eissa, Lolie C Yu, Alfred Gillio, Lisa Madden, Alan Knutsen, Ami J Shah, Kenneth Desantes, Jessie Barnum, Larisa Broglie, Avni Y Joshi, Gary Kleiner, Jasmeen Dara, Susan Prockop, Caridad Martinez, Talal Mousallem, Joseph Oved, Lauri Burroughs, Rebecca Marsh, Troy R Torgerson, Jennifer W Leiding, Sung Yun Pai, Donald B Kohn, Michael A Pulsipher, Linda M Griffith, Luigi D Notarangelo, Morton J Cowan, Jennifer Puck, Christopher C Dvorak, Elie Haddad

Faculty, Staff and Students Publications

Severe combined immunodeficiency (SCID) is characterized by a severe deficiency in T cell numbers. We analyzed data collected (n = 307) for PHA-based T cell proliferation from the PIDTC SCID protocol 6901, using either a radioactive or flow cytometry method. In comparing the two groups, a smaller number of the patients tested by flow cytometry had < 10% of the lower limit of normal proliferation as compared to the radioactive method (p = 0.02). Further, in patients with CD3+ T cell counts between 51 and 300 cells/μL, there was a higher proliferative response with the PHA flow assay compared to the 3H-T assay (p < 0.0001), suggesting that the method of analysis influences the resolution and interpretation of PHA results. Importantly, we observed many SCID patients with profound T cell lymphopenia having normal T cell proliferation when assessed by flow cytometry. We recommend this test be considered only as supportive in the diagnosis of typical SCID.

Current And Future Perspectives On The Consensus Guideline For Food Protein-Induced Enterocolitis Syndrome (Fpies), Sara Anvari, Melanie A Ruffner, Anna Nowak-Wegrzyn

Faculty, Staff and Students Publications

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated food allergy presenting with delayed onset of projectile vomiting in the absence of cutaneous and respiratory symptoms. The pathophysiology of FPIES remains poorly characterized. The first international consensus guidelines for FPIES were published in 2017 and provided clinicians with parameters on the diagnosis and treatment of FPIES. The guidelines have served as a resource in the recognition and management of FPIES, contributing to an increased awareness of FPIES. Since then, new evidence has emerged, shedding light on adult-onset FPIES, the different phenotypes of FPIES, the recognition of new food triggers, center-specific …

Scurvy In An Adolescent: A Case Report, Tony Du, Abhay Kulkarni

Faculty, Staff and Students Publications

Scurvy is a rare condition characterized by a deficiency in dietary vitamin C. Historically a disease taught in the context of long ocean voyages with limited vitamin intake, it is now rare in developed nations. The classical physical exam findings include gingival bleeding, perifollicular hemorrhages, and corkscrew hairs. We discuss the case of a 15-year-old female with scurvy whose initial presentation suggested more common diagnoses seen in the emergency department setting. Her course was complicated by a prior history of anorexia nervosa and a restrictive diet that lacked necessary vitamins. Once the patient's dietary habits were identified, a detailed physical …