Medical Specialties Commons^™

Dehydrated Hereditary Stomatocytosis Causing Sever Ascites Leading To Pulmonary Hypoplasia And Respiratory Insufficiency In A Neonate, Michael D. Mcdowell Jr, Christopher M. Oermann

Posters

Care Presentation: EM is a 32.5-week EGA infant who was born with sever congenital ascites (following repeated prenatal paracentesis) identified on prenatal ultrasound. Initial chest-abdomen imaging is shown in Figure 1. He required CPAP at delivery but was weaned of high flow nasal cannula with intermittent episodes of prolonged intubation during repeat paracentesis. Paracentesis demonstrated simple ascites rather than chylous fluid. Lymphangiograms demonstrated transient lymphatic leakage with resolution. Genetic evaluation revealed a pathogenic mutation in the PIEZO 1 gene for autosomal dominant DHS. This disorder had reduced penetrance and variable expressivity and symptoms range for absence of clinical features to …

Gonadotropin Releasing Hormone (Gnrh) Agonist Improves Hyperandrogenism In An Adolescent Female With A Novel Insulin Receptor Gene Mutation, Emily Paprocki, Romina Barral, Heidi Vanden Brink, Maria E. Lujan, Tania S. Burgert

Posters

We describe a case of an adolescent female with Type A insulin resistance whose severe hirsutism and hyperandrogenism were improved with leuprolide, despite extreme hyperinsulinemia. Ovarian volume decreased in association with the resolution of hyperandrogenism, consistent with ovarian size reflecting hyperandrogenism in adolescents.

This case allowed us to examine the frequently debated bidirectional relationship between insulin and hyperandrogenism, often deemed independent of gonadotropin activity.

Fetal Ventricular Measurement In Determination For Intrauterine Closure Of Myelomeningoceles, Paige Lundy, Emanuel Vlastos, Paul A. Grabb

Posters

Prenatal closure of myelomeningoceles for fetuses with ventricular width of greater than 15 mm at the time of intrauterine screening (19-25 weeks) has been discouraged, but little is documented regarding the details of ventricle measurement, modality, and timing. This study concludes that ultrasound and MRI provide different results in regards to fetal ventricle size. If treatment recommendations are going to be offered or withheld based on the 15 mm "rule" the method of fetal imaging must be taken into account.

Pediatric Ppi Use And Fractures, Nathan R. Fleishman, Troy Richardson, Thomas M. Attard

Posters

This study compared the rate of fracture among pediatric patients exposed to proton pump inhibitors to the rate among patients without exposure, and also considered the fracture location among children exposed to proton pump inhibitors.

Quality Improvement: Implementing A Foot Exam To Improve Care For Patients With Diabetes Mellitus, Emily Paprocki, Ryan Mcdonough, Tiffany Musick, Joseph Cernich

Posters

The American Diabetes Association guidelines recommend an annual foot exam in patients with type 1 and 2 diabetes who are ten years old or older and who have been diagnosed with diabetes at least five years. Quality improvement tools were used to improve the care provided to our patients by implementing an annual food exam.

Outcomes Following Dynamic Compression Bracing For Pectus Carinatum, Charlene Dekonenko, Robert M. Dorman, Amy L. Pierce, Beth A. Orrick, David Juang, Pablo Aguayo, Jason D. Fraser, Tolulope A. Oyetunji, Charles L. Snyder, Shawn D. St Peter, G W. Holcomb Iii

Posters

We describe our experience with dynamic compression bracing for pectus carinatum patients and their satisfaction with bracing.

Tisagenlecleucel Model-Based Cellular Kinetic Analysis Of Chimeric Antigen Receptor-T Cells., Andrew M. Stein, Stephan A. Grupp, John E. Levine, Theodore W. Laetsch, Michael A. Pulsipher, Michael W. Boyer, Keith August, Bruce L. Levine, Lori Tomassian, Sweta Shah, Mimi Leung, Pai-Hsi Huang, Rakesh Awasthi, Karen Thudium Mueller, Patricia A. Wood, Carl H. June

Manuscripts, Articles, Book Chapters and Other Papers

Tisagenlecleucel is a chimeric antigen receptor-T cell therapy that facilitates the killing of CD19+ B cells. A model was developed for the kinetics of tisagenlecleucel and the impact of therapies for treating cytokine release syndrome (tocilizumab and corticosteroids) on expansion. Data from two phase II studies in pediatric and young adult relapsed/refractory B cell acute lymphoblastic leukemia were pooled to evaluate this model and evaluate extrinsic and intrinsic factors that may impact the extent of tisagenlecleucel expansion. The doubling time, initial decline half-life, and terminal half-life for tisagenlecleucel were 0.78, 4.3, and 220 days, respectively. No impact of tocilizumab or …

