Medicine and Health Sciences Commons^™

State Of The Art Bowel Management For Pediatric Colorectal Problems: Hirschsprung Disease., Elizaveta Bokova, Ninad Prasade, Sanjana Janumpally, John Rosen, Irene Isabel P. Lim, Marc A. Levitt, Rebecca M. Rentea

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After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with …

One Size Does Not Fit All: Advanced Practice Provider Considerations For The Antimicrobial Steward., Kyleen E. Swords, Gina Weddle, Joshua C. Herigon, Paula D. Stering, Matthew S L Lee

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Advanced practice providers are a diverse and established group of antimicrobial prescribers in both ambulatory and inpatient settings. We outline important considerations for antimicrobial stewardship programs and stewards to consider when engaging this important group of providers.

Implementing Text-Messaging To Support And Enhance Delivery Of Health Behavior Change Interventions In Low- To Middle-Income Countries: Case Study Of The Lifestyle Africa Intervention., Frank T. Materia, Joshua M. Smyth, Thandi Puoane, Lungiswa Tsolekile, Kathy Goggin, Stephen R. Kodish, Andrew T. Fox, Ken Resnicow, Scott Werntz, Delwyn Catley

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The prevalence of non-communicable diseases, such as diabetes and cardiovascular disease, is rising in low- and middle-income countries (LMICs). Health behavior change (HBC) interventions such as the widely used Diabetes Prevention Program (DPP) are effective at reducing chronic disease risk, but have not been adapted for LMICs. Leveraging mobile health (mHealth) technology such as text messaging (SMS) to enhance reach and participant engagement with these interventions has great promise, yet we lack evidence-informed approaches to guide the integration of SMS specifically to support HBC interventions in LMIC contexts. To address this gap, we integrated guidance from the mHealth literature with …

Associations Of Biomarkers Of Kidney Tubule Health, Injury, And Inflammation With Left Ventricular Hypertrophy In Children With Ckd., Kuan Jiang, Jason H. Greenberg, Alison Abraham, Yunwen Xu, Jeffrey R. Schelling, Harold I. Feldman, Sarah J. Schrauben, Sushrut S. Waikar, Michael G. Shlipak, Nicholas Wettersten, Steven G. Coca, Ramachandran S. Vasan, Orlando M. Gutierrez, Joachim H. Ix, Bradley A. Warady, Paul L. Kimmel, Joseph V. Bonventre, Chirag R. Parikh, Mark M. Mitsnefes, Michelle R. Denburg, Susan Furth, Ckd Biomarkers Consortium

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KEY POINTS:

• Higher plasma and urine kidney injury molecule-1, urine monocyte chemoattractant protein-1, and lower urine alpha-1-microglobulin were associated with left ventricular hypertrophy, even after adjustment for confounders.
• Biomarkers of tubular injury, dysfunction, and inflammation may indicate the severity of kidney pathology and are associated with left ventricular hypertrophy.

BACKGROUND: Left ventricular hypertrophy (LVH) is common in children with CKD and is associated with an increased risk of cardiovascular disease and mortality. We have shown that several plasma and urine biomarkers are associated with increased risk of CKD progression. As CKD is associated with LVH, we sought to investigate the …

Don't Look Back And Spray! Risk Of Endoscope Adhesion With Hemospray Application In Retroflexion In A Pediatric Patient., Sarah T. Edwards, Diana G. Lerner, Moises Alatorre-Jimenez, Thomas M. Attard

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Although the vast majority of recognized pediatric upper gastrointestinal bleeding (GIB) resolves spontaneously, gastrointestinal hemorrhage is the most common indication for urgent or emergent therapeutic endoscopy in pediatric practice. The application of hemostatic powders, including TC-325 (Hemospray, Cook Medical, Winston-Salem, NC, USA), has shown considerable impact on the control of acute bleeding, with the advantage of potentially covering an extensive area and requiring less technical expertise. We report a case of transient adherence of an esophagogastroduodenoscopy following Hemospray application in a 22-month-old with upper GIB. Our experience does not detract from the significant gains in the management of pediatric GIB …

