Medicine and Health Sciences Commons^™

Viral Whole Genome Sequencing For Antiviral Resistance In A Child With Dock8 Deficiency And Recurrent Hsv-1, Sean Stout, A. L. Greninger, Rangaraj Selvarangan, A. F. Freeman, Brandon D. Newell, Erin Stahl, Dwight Yin

Posters

We present the case of a child with dedicator of cytokinesis 8 (DOCK8) deficiency and chronic, resistant HSV-1 mucocutaneous infections to illustrate the clinical utility of viral whole genome sequencing to detect active and latent HSV resistance mutations.

Congenital Abnormalities Of The Lower Airways And Lungs, Christopher M. Oermann

Presentations

Participants will be able to: Describe the 5 stages of fetal lung development. Summarize the prenatal identification and management of congenital abnormalities of the lower airways and lung (CALA). Explain the classification of CALAL. Compare the clinical characteristics, pathology, and management of common CALAL. Discuss the long term prognosis of CALAL.

A Review Of First Year Fellow Endoscopic Skills And Time To Proficiency, Antoinette Adjowa O. Walker, Dalya El Tawil, Jennifer Colombo, Nicholas Clark, Kimberly J. Reid

Posters

Through this quality improvement project, we have assessed the current fellows' upper endoscopy skill level during their first year and determined that the time to achieving proficiency is significantly longer than desired. The goal is to prepare new fellows for endoscopy prior to the beginning of their rotations with a curriculum that includes assigned reading materials, videos, and simulated endoscopic cases, to promote safety and increase satisfaction of patients by improving the readiness, skill level, and the confidence of first year inexperienced fellows.

Who Codes In The Nicu: An Analysis Of Demographics And Factors That Place Neonates At Higher/Lower Risk Of A Serious Code Event And Prognosis Post-Code, Danielle N. Gonzales, Ashley K. Sherman, Jennifer Dremann, Staci Elliott, Amelia Gute, Amber Bellinghausen, Jessica Brunkhorst, Danielle Reed

Posters

This study analyzed resuscitation events in a level IV neonatal intensive care unit from 2012-2017 to determine whether there are identifiable differences between those who have a rapid response event and those with a short or long code and determine factors post-event that may impact survival to discharge.

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.

Evaluation Of The Project P.A.T.H.S. In Mainland China: Findings Based On Student Diaries, Daniel T. L. Shek, Xiaoqin Zhu, Janet T. Y. Leung, Tak Yan Lee, Florence K. Y. Wu

Pediatrics Faculty Publications

Objectives: Based on 859 student diaries, the present study evaluated a positive youth development program entitled “Tin Ka Ping (TKP) Positive Adolescent Training through Holistic Social programs (P.A.T.H.S.) ” project implemented in mainland China during the 2015–2016 academic year. Method: To understand the perceived effectiveness of the students, the study analyzed quantitative as well as qualitative data derived from the student diaries. Results: The students held very positive views toward the program and the implementers. The narratives of the students also showed that they perceived improvement in the intrapersonal, interpersonal, familial, and societal domains after joining the program. Conclusion: In …

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.

Delayed Initiation Of Therapeutic Hypothermia For Outborn Infants Is Associated With Adverse Outcomes, Nabeel Hashmi, Max Sale, Leah Fox, Jay Kerecman, Lauren Mcallister, Misty Melendi, Frances Lucas

MaineHealth Maine Medical Center

Delayed Initiantion of Therapeutic Hypothermia for Outborn Infants is Associated with Adverse Outcomes

Feasibility Of Pre-Operative Mtor Inhibitor Sirolimus In Children And Young Adults With Desmoid Tumor, Stephanie Verwys, Clara Magyar, Kathleen Glick, Douglas Hawkins, Archana Sharma, Brenda Weigel, Katherine Chastain, Joseph Khoury, Michele Manalang, Sarah Dry, Noah Federman, Aaron Weiss

MaineHealth Maine Medical Center

Background:

• Desmoid tumor represents an intermediate grade neoplasm with a striking predilection for locally invasive growth and recurrence following resection

• More effective, well-tolerated non-surgical treatment options are needed

• Current approaches

• If feasible, watchful waiting is the preferred approach

• 20-30% spontaneous regression

• In situations where treatment is indicated, the following approaches are utilized

• Surgery is the primary approach if minimal morbidity is anticipated

• Medical therapies

• Cytotoxic drugs

• Tyrosine kinase inhibitors

• Hydroxyurea

• Gamma secretase inhibitors

• mTOR Inhibitor Rationale

• Desmoid tumor is well-known to be associated with deregulation of …

Outcomes Of Implementation Of An Evidence-Based Enteral Feeding Protocol In Neonates Weighing Less Than 1800g At Birth, Rebecca Edwards

MaineHealth Maine Medical Center

Background:

Several studies have demonstrated that the implementation of standardized enteral feeding guidelines can lead to positive outcomes in the neonatal intensive care unit (NICU), including improved nutrition, decreased need for parenteral nutrition (PN), central lines and decreased costs. Furthermore, implementation of standard feeding regimens have significantly reduced the incidence of necrotizing enterocolitis (NEC), a devastating gastrointestinal emergency in neonates. It has been reported that NEC has an iatrogenic component related to variations in feeding practices. While the exact mechanism of the reduced risk of NEC and implementation of feeding protocols is unclear, standard feeding regimens likely improve consistency in …