Decreasing Unplanned Extubations By Taping Technique & Creating A Culture Of Safety, Karishma Rao, Beckie Palmer, Christopher R. Nitkin, Christian Anthony Schneider, Brandy Huitt, Molly Terhune, Ashley Orwick, Dianne Wilderson, Sarah Carboneau, Jenny Mckee, Kerrie A. Meinert, Eugenia K. Pallotto

Presentations

Describes strategies used to decrease the unplanned extubation rate in the Level IV neonatal intensive care unit to less than 1.0 event per 100 ventilator days by December 2018.

Neuroblastoma In Adolescents And Children Older Than 10 Years: Unusual Clinicopathologic And Biologic Features, Laura Mccarthy, Katherine Chastain, Terrie Flatt, Eugenio Taboada, Robert E. Garola, John Herriges, Linda D. Cooley, Atif Ahmed

Posters

This poster describes four cases of neuroblastoma diagnosed since 2008 in children greater than 10 years and presents their clinical, histologic and biologic features, emphasizing unusual clinicopathologic characteristics and the role of DNA microarray analysis and Next Generation Sequencing in their management.

Predictive Performance Of Existing Population Pharmacokinetic Models Of Tacrolimus In Pediatric Kidney Transplant Recipients, Alenka Chapron, Susan M. Abdel-Rahman

Posters

With the goal of developing a clinician-driven tacrolimus (TAC) pharmacokinetic dosing tool, our initial objectives were 1) to examine whether published TAC population pharmacokinetic (popPK) models could serve as a foundation for the dosing tool, and 2) evaluate their performance predicting TAC concentrations in an independent cohort of Children's Mercy patients.

A Nsqip Analysis Of Post-Operative Antibiotic Utilization In Uncomplicated Appendicitis, Charlene Dekonenko, Robert M. Dorman, Janelle R. Noel-Macdonnell, Tolulope A. Oyetunji

Posters

APSA 2010 recommendations do not support antibiotic use post-operatively following appendectomy for uncomplicated appendicitis, but compliance varies by institution. This study determined the rate of post-operative antibiotic use in uncomplicated appendicitis and found that 10% of patients were discharged on antibiotics, using the NSQIP files.

Self-Reported Outcomes Following Cholecystectomy For Pediatric Hyperkinetic Biliary Dyskinesia, Charlene Dekonenko, Joseph A. Sujka, Robert Michael Dorman, Tolulope A. Oyetunji, Shawn D. St Peter

Posters

The aim of this study is to determine if children with hyperkinetic biliary dyskinesia have resolution of symptoms after laparoscopic cholecystectomy at our institution.

Conclusion: Cholecystectomy for hyperkinetic biliary dyskinesia may or may not improve symptoms. Further research should focus on pathophysiology of the disease in order to create an evidence-based definition of biliary dyskinesia in children to determine who will benefit from cholecystectomy.

Health-Related Quality Of Life In Glomerular Disease., Pietro A. Canetta, Jonathan P. Troost, Shannon Mahoney, Amy J. Kogon, Noelle Carlozzi, Sharon M. Bartosh, Yi Cai, T Keefe Davis, Hilda Fernandez, Alessia Fornoni, Rasheed A. Gbadegesin, Emily Herreshoff, John D. Mahan, Patrick H. Nachman, David T. Selewski, Christine B. Sethna, Tarak Srivastava, Katherine R. Tuttle, Chia-Shi Wang, Ronald J. Falk, Ali G. Gharavi, Brenda W. Gillespie, Larry A. Greenbaum, Lawrence B. Holzman, Matthias Kretzler, Bruce M. Robinson, William E. Smoyer, Lisa M. Guay-Woodford, Bryce Reeve, Debbie S. Gipson, Curegn Consortium

Manuscripts, Articles, Book Chapters and Other Papers

There is scant literature describing the effect of glomerular disease on health-related quality of life (HRQOL). The Cure Glomerulonephropathy study (CureGN) is an international longitudinal cohort study of children and adults with four primary glomerular diseases (minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy). HRQOL is systematically assessed using items from the Patient-Reported Outcomes Measurement Informative System (PROMIS). We assessed the relationship between HRQOL and demographic and clinical variables in 478 children and 1115 adults at the time of enrollment into CureGN. Domains measured by PROMIS items included global assessments of health, mobility, anxiety, fatigue, and sleep …