Infants Admitted To Us Intensive Care Units For Rsv Infection During The 2022 Seasonal Peak., Natasha Halasa, Laura D. Zambrano, Justin Z. Amarin, Laura S. Stewart, Margaret M. Newhams, Emily R. Levy, Steven L. Shein, Christopher L. Carroll, Julie C. Fitzgerald, Marian G. Michaels, Katherine Bline, Melissa L. Cullimore, Laura Loftis, Vicki L. Montgomery, Asumthia S. Jeyapalan, Pia S. Pannaraj, Adam J. Schwarz, Natalie Z. Cvijanovich, Matt S. Zinter, Aline B. Maddux, Melania M. Bembea, Katherine Irby, Danielle M. Zerr, Joseph D. Kuebler, Christopher J. Babbitt, Mary Glas Gaspers, Ryan A. Nofziger, Michele Kong, Bria M. Coates, Jennifer E. Schuster, Shira J. Gertz, Elizabeth H. Mack, Benjamin R. White, Helen Harvey, Charlotte V. Hobbs, Heda Dapul, Andrew D. Butler, Tamara T. Bradford, Courtney M. Rowan, Kari Wellnitz, Mary Allen Staat, Cassyanne L. Aguiar, Saul R. Hymes, Adrienne G. Randolph, Angela P. Campbell, Rsv-Pic Investigators

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IMPORTANCE: Respiratory syncytial virus (RSV) is the leading cause of lower respiratory tract infections (LRTIs) and infant hospitalization worldwide.

OBJECTIVE: To evaluate the characteristics and outcomes of RSV-related critical illness in US infants during peak 2022 RSV transmission.

DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional study used a public health prospective surveillance registry in 39 pediatric hospitals across 27 US states. Participants were infants admitted for 24 or more hours between October 17 and December 16, 2022, to a unit providing intensive care due to laboratory-confirmed RSV infection.

EXPOSURE: Respiratory syncytial virus.

MAIN OUTCOMES AND MEASURES: Data were captured on demographics, …

The Evolving Landscape Of Immunotherapy For The Treatment Of Allergic Conditions., Aarti Pandya, Esosa Adah, Bridgette Jones, Rachel Chevalier

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Allergic conditions, such as asthma, chronic urticaria, atopic dermatitis (AD), and eosinophilic esophagitis, have long been treated with oral and topical steroids which resulted in negative off-target effects. However, newer biologic medications are increasingly being developed and approved for treatment of these conditions. These medications have a variety of mechanisms of action to target pathophysiology specific to these diseases. As biologics become more targeted, fewer off-target effects are seen improving tolerability for patients as well as expanded options for treatment of these conditions. This review discusses monoclonal antibody therapies (omalizumab, mepolizumab, reslizumab, benralizumab, dupilumab, tezepelumab, and tralokinumab) including their safety …

Sars-Cov-2 Infections In Patients Enrolled On The Children's Oncology Group Standard-Risk B-Cell Acute Lymphoblastic Leukemia Trial, Aall1731., Caitlin W. Elgarten, John A. Kairalla, Joel Thompson, Tamara P. Miller, Cindy Wang, Susan Conway, Mignon L. Loh, Elizabeth A. Raetz, Sumit Gupta, Rachel E. Rau, Anne Angiolillo, Karen R. Rabin, Sarah Alexander

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Hematologic malignancy is a risk factor for severe coronavirus disease 2019 (COVID-19) in adults; however, data specific to children with leukemia are limited. High-quality infectious adverse event data from the ongoing Children's Oncology Group (COG) standard-risk B acute lymphoblastic leukemia/lymphoma (ALL/LLy) trial, AALL1731, were analyzed to provide a disease-specific estimate of SARS-CoV-2 infection outcomes in pediatric ALL. Of 253 patients with reported infections, the majority (77.1%) were asymptomatic or mildly symptomatic (CTCAE grade 1/2) and there was a single COVID-19-related death. These data suggest SARS-CoV-2 infection does not confer substantial morbidity among young patients with B-lymphoblastic leukemia/lymphoma (B-ALL/LLy).

