Pediatrics Commons^™

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

Manuscripts, Articles, Book Chapters and Other Papers

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 …

2023-2024 Pediatric Subspecialty Trainees, Children's Mercy Kansas City

Residents and Fellows Portraits

Photo composites of the graduate pediatric trainees in adolescent medicine, allergy/immunology, child abuse pediatrics, child neurology, clinical neuro-psychology, coagulation, clinical child psychology, clinical child psychology internship, epilepsy, hospice & palliative care, pediatric anesthesiology, pediatric bioethics, neonatal-perinatal medicine, pediatric adolescent gynecology, pediatric cardiology, pediatric EM/child abuse, pediatric critical care medicine, pediatric dentistry, pediatric emergency medicine, pediatric endocrinology, pediatric gastroenterology, pediatric hematology/oncology, pediatric hospital medicine, pediatric infectious diseases, pediatric nephrology, pediatric ophthalmology; pediatric optometry, pediatric orthopedic surgery, pediatric otolaryngology, pediatric pulmonology, pediatric rehabilitation, pediatric sports medicine, pediatric surgery, sleep medicine, pharmacy, pediatric residency and med/peds residency.

Implementation Of A Patient Care Specialist Role To Enhance The Patient Experience In A Pediatric Emergency Department, Jillian Brodeur

Doctor of Nursing Practice Projects

Background: Patient-centered care (PCC) has been an emerging trend in healthcare for the past two decades (Walsh et al., 2022). PCC and the patient experience have become essential measures of the quality of care provided and are linked to reimbursement dollars and improved patient outcomes (Rapport et al., 2019).

Purpose: The purpose of the Doctor of Nursing Practice (DNP) project was to improve the patient experience in the Emergency Department (ED) of a metropolitan hospital. The DNP project focused on the benefit of a patient advocate in the ED to improve the patient experience. The project sought to improve …

Developing And Testing A Clinical Care Bundle Incorporating Caffeine Citrate To Manage Apnoea Of Prematurity In A Resource-Constrained Setting: A Mixed Methods Clinical Feasibility Study Protocol, Grace Irimu, Ferdinand Okwaro, Jesse Coleman, Mary Waiyego, Dorothy Chomba, Millicent Parsimei, Cynthia Shitote, Roseline Ochieng, Jasmit Shah, William Macharia

Paediatrics and Child Health, East Africa

Background Apnoea of prematurity (AOP) is a common condition among preterm infants. Methylxanthines, such as cafeine and aminophylline/theophylline, can help prevent and treat AOP. Due to its physiological benefts and fewer side efects, cafeine citrate is recommended for the prevention and treatment of AOP. However, cafeine citrate is not available in most resource-constrained settings (RCS) due to its high cost. Challenges in RCS using caffeine citrate to prevent AOP include identifying eligible preterm infants where gestational age is not always known and the capability for continuous monitoring of vital signs to readily identify apnoea. We aim to develop an evidencebased …

Conjunctivitis, Neonatal, Children's Mercy Kansas City

Care Process Models

No abstract provided.

Heavy Menstrual Bleeding, Children's Mercy Kansas City

Care Process Models

No abstract provided.

Eating Disorders - Medical Work-Up And Stabilization, Children's Mercy Kansas City

Care Process Models

No abstract provided.

Transpyloric Feed In Reflux-Associated Apnea In Preterm Newborns: A Prospective Study, Tamoghna Biswas, Tapas K. Sabui, Somosri Roy, Rakesh Mondal, Shubhabrata Majumdar, Sudipta Misra

HCA Healthcare Journal of Medicine

Background

The etiological correlation between gastroesophageal reflux (GER) and apnea is controversial. We conducted a prospective interventional study designed to address the controversy.

Methods

Preterm neonates with apnea at a tertiary care center, who had clinical features of GER without any other comorbidities likely to cause apnea, were included in the study. The enrolled neonates underwent continuous transpyloric tube feeding for 72 hours. The primary outcome measure was the difference in the number of apneic episodes pre- and post-initiation of nasoduodenal (ND) feeding. Secondary outcome measures included the incidence of necrotizing enterocolitis, other gastrointestinal disturbances, and mortality.

Results

Sixteen preterm …

Constant Site Hemodialysis In The Pediatric Population: Successful Use Of Vwings In Three Patients, Jordy Salcedo-Giraldo, Maxwell Kilcoyne, Chi Chi Do-Nguyen, Achintya Moulick, Susan Conley, Paul Brady, Vicki Mahan, Randy Stevens

Transformative Medicine (T-Med)

Introduction: Reliable vascular access for hemodialysis is challenging in the pediatric population. The Venous Window Needle Guide (VWING; Vital Access Corp, Salt Lake City, Utah) is an implanted surgical device that allows for constant site access but has not been reported in pediatric patients.