How To Conduct Clinical Trials In Children: A Tutorial., Valentina Shakhnovich, Christoph P. Hornik, Gregory L. Kearns, Jaylene Weigel, Susan M. Abdel-Rahman

Manuscripts, Articles, Book Chapters and Other Papers

Despite a growing interest in, and commitment to, implementing pediatric clinical trials, approximately one in every five trials in children fails because of inappropriate study design, suboptimal experiment planning, or inadequate participant enrollment. This tutorial, presented from the perspectives of seasoned pediatric investigators, an experienced research coordinator, and an established pediatric clinical trials network, is designed to provide practical guidance for successfully implementing pediatric clinical trials at an academic center or another comparable institution.

Improving Discharge Communication With Outpatient Providers On The Hospitalist Service, Nicholas Clark, Julia Simmons, Angela Etzenhouser, Troy Richardson, Patrick Brady, Eugenia K. Pallotto

Posters

Describes a quality improvement project to increase two-way discharge communication between pediatric hospitalists and primary care providers from 7% to >80% within 18 months.

Practice Variations In Sedated Echocardiography And Impact On Repeat Echocardiography In Non-Refractory Kawasaki Disease, Nicholas Clark, Troy Richardson, Jennifer E. Schuster, Anitha Parthiban, Henry T. Puls

Posters

This study discovered significant variation among children's hospitals in the use of sedated echocardiiography for children <30 months of age with non-refractory Kawasaki disease prior to the American Heart Association 2017 Kawasake disease guideline update. Consideration should be given to performing sedated echocardiography as it may reduce the need for repeat echos without impacting length of stay or total costs.

The Effect Of Antihypertensive Dosing On Hypertension In Children With Chronic Kidney Disease, Benjamin A. Matta, Uri S. Alon, Bradley Warady Md, Tarak Srivastava, Darcy Weidemann

Posters

Background

Hypertension (HTN) is a highly prevalent and major risk factor for poor cardiovascular and renal outcomes in chronic kidney disease (CKD). Previous research suggests that HTN is underdiagnosed and undertreated in children with CKD. To our knowledge no studies have investigated the effect of antihypertensive (antiHTN) dose on blood pressure (BP) control in this population.

Objective

To determine the effect of antiHTN dose on HTN status in children with CKD.

Methods

Study population: 255 participants studied in the Chronic Kidney Disease in Children (CKiD) study at their 3rd visit, taking at least 1 antiHTN drug.

Cumulative Drug Dose Index …

Improving Bronchiolitis Care Across Multiple Care Settings Using A Deimplementation Bundle, Amanda Nedved, Kathleen Berg, Troy Richardson, Amanda Montalbano, Matthew B. Johnson

Posters

Despite the availability of a bronchiolitis CPG; variation in care practices still existed at our institution. The aim of our quality improvement (QI) project was to increase overall adherence with our institutional CPG in patients 60 days to 24 months of age diagnosed with bronchiolitis in the urgent care centers (UCC), emergency departments (ED), and general inpatient units from a baseline of 40.9% to 60% by the end of two bronchiolitis seasons.

Total And Free Plasma Bilirubin And Clinical Outcomes In Severe Hyperbilirubinemia, Sean M. Riordan, Jean-Baptiste Lepichon, Steven Shapiro, Tina Slusher, Fatima Abdullahi, Hafsat M. Suleiman, Victor C. Pam, Mamu B. Samuel, Christopher S. Yilgwan, Christian Isichei, Idris Y. Mohammed

Posters

Acute bilirubin encephalopathy (ABE) and kernicterus spectrum disorder (KSD) have become relatively uncommon in high income countries but remain a major cause of morbidity and mortality in low- and middle-income countries. To better understand the relationship between free (Bf) and total (TB) bilirubin levels and the development of ABE and KSD we followed infants born in three large tertiary centers in northern and central Nigeria (Jos, Kano and Zaria).