Comprehensive Molecular And Clinical Characterization Of Nup98 Fusions In Pediatric Acute Myeloid Leukemia, Eline J M Bertrums, Jenny L. Smith, Lauren Harmon, Rhonda E. Ries, Yi-Cheng J. Wang, Todd A. Alonzo, Andrew J. Menssen, Karen M. Chisholm, Amanda R. Leonti, Katherine Tarlock, Fabiana Ostronoff, Era L. Pogosova-Agadjanyan, Gertjan J L Kaspers, Henrik Hasle, Michael Dworzak, Christiane Walter, Nora Muhlegger, Cristina Morerio, Laura Pardo, Betsy Hirsch, Susana Raimondi, Todd M. Cooper, Richard Aplenc, Alan S. Gamis, Edward A. Kolb, Jason E. Farrar, Derek Stirewalt, Xiaotu Ma, Tim I. Shaw, Scott N. Furlan, Lisa Eidenschink Brodersen, Michael R. Loken, Marry M. Van Den Heuvel-Eibrink, C Michel Zwaan, Timothy J. Triche, Bianca F. Goemans, Soheil Meshinchi

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NUP98 fusions comprise a family of rare recurrent alterations in AML, associated with adverse outcomes. In order to define the underlying biology and clinical implications of this family of fusions, we performed comprehensive transcriptome, epigenome, and immunophenotypic profiling of 2,235 children and young adults with AML and identified 160 NUP98 rearrangements (7.2%), including 108 NUP98-NSD1 (4.8%), 32 NUP98-KDM5A (1.4%) and 20 NUP98-X cases (0.9%) with 13 different fusion partners. Fusion partners defined disease characteristics and biology; patients with NUP98-NSD1 or NUP98-KDM5A had distinct immunophenotypic, transcriptomic, and epigenomic profiles. Unlike the two most prevalent NUP98 fusions, NUP98-X variants are typically not …

Prevalence, Management And Efficacy Of Treatment In Portal Vein Obstruction After Paediatric Liver Transplantation: Protocol Of The Retrospective International Multicentre Portal Registry., Bader A. Alfares, Hubert P J Van Der Doef, Barbara E. Wildhaber, Thomas Casswall, Greg Nowak, Martin Delle, Denise Aldrian, Valeria Berchtold, Georg F. Vogel, Piotr Kaliciński, Malgorzata Markiewicz-Kijewska, Adam Kolesnik, Jesús Q. Bernabeu, María Mercadal Hally, Mauricio Larrarte K, Paolo Marra, Michela Bravi, Domenico Pinelli, Mureo Kasahara, Seisuke Sakamoto, Hajime Uchida, Vidyadhar Mali, Marion Aw, Stéphanie Franchi-Abella, Emmanuel Gonzales, Florent Guérin, Guillermo Cervio, Julia Minetto, Sergio Sierre, Martin De Santibañes, Victoria Ardiles, Jimmy Walker Uno, Helen Evans, David Duncan, John Mccall, Steffen Hartleif, Ekkehard Sturm, Jai Patel, Marumbo Mtegha, Raj Prasad, Cristina T. Ferreira, Luiza S. Nader, Marco Farina, Catalina Jaramillo, Manuel I. Rodriguez-Davalos, Peter Feola, Amit A. Shah, Phoebe M. Wood, Michael R. Acord, Ryan T. Fischer, Bhargava Mullapudi, Richard J. Hendrickson, Rajeev Khanna, Viniyendra Pamecha, Amar Mukund, Khalid Sharif, Girish Gupte, Simon Mcguirk, Gilda Porta, Marco Spada, Tommaso Alterio, Giuseppe Maggiore, Winita Hardikar, Marisa Beretta, Rudi Dierckx, Ruben H J De Kleine, Reinoud P H Bokkers

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INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO.

METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (agedyears) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are …

An "All-Data-On-Hand" Deep Learning Model To Predict Hospitalization For Diabetic Ketoacidosis In Youth With Type 1 Diabetes: Development And Validation Study., David D. Williams, Diana Ferro, Colin Mullaney, Lydia Skrabonja, Mitchell Barnes, Susana R. Patton, Brent Lockee, Erin M. Tallon, Craig Vandervelden, Cintya Schweisberger, Sanjeev Mehta, Ryan Mcdonough, Marcus Lind, Leonard D'Avolio, Mark A. Clements

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BACKGROUND: Although prior research has identified multiple risk factors for diabetic ketoacidosis (DKA), clinicians continue to lack clinic-ready models to predict dangerous and costly episodes of DKA. We asked whether we could apply deep learning, specifically the use of a long short-term memory (LSTM) model, to accurately predict the 180-day risk of DKA-related hospitalization for youth with type 1 diabetes (T1D).