Quality Initiative To Reduce Cardiac Ct Angiography Radiation Exposure In Patients With Congenital Heart Disease, Fatima Ali, Arjumand Rizvi, Huzaifa Ahmad, Phillip Mcgonagill, Muneeb Khan, Rajesh Krishnamurthy, Zafar Jamil, Naila Nadeem, Mohammad Yousuf, Babar Hasan

Department of Paediatrics and Child Health

Background: The use of cardiac computed tomography angiography (CCTA) as a complementary diagnostic modality to echocardiography in patients with congenital heart diseases (CHDs) is expanding in low- and middle-income countries. The adoption of As Low As Reasonably Achievable techniques is not widespread, resulting in significant unintended radiation exposure, especially in children. Simple quality improvement measures geared toward reducing radiation dose can have a impact on patient safety in resource-limited centers in low- and middle-income countries.
Objectives: To determine how a quality improvement initiative can reduce radiation exposure during CCTA in patients with CHD.
Methods: We designed a key driver -based …

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.

Associations Of First Trimester Co-Use Of Tobacco And Cannabis With Prenatal Immune Response And Psychosocial Well-Being, Kristin Ashford, Amanda Fallin-Bennett, Andrea Mccubbin, Amanda T. Wiggins, Sheila Barnhart, Joshua A. Lile

Perinatal Research and Wellness Center Faculty Publications

PURPOSE: This study aims to describe the association of first trimester co-use of tobacco and cannabis with maternal immune response and psychosocial well-being, relative to tobacco use only.

METHODS: A preliminary midpoint analysis included 138 pregnant women with biologically verified tobacco use, 38 of whom (28%) also tested positive for recent cannabis use. Maternal perceived stress (Perceived Stress Scale), depressive symptoms (Edinburgh Postnatal Depression Scale), and serum immune markers (IL-1β, IL-2, IL-6, IL-8, IL-10, TNFα, CRP, MMP8), were collected, although cytokine data were only available for 122 women.

RESULTS: Participant average age was 29.1 years, approximately half had a high …

Per- And Polyfluoroalkyl Substances And Bone Mineral Density In Mid-Childhood, Shravanthi M. Seshasayee, Rachel Cluett, Lisa B. Rokoff, Sheryl L. Rifas-Shiman, Diane R. Gold, Brent Coull, Catherine M. Gordon, Clifford J. Rosen, Emily Oken, Sharon K. Sagiv, Abby F. Fleisch

MaineHealth Maine Medical Center

Background:

• Identifying factors that impair bone accrual during childhood is a critical step toward osteoporosis prevention.

• One potential risk factor not well characterized in childhood is the role of chemicals in the environment.

• Perfluoroalkyl substances (PFASs) are synthetic additives used to make clothing, furniture, and cookware stain repellant and are detectable in almost all US adults.

• PFASs act as PPAR-γ agonists,2 androgen receptor antagonists, and directly intercalate into bone, raising the possibility that they may lead to low bone accrual.

• While two population-based studies in adults have shown associations between PFASs and low areal bone …

The Incidence, Aetiology, And Adverse Clinical Consequences Of Less Severe Diarrhoeal Episodes Among Infants And Children Residing In Low-Income And Middle-Income Countries: A 12-Month Case-Control Study As A Follow-On To The Global Enteric Multicenter Study (Gems), Karen L. Kotloff, Dilruba Nasrin, William C. Blackwelder, Yukun Wu, Tamer Farag, Sandra Panchalingham, Samba O. Sow, Anita K. M. Zaidi, Shahida Qureshi, Farheen Quadri

Department of Paediatrics and Child Health

Background: Diarrheal diseases remain a leading cause of illness and death among children younger than 5 years in low-income and middle-income countries. The Global Enteric Multicenter Study (GEMS) has described the incidence, aetiology, and sequelae of medically attended moderate-to-severe diarrhoea (MSD) among children aged 0-59 months residing in censused populations in sub-Saharan Africa and south Asia, where most child deaths occur. To further characterise this disease burden and guide interventions, we extended this study to include children with episodes of less-severe diarrhoea (LSD) seeking care at health centres serving six GEMS sites.
Methods: We report a 1-year, multisite, age-stratified, matched …

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.

Gene Domain-Specific Dna Methylation Episignatures Highlight Distinct Molecular Entities Of Adnp Syndrome., Eric G Bend, Erfan Aref-Eshghi, David B Everman, R Curtis Rogers, Sara S Cathey, Eloise J Prijoles, Michael J Lyons, Heather Davis, Katie Clarkson, Karen W Gripp, Dong Li, Elizabeth Bhoj, Elaine Zackai, Paul Mark, Hakon Hakonarson, Laurie A Demmer, Michael A Levy, Jennifer Kerkhof, Alan Stuart, David Rodenhiser, Michael J Friez, Roger E Stevenson, Charles E Schwartz, Bekim Sadikovic

Paediatrics Publications

BACKGROUND:
ADNP syndrome is a rare Mendelian disorder characterized by global developmental delay, intellectual disability, and autism. It is caused by truncating mutations in ADNP, which is involved in chromatin regulation. We hypothesized that the disruption of chromatin regulation might result in specific DNA methylation patterns that could be used in the molecular diagnosis of ADNP syndrome.

RESULTS:
We identified two distinct and partially opposing genomic DNA methylation episignatures in the peripheral blood samples from 22 patients with ADNP syndrome. The "epi-ADNP-1" episignature included ~ 6000 mostly hypomethylated CpGs, and the "epi-ADNP-2" episignature included ~ 1000 predominantly hypermethylated CpGs. The …

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).