Weighted Pathway Genetic Load Analysis Of Hyperbilirubinemic Infants Indicates A Potential Genetic Component For Susceptibility To Bilirubin Neurotoxicity, Sean M. Riordan, Jean-Baptiste Lepichon, Steven Shapiro, John Cowden, Monica Villagullen, Laurence Thielemans, Dina Villanueva Garcia, Jesus Aguirre-Hernandez

Posters

Severe kernicterus spectrum disorder (KSD) is described as motor and auditory deficits resulting from brain damage caused by hyperbilirubinemia. The severity of HB does not always predict the severity of injury. The lack of a strong monogenetic link to susceptibility suggests bilirubin-induced brain damage may be due to impaired bilirubin response pathways. This poster describes work to use a modified pathway genetic load (mPGL) score method to perform a targeted genetic analysis of whole exome data from patients with various degrees of neonatal HB, with an ultimate goal of developing a neonatal screen to susceptibiltiy to bilirubin neurotoxicity.

Developing Technologically Advanced Research In Low- And Middle-Income Countries, Isa Abdulkadir, Tina Slusher, Fidelia Bode-Thomas, Sean Riordan, Jean-Baptiste Lepichon, Laila Hassan, Shehu Abdullahi, Akinyemi Ofakunrin, Stephen Oguche, Steven Shapiro, William Ogala

Posters

This poster presents a collaboration between a group of scientists in Nigeria and the USA in developing a genomic research project and the solutions adopted in tackling the ethical implications and partnership equity and ownership of such a project.

Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections In Children With Cystic Fibrosis., Peter Jorth, Zarmina Ehsan Md, Amir Rezayat, Ellen Caldwell, Christopher Pope, John J. Brewington, Christopher H. Goss, Dan Benscoter, John P. Clancy, Pradeep K. Singh

Manuscripts, Articles, Book Chapters and Other Papers

Culture and sequencing have produced divergent hypotheses about cystic fibrosis (CF) lung infections. Culturing suggests that CF lungs are uninfected before colonization by a limited group of CF pathogens. Sequencing suggests diverse communities of mostly oral bacteria inhabit lungs early on and diversity decreases as disease progresses. We studied the lung microbiota of CF children using bronchoscopy and sequencing, with measures to reduce contamination. We found no evidence for oral bacterial communities in lung lavages that lacked CF pathogens. Lavage microbial diversity varied widely, but decreases in diversity appeared to be driven by increased CF pathogen abundance, which reduced the …

Ucp1 Expression-Associated Gene Signatures Of Human Epicardial Adipose Tissue., Kanta Chechi, Jinchu Vijay, Pierre Voisine, Patrick Mathieu, Yohan Bossé, Andre Tchernof, Elin Grundberg, Denis Richard

Manuscripts, Articles, Book Chapters and Other Papers

Multiple reports of uncoupling protein 1 (UCP1) expression have established its presence in human epicardial adipose tissue (eAT). Its functional relevance to eAT, however, remains largely unknown. In a recent study, we reported that adrenergic stimulation of eAT was associated with downregulation of secreted proteins involved in oxidative stress-related and immune-related pathways. Here, we explored the UCP1-associated features of human eAT using next-generation deep sequencing. Paired biopsies of eAT, mediastinal adipose tissue (mAT), and subcutaneous adipose tissue (sAT) obtained from cardiac surgery patients, with specific criteria of high and low expression of UCP1 in eAT, were subjected to RNA sequencing. …

Aztreonam Lysine Inhalation Solution In Cystic Fibrosis., Claire Elson, Joel Mermis, Deepika Polineni, Christopher M. Oermann

Manuscripts, Articles, Book Chapters and Other Papers

Patients with cystic fibrosis (CF) develop pulmonary disease secondary to airway infection and dysregulated inflammation. Therapeutic innovations such as nebulized antimicrobial therapy targeting specific pathogens have resulted in improvements in quality of life and life expectancy. Aztreonam lysine for inhalation (AZLI) solution was initially approved to improve respiratory symptoms in CF patients with Pseudomonas aeruginosa (PA) in 2010 by the Food and Drug Administration. Since then, research broadening labeling and clinical application has been developed. In this review, we analyze published and ongoing research regarding AZLI therapy in CF. A search of the Cochrane Database of Systematic Reviews and the …

Defining And Measuring Quality In Acute Paediatric Trauma Stabilisation: A Phenomenographic Study., Ralph James Mackinnon, Karin Pukk-Härenstam, Ulrica Von Thiele Schwarz, Christopher S. Kennedy, Terese Stenfors

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: Trauma is the leading cause of death in children. The lack of an accepted definition of what constitutes a high-quality stabilisation of a traumatically injured child has limited the evaluation of direct interventions in simulation-based education and service-delivery models to improve trauma care. The aim of this study was to create a framework that delineates quality by exploring the perceptions of the multi-disciplinary team providing and improving this initial care.