OBJECTIVE: We aimed to describe the development of an LSTM model to predict the 180-day risk of DKA-related hospitalization for youth with T1D.

METHODS: We used 17 consecutive calendar quarters of clinical data (January 10, 2016, to March 18, …

Notes From The Field: Multipathogen Respiratory Virus Testing Among Primary And Secondary School Students And Staff Members In A Large Metropolitan School District - Missouri, November 2, 2022-April 19, 2023., Jennifer Goldman, Brian R. Lee, Janelle Porter, Anila Deliu, Shannon Tilsworth, Olivia M. Almendares, Sadia Sleweon, Hannah L. Kirking, Rangaraj Selvarangan, Jennifer E. Schuster

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No abstract provided.

The Building Healthy Eating And Self-Esteem Together For University Students Mobile App To Treat Eating Disorders: User-Centered Research Design And Feasibility Study., Kelsie T. Forbush, Kara A. Christensen Pacella, Marianna L. Thomeczek, Sara R. Gould, Danielle A N Chapa, Brianne N. Richson, Victoria L. Perko, Joseph Ayres, Yiyang Chen, Sonakshi Negi

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BACKGROUND: University students are an at-risk group for the development of eating disorders (EDs); however, many college campuses lack sufficient resources to provide ED specialty care. Students report unique reasons for not seeking ED treatment, including the desire to solve the problem on their own (eg, seeking help from friends, self-medicating, or waiting to see if their problems improve), inability to afford treatment, lack of time to participate in the treatment, fear of seeing their primary care physician, and lack of recognition of their issues as an ED. Mobile health (mHealth) apps may be a cost-effective, helpful adjunctive tool to …

Potential Biomarker Of Brain Response To Opioid Antagonism In Adolescents With Eating Disorders: A Pilot Study., Stephani L. Stancil, Hung-Wen Yeh, Morgan G. Brucks, Amanda S. Bruce, Michaela Voss, Susan Abdel-Rahman, William M. Brooks, Laura E. Martin

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BACKGROUND: Eating Disorders (ED) affect up to 5% of youth and are associated with reward system alterations and compulsive behaviors. Naltrexone, an opioid antagonist, is used to treat ED behaviors such as binge eating and/or purging. The presumed mechanism of action is blockade of reward activation; however, not all patients respond, and the optimal dose is unknown. Developing a tool to detect objective drug response in the brain will facilitate drug development and therapeutic optimization. This pilot study evaluated neuroimaging as a pharmacodynamic biomarker of opioid antagonism in adolescents with ED.

METHODS: Youth aged 13-21 with binge/purge ED completed functional …

Developing A National-Scale Exposure Index For Combined Environmental Hazards And Social Stressors And Applications To The Environmental Influences On Child Health Outcomes (Echo) Cohort., Sheena E. Martenies, Mingyu Zhang, Anne E. Corrigan, Anton Kvit, Timothy Shields, William Wheaton, Deana Around Him, Judy Aschner, Maria M. Talavera-Barber, Emily S. Barrett, Theresa M. Bastain, Casper Bendixsen, Carrie V. Breton, Nicole R. Bush, Ferdinand Cacho, Carlos A. Camargo, Kecia N. Carroll, Brian S. Carter, Andrea E. Cassidy-Bushrow, Whitney Cowell, Lisa A. Croen, Dana Dabelea, Cristiane S. Duarte, Anne L. Dunlop, Todd M. Everson, Rima Habre, Tina V. Hartert, Jennifer B. Helderman, Alison E. Hipwell, Margaret R. Karagas, Barry M. Lester, Kaja Z. Lewinn, Sheryl Magzamen, Rachel Morello-Frosch, Thomas G. O'Connor, Amy M. Padula, Michael Petriello, Sheela Sathyanarayana, Joseph B. Stanford, Tracey J. Woodruff, Rosalind J. Wright, Amii M. Kress, Program Collaborators For Environmental Influences On Child Health Outcomes