Case description: Three patients under 18 years of had a brachiobasilic fistula created with a minimum of 6 weeks for fistula maturation prior to VWING placement. This paper describes the management of constant site dialysis using VWING as well as strategies used for successful long-term management. Patients include a 15-year-old male with end-stage renal disease (ESDR) …

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

Manuscripts, Articles, Book Chapters and Other Papers

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

Manuscripts, Articles, Book Chapters and Other Papers

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 …

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

Manuscripts, Articles, Book Chapters and Other Papers

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 …

Evaluation Of The Orally Bioavailable 4-Phenylbutyrate-Tethered Trichostatin A Analogue Ar42 In Models Of Spinal Muscular Atrophy, Casey J. Lumpkin, Ashlee W. Harris, Andrew J. Connell, Ryan W. Kirk, Joshua A. Whiting, Luciano Saieva, Livio Pellizzoni, Arthur H.M. Burghes, Matthew E.R. Butchbach

Department of Pediatrics Faculty Papers

Proximal spinal muscular atrophy (SMA) is a leading genetic cause for infant death in the world and results from the selective loss of motor neurons in the spinal cord. SMA is a consequence of low levels of SMN protein and small molecules that can increase SMN expression are of considerable interest as potential therapeutics. Previous studies have shown that both 4-phenylbutyrate (4PBA) and trichostatin A (TSA) increase SMN expression in dermal fibroblasts derived from SMA patients. AR42 is a 4PBA-tethered TSA derivative that is a very potent histone deacetylase inhibitor. SMA patient fibroblasts were treated with either AR42, AR19 (a …

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

Manuscripts, Articles, Book Chapters and Other Papers

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). …

Barriers And Facilitators To Achieving Well-Being In Pediatric Providers, Whitney Marvin, Jillian Harvey, Natasha Ruth

Journal of Wellness

Introduction: Physician well-being has become increasingly important for health systems across the country due to the strong ties between quality, safety, and overall patient outcomes. Burnout has increased steadily and has been exacerbated by the COVID-19 pandemic. This study seeks to successfully identify factors that both enhance and detract from well-being in a cohort of pediatric faculty and advanced practice providers (APPs).

Methods: This study utilized a multimethod approach including surveys and a total of 8 semi-structured, virtual focus groups of pediatricians and advanced practice providers (orchestrated through Microsoft Teams) to understand perspectives on burnout and well-being. Each …

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

Manuscripts, Articles, Book Chapters and Other Papers

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 …

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

Manuscripts, Articles, Book Chapters and Other Papers

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 …

Improving Communication Access With Deaf People Through Nursing Simulation: A Cross-Disciplinary Collaboration, Jamie L. Mccartney Ph.D., Tracy Gidden, Jennifer Biggs, Kathy Geething, Karl Kosko Ph.D.

Journal of Gender, Ethnic, and Cross-Cultural Studies

Baccalaureate nursing and sign language interpreting students participated in a pediatric discharge simulation with a deaf person playing the role of the baby’s parent. At the conclusion of the simulation, participants were emailed a consent letter and a link to a 17-item questionnaire developed by the authors. Responses were analyzed both quantitatively and qualitatively, whereby nonparametric statistics were calculated to examine Likert-scale items. A Mann-Whitney test statistic was calculated, instead of an independent samples t-test, given the smaller sample in the current study (n = 26). A question was posed to participants that evaluated their self-perception of the effectiveness of …

A Rare Case Of Combined Immunodeficiency Syndrome With Otomastoiditis, Parotitis & Facial Nerve Palsy, Parth Garg, Amulya T M, Prakash B.G., Sandhya D

Digital Journal of Clinical Medicine

This article presents a case study of a 4-month-old female baby with combined immunodeficiency syndrome complicated by bilateral otomastoiditis, right-sided parotitis, and facial nerve palsy. The patient presented with symptoms of fever, cold, earache, and ear discharge. Clinical examination revealed redness and swelling in the post-auricular region, along with bloody discharge from the right ear. Further investigations, including blood tests, culture sensitivity, ultrasound scans, and imaging studies, confirmed the diagnosis. The patient was treated with appropriate antibiotics, antifungals, and supportive care. The article discusses the challenges in diagnosing and managing primary immunodeficiency diseases (PIDs), emphasising the importance of early recognition …

Bone Growth Induction In Mucopolysaccharidosis Iva Mouse, Estera Rintz, Angélica María Herreño-Pachón, Betul Celik, Fnu Nidhi, Shaukat Khan, Eliana Benincore-Flórez, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is caused by a deficiency of the N-acetylgalactosamine-6-sulfate-sulfatase (GALNS) enzyme, leading to the accumulation of glycosaminoglycans (GAG), keratan sulfate (KS) and chondroitin-6-sulfate (C6S), mainly in cartilage and bone. This lysosomal storage disorder (LSD) is characterized by severe systemic skeletal dysplasia. To this date, none of the treatment options for the MPS IVA patients correct bone pathology. Enzyme replacement therapy with elosulfase alpha provides a limited impact on bone growth and skeletal lesions in MPS IVA patients. To improve bone pathology, we propose a novel gene therapy with a small peptide as a growth-promoting …