Methods: Interviews were conducted with 36 experienced UK trauma team members and governance administrators (clinical directors to executive board level), from three standard UK trauma units. This study used …

3 Cm Researchers Honored With Endowed Chairs, Children's Mercy Hospital

Our Story Continues

Susan Abdel-Rahman, PharmD received the Marion Merrell Dow/Missouri Chair in Pediatric Clinical Pharmacology. Shao Jiang, MD received the Frederick J. McCoy Endowed Chair in Plastic Surgery and Craniofacial Research. Cy Nadler, PhD received the Josh Barnds and Stella Carlson Endowed Professorship for Autism.

Peritoneal Dialysis Vintage And Glucose Exposure But Not Peritonitis Episodes Drive Peritoneal Membrane Transformation During The First Years Of Pd., Maria Bartosova, Betti Schaefer, Karel Vondrak, Peter Sallay, Christina Taylan, Rimante Cerkauskiene, Maria Dzierzega, Gordana Milosevski-Lomic, Rainer Büscher, Ariane Zaloszyc, Philipp Romero, Felix Lasitschka, Bradley A. Warady, Franz Schaefer, Akos Ujszaszi, Claus Peter Schmitt

Manuscripts, Articles, Book Chapters and Other Papers

The impact of peritoneal dialysis (PD) associated peritonitis on peritoneal membrane integrity is incompletely understood. Children are particularly suited to address this question, since they are largely devoid of preexisting tissue damage and life-style related alterations. Within the International Peritoneal Biobank, 85 standardized parietal peritoneal tissue samples were obtained from 82 children on neutral pH PD fluids with low glucose degradation product (GDP) content. 37 patients had a history of peritonitis and 16 of the 37 had two or more episodes. Time interval between tissue sampling and the last peritonitis episode was 9 (4, 36) weeks. Tissue specimen underwent digital …

Screening Pediatric Patients For Readmission Risk, Sarah Bradshaw, Blair Buenning

Presentations

Podium Presentation of HARRPS Research Project and Findings

Inotuzumab Ozogamicin In Pediatric Patients With Relapsed/Refractory Acute Lymphoblastic Leukemia., Deepa Bhojwani, Richard Sposto, Nirali N. Shah, Vilmarie Rodriguez, Constance Yuan, Maryalice Stetler-Stevenson, Maureen M. O'Brien, Jennifer L. Mcneer, Amrana Quereshi, Aurelie Cabannes, Paul Schlegel, Claudia Rossig, Luciano Dalla-Pozza, Keith August, Sarah Alexander, Jean-Pierre Bourquin, Michel Zwaan, Elizabeth A. Raetz, Mignon L. Loh, Susan R. Rheingold

Manuscripts, Articles, Book Chapters and Other Papers

Although inotuzumab ozogamicin (InO) is recognized as an effective agent in relapsed acute lymphoblastic leukemia (ALL) in adults, data on safety and efficacy in pediatric patients are scarce. We report the use of InO in 51 children with relapsed/refractory ALL treated in the compassionate use program. In this heavily pretreated cohort, complete remission was achieved in 67% of patients with overt marrow disease. The majority (71%) of responders were negative for minimal residual disease. Responses were observed irrespective of cytogenetic subtype or number or type of prior treatment regimens. InO was well-tolerated; grade 3 hepatic transaminitis or hyperbilirubinemia were noted …

Identification Of Inadvertent Azygous Vein Cannulation Using Transthoracic Echocardiography During Venoarterial Extracorporeal Membrane Oxygenation Initiation., Bethany Runkel, Jason D. Fraser, John Daniel, Karina M. Carlson

Manuscripts, Articles, Book Chapters and Other Papers

It is well known that optimal cannula positioning during initiation of venoarterial (VA) extracorporeal membrane oxygenation (ECMO) is directly related to ECMO circuit function. Malpositioned cannulae can have deleterious effects on clinical outcomes, and inadvertent cannulation of the azygous vein during initiation of ECMO is a rare but potentially devastating complication that has been reported only a few times in the literature. Here we report a case of azygous vein cannulation in a neonate that was not identified on chest radiography but was recognized and corrected expeditiously with the use of transthoracic echocardiography.