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Tools for assessing multiple exposures across several domains (e.g., physical, chemical, and social) are of growing importance in social and environmental epidemiology because of their value in uncovering disparities and their impact on health outcomes. Here we describe work done within the Environmental influences on Child Health Outcomes (ECHO)-wide Cohort Study to build a combined exposure index. Our index considered both environmental hazards and social stressors simultaneously with national coverage for a 10-year period. Our goal was to build this index and demonstrate its utility for assessing differences in exposure for pregnancies enrolled in the ECHO-wide Cohort Study. Our unitless …

Overactive Bladder Syndrome Symptoms In Youth With Abdominal Pain-Associated Disorders Of Gut-Brain Interaction., Hunter J. Friesen, Pierce Thompson, Jennifer Verrill Schurman, Jennifer Colombo, Craig A. Friesen

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The purpose of the current study was to assess the frequency of overactive bladder syndrome (OBS) symptoms and their relationship to gastrointestinal symptoms in youth with abdominal pain-associated disorders of gut-brain interaction (AP-DGBI). This is a retrospective study of 226 youth diagnosed with an AP-DGBI. As part of standard care, all patients completed a symptom questionnaire regarding gastrointestinal and non-gastrointestinal symptoms including increased urinary frequency, nighttime urination, and urinary urgency. Overall, 54% of patients reported at least one OBS symptom. Increased frequency of urination was reported by 19%, urinary urgency by 34%, and nighttime urination by 36%. Increased frequency of …

Prognostic And Predictive Value Of Endothelial Dysfunction Biomarkers In Sepsis-Associated Acute Kidney Injury: Risk-Stratified Analysis From A Prospective Observational Cohort Of Pediatric Septic Shock., Mihir R. Atreya, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Scott L. Weiss, Michael T. Bigham, Parag N. Jain, Adam J. Schwarz, Riad Lutfi, Jeffrey Nowak, Geoffrey L. Allen, Neal J. Thomas, Jocelyn R. Grunwell, Torrey Baines, Michael Quasney, Bereketeab Haileselassie, Matthew N. Alder, Stuart L. Goldstein, Natalja L. Stanski

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BACKGROUND: Sepsis-associated acute kidney injury (SA-AKI) is associated with high morbidity, with no current therapies available beyond continuous renal replacement therapy (CRRT). Systemic inflammation and endothelial dysfunction are key drivers of SA-AKI. We sought to measure differences between endothelial dysfunction markers among children with and without SA-AKI, test whether this association varied across inflammatory biomarker-based risk strata, and develop prediction models to identify those at highest risk of SA-AKI.

METHODS: Secondary analyses of prospective observational cohort of pediatric septic shock. Primary outcome of interest was the presence of ≥ Stage II KDIGO SA-AKI on day 3 based on serum creatinine …

Oropharyngeal Teratoma: Five-Month-Old Presenting With Failure To Thrive And Severe Obstructive Sleep Apnea., Anna Lawrence, Melissa Gener, Shao Jiang, Jill M. Arganbright

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Oropharyngeal teratomas are an extremely rare congenital tumor. They are often diagnosed prenatally and can cause significant airway obstruction and feeding difficulties at birth. We present a five-month-old female that was diagnosed with a palatal teratoma that presented with failure to thrive, difficulty feeding, and eventually with severe obstructive sleep apnea. We present a five-month-old term, otherwise healthy female who became stridulous after an episode of the respiratory syncytial virus at one month old. At three months old, an otolaryngologist diagnosed mild laryngomalacia with no mass identified, and no surgical intervention was recommended. Due to continued poor weight gain, at …

Treatment Of Covid-19 Symptoms With Over The Counter (Otc) Medicines Used For Treatment Of Common Cold And Flu., Ronald Eccles, Guy Boivin, Benjamin J. Cowling, Andrew Pavia, Rangaraj Selvarangan

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Persons suffering from acute upper respiratory tract viral infections (URTI) commonly use over the counter (OTC) medicines to relieve symptoms such as fever, muscle aches, cough, runny nose, sore throat and nasal congestion. At present OTC medicines are only licensed for treatment of common cold and flu symptoms and not for treatment of the same symptoms associated with COVID-19. The innate immune response responsible for the mechanisms of the symptoms of URTI is the same for all respiratory viruses including SARS-CoV-2 and these symptoms can be relieved by treatment with the same OTC medicines as available for treatment of colds …

Hlh-Like Toxicities Predict Poor Survival After The Use Of Tisagenlecleucel In Children And Young Adults With B-All., Kevin O. Mcnerney, Stephanie J. Si Lim, Kyle Ishikawa, Alexandra Dreyzin, Anant Vatsayan, John J. Chen, Christina Baggott, Snehit Prabhu, Holly L. Pacenta, Christine Philips, Jenna Rossoff, Heather E. Stefanski, Julie-An Talano, Amy Moskop, Michael Verneris, Douglas Myers, Nicole A. Karras, Patrick Brown, Challice L. Bonifant, Muna Qayed, Michelle Hermiston, Prakash Satwani, Christa Krupski, Amy K. Keating, Susanne H C Baumeister, Vanessa A. Fabrizio, Vasant Chinnabhandar, Emily Egeler, Sharon Mavroukakis, Kevin J. Curran, Crystal L. Mackall, Theodore W. Laetsch, Liora M. Schultz

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Chimeric antigen receptor-associated hemophagocytic lymphohistiocytosis (HLH)-like toxicities (LTs) involving hyperferritinemia, multiorgan dysfunction, coagulopathy, and/or hemophagocytosis are described as occurring in a subset of patients with cytokine release syndrome (CRS). Case series report poor outcomes for those with B-cell acute lymphoblastic leukemia (B-ALL) who develop HLH-LTs, although larger outcomes analyses of children and young adults (CAYAs) with B-ALL who develop these toxicities after the administration of commercially available tisagenlecleucel are not described. Using a multi-institutional database of 185 CAYAs with B-ALL, we conducted a retrospective cohort study including groups that developed HLH-LTs, high-grade (HG) CRS without HLH-LTs, or no to low-grade …

Detrimental Effects Of Pcsk9 Loss-Of-Function In The Pediatric Host Response To Sepsis Are Mediated Through Independent Influence On Angiopoietin-1., Mihir R. Atreya, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Scott L. Weiss, Michael T. Bigham, Parag N. Jain, Adam J. Schwarz, Riad Lutfi, Jeffrey Nowak, Geoffrey L. Allen, Neal J. Thomas, Jocelyn R. Grunwell, Torrey Baines, Michael Quasney, Bereketeab Haileselassie, Matthew N. Alder, Patrick Lahni, Scarlett Ripberger, Adesuwa Ekunwe, Kyle R. Campbell, Keith R. Walley, Stephen W. Standage

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BACKGROUND: Sepsis is associated with significant mortality. Yet, there are no efficacious therapies beyond antibiotics. PCSK9 loss-of-function (LOF) and inhibition, through enhanced low-density lipoprotein receptor (LDLR) mediated endotoxin clearance, holds promise as a potential therapeutic approach among adults. In contrast, we have previously demonstrated higher mortality in the juvenile host. Given the potential pleiotropic effects of PCSK9 on the endothelium, beyond canonical effects on serum lipoproteins, both of which may influence sepsis outcomes, we sought to test the influence of PCSK9 LOF genotype on endothelial dysfunction.

METHODS: Secondary analyses of a prospective observational cohort of pediatric septic shock. Genetic variants …

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

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Hypoglycemia is concerning for neurological complications in infants and children. Determining the cause of hypoglycemia is essential in providing appropriate treatment. Hyperinsulinism and growth hormone deficiency are known causes of hypoglycemia but are not commonly found together. We report a 4-month-old boy who presented with severe hypoglycemia and was found to have both hyperinsulinism and growth hormone deficiency. Treatment with both recombinant human growth hormone and diazoxide led to blood glucose normalization. Subsequently, he was found to have a genetic diagnosis of 20p11.22p11.21 deletion. 20p11 deletions have been associated with hypopituitarism, most commonly seen in growth hormone deficiency causing hypoglycemia. …

Perinatal Palliative Care In Sub-Saharan Africa: Recommendations For Practice, Future Research, And Guideline Development., Mahlet Abayneh, Sharla Rent, Peter Odion Ubuane, Brian S. Carter, Solomie Jebessa Deribessa, Betelehem B. Kassa, Atnafu Mekonnen Tekleab, Stephanie K. Kukora

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Worldwide, sub-Saharan Africa has the highest burden of global neonatal mortality (43%) and neonatal mortality rate (NMR): 27 deaths per 1,000 live births. The WHO recognizes palliative care (PC) as an integral, yet underutilized, component of perinatal care for pregnancies at risk of stillbirth or early neonatal death, and for neonates with severe prematurity, birth trauma or congenital anomalies. Despite bearing a disproportionate burden of neonatal mortality, many strategies to care for dying newborns and support their families employed in high-income countries (HICs) are not available in low-and-middle-income countries (LMICs). Many institutions and professional societies in LMICs lack guidelines or …

Persistent Increase In Serum Ferritin Levels Despite Converting To Permanent Vascular Access In Pediatric Hemodialysis Patients: Pediatric Nephrology Research Consortium Study., Ali Mirza Onder, Md Abu Yusuf Ansari, Fang Deng, Matthew M. Grinsell, Larry Patterson, Jennifer Jetton, Sahar Fathallah-Shaykh, Daniel Ranch, Diego Aviles, Lawrence Copelovitch, Eileen Ellis, Vimal Chadha, Ayah Elmaghrabi, Jen-Jar Lin, Lavjay Butani, Maha Haddad, Olivera Marsenic, Paul Brakeman, Raymond Quigley, H Stella Shin, Rouba Garro, Rupesh Raina, Craig B. Langman

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Our objective was to examine serum ferritin trends after conversion to permanent vascular access (PVA) among children who started hemodialysis (HD) using tunneled cuffed catheters (TCC). Retrospective chart reviews were completed on 98 subjects from 20 pediatric HD centers. Serum ferritin levels were collected at the creation of PVA and for two years thereafter. There were 11 (11%) arteriovenous grafts (AVG) and 87 (89%) arteriovenous fistulae (AVF). Their mean TCC use was 10.4 ± 17.3 months. Serum ferritin at PVA creation was elevated at 562.64 ± 492.34 ng/mL, increased to 753.84 ± 561.54 ng/mL (p = < 0.001) in the first year and remained at 759.60 ± 528.11 ng/mL in the second year (p = 0.004). …

State Of The Art Bowel Management For Pediatric Colorectal Problems: Functional Constipation., Elizaveta Bokova, Wendy Jo Svetanoff, John Rosen, Marc A. Levitt, Rebecca M. Rentea

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BACKGROUND: Functional constipation (FC) affects up to 32% of the pediatric population, and some of these patients are referred to pediatric surgery units to manage their constipation and/or fecal incontinence. The aim of the current paper is to report the recent updates on the evaluation and management of children with FC as a part of a manuscript series on bowel management in patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and FC.

METHODS: A literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published within the last 5-10 years.

RESULTS: The first …

Strengthening Tobacco 21 Implementation And Enforcement To Reduce Tobacco-Related Health Disparities: A Stakeholder Engagement Project., Summer Woolsey, Athena K. Ramos, Kaeli Samson, Delwyn Catley, Keyonna M. King, Rob Crane, Hongying Daisy Dai

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INTRODUCTION: As a part of a priority-setting stakeholder engagement project to strengthen the impact of the federal Tobacco 21 (T21) law, we conducted a qualitative study to solicit input from a nationwide sample of tobacco control stakeholders regarding the implementation, enforcement, and equity implications of the T21 law.

METHODS: Following the T21 policy evaluation guidance developed by the Centers for Disease Control (CDC), we identified T21 experts in four domains: policy, evaluation, subject matter, and implementation from a national search of stakeholders (invitations, n=1279) to account for geographical variation. This study presents results from five focus groups conducted in December …

Pharmacogenetic Testing For The Pediatric Gastroenterologist: Actionable Drug-Gene Pairs To Know., Tracy L. Sandritter, Rachel Chevalier, Rebecca Abt, Valentina Shakhnovich

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Gastroenterologists represent some of the earlier adopters of precision medicine through pharmacogenetic testing by embracing upfront genotyping for thiopurine S-methyltransferase nucleotide diphosphatase (TPMT) before prescribing 6-mercaptopurine or azathioprine for the treatment of inflammatory bowel disease. Over the last two decades, pharmacogenetic testing has become more readily available for other genes relevant to drug dose individualization. Common medications prescribed by gastroenterologists for conditions other than inflammatory bowel disease now have actionable guidelines, which can improve medication efficacy and safety; however, a clear understanding of how to interpret the results remains a challenge for many clinicians, precluding wide implementation of …

Evolutionary And Functional History Of The Escherichia Coli K1 Capsule., Sergio Arredondo-Alonso, George Blundell-Hunter, Zuyi Fu, Rebecca A. Gladstone, Alfred Fillol-Salom, Jessica Loraine, Elaine Cloutman-Green, Pål J. Johnsen, Ørjan Samuelsen, Anna K. Pöntinen, François Cléon, Susana Chavez-Bueno, Miguel A. De La Cruz, Miguel A. Ares, Manivanh Vongsouvath, Agnieszka Chmielarczyk, Carolyne Horner, Nigel Klein, Alan Mcnally, Joice N. Reis, José R. Penadés, Nicholas R. Thomson, Jukka Corander, Peter W. Taylor, Alex J. Mccarthy

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Escherichia coli is a leading cause of invasive bacterial infections in humans. Capsule polysaccharide has an important role in bacterial pathogenesis, and the K1 capsule has been firmly established as one of the most potent capsule types in E. coli through its association with severe infections. However, little is known about its distribution, evolution and functions across the E. coli phylogeny, which is fundamental to elucidating its role in the expansion of successful lineages. Using systematic surveys of invasive E. coli isolates, we show that the K1-cps locus is present in a quarter of bloodstream infection isolates and has emerged …

Congenital Malformations And Preeclampsia Associated With Integrase Inhibitor Use In Pregnancy: A Single-Center Analysis., Christiana Smith, Angela J. Fought, Joyce F. Sung, Jennifer R. Mckinney, Torri D. Metz, Kirk B. Fetters, Sarah Lazarus, Shannon Capraro, Emily Barr, Carrie Glenny, Jenna Buehler, Adriana Weinberg, Chip Perinatal Medical Team

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Antiretroviral therapy (ART) decreases perinatal HIV transmission, but concerns exist regarding maternal and infant safety. We compared the incidence of congenital malformations and other adverse outcomes in pregnancies exposed to integrase inhibitor (INSTI) versus non-INSTI ART.

SETTING: Single-site review of all pregnancies among women living with HIV between 2008 and 2018.

METHODS: We used binomial family generalized estimating equations to model the relationship of congenital anomalies and pregnancy outcomes with exposure to INSTI or dolutegravir (DTG) versus non-INSTI ART.

RESULTS: Among 257 pregnancies, 77 women received ≥1 INSTI (54 DTG, 14 elvitegravir, 15 raltegravir), 167 received non-INSTI, and 3 …

Diabetes-Related Quality Of Life Assessment In Children Following Total Pancreatectomy With Islet Autotransplantation., Jacob M. Redel, Lindsey Hornung, Deborah Elder, Jaimie D. Nathan, Sarah Corathers, Kristin L. Rich, Maisam Abu-El-Haija

Manuscripts, Articles, Book Chapters and Other Papers

Total pancreatectomy with islet autotransplantation (TPIAT) can improve pain and reduce functional impairment associated with acute recurrent or chronic pancreatitis. However, long-term glucose monitoring and insulin therapy are often required, which can adversely affect the quality of life. We sought to evaluate diabetes-related quality of life (DR-QOL) in youth who underwent TPIAT and compare it to the youth with new-onset type 1 diabetes (T1D). The Pediatric Quality of Life Inventory™ 3.2 Diabetes Module (PedsQL™ DM) was used to assess DR-QOL in 46 youth (< 20 years old) who underwent TPIAT. The PedsQL™ DM scores were analyzed for statistically significant changes and minimally important clinical differences (MCID) over time post-TPIAT. Scores at 12 months (n = 52) and 24 months (n = 58) after diagnosis of T1D. The diabetes …