Medical Specialties Commons^™

Improving Thromboprophylaxis In Hospitalized Pediatric Inflammatory Bowel Disease Patients- A Quality Improvement Project, Amy Issa, Panam Kaur

Research Days

Background/Project Intent (Aim Statement): Pediatric inflammatory bowel disease (IBD) patients in acute flare have 1.5 to 3-fold higher risk of thromboembolism compared to the general population. Anticoagulation therapy is recommended for pediatric IBD patients who have ≥ 1 risk factors for venous thromboembolic events. At Children’s Mercy Hospital (CMH), approximately 80 patients/year are admitted for acute flare, of which, roughly 30% receive appropriate thromboprophylaxis. Lack of standardization of thromboprophylaxis and an educational gap amongst providers are significant barriers. Our goal is to standardize and improve the rates of thromboprophylaxis in IBD patients admitted at CMH from 30% to 60% by …

Mini-Ace® Low-Profile Appendicostomy Button, Joseph Lopez

Research Days

Purpose: Malone antegrade continence enemas (MACE) provide a conduit in which the patient can achieve improved continence, be clean of stool, and gain independence in maintaining bowel function. The Mini-ACE® is a low-profile balloon button that is used to facilitate administration of antegrade enemas. We sought to describe our practice and short-term outcomes.

Methods: We present our experience using the Mini-ACE® at Children’s Mercy – Kansas City from April 2019 to September 2020. Patient demographics, colorectal diagnoses, tube sizes, flush regimens, patient experience and outcomes were examined.

Results: Thirty patients were included; 18 (60%) were male. The average age at …

Umbilical Access In Laparoscopic Surgery In Infants Less Than 3 Months: Single Institution Retrospective Review, James Fraser

Research Days

Background/Objectives/Goal: Umbilical access in laparoscopic surgery has been cited as a potential factor for increased complications in low-birth-weight infants and those less than three months old. Previous series noted a self-reported complication rate of 10.6% among 329 pediatric surgeons via anonymous survey, citing carbon dioxide (CO2) embolism as the most common complication. We report four-year outcomes with blunt transumbilical laparoscopic access to examine the safety of this technique.

Methods/Design: Following IRB approval, a retrospective database of patients less than three months of age who underwent laparoscopic pyloromyotomy or inguinal hernia repair from 2016-2019 at a tertiary care academic center was …

Pediatric Pulmonary Artery Aneurysms Causing Pulmonary Hemorrhage: A Case Of Transcatheter Intervention While On Va Ecmo Support, Igor Areinamo

Research Days

Background: The incidence of pulmonary artery aneurysm (PAA) in children remains unknown. There are few reports of a transcutaneous interventional approach to address this type of pathology. Management of PAAs while on VA ECMO has not been described in pediatrics.

Objectives/Goal:

Methods/Design:

Case: 9-year-old previously healthy male presented with MRSA associated ARDS and sepsis requiring urgent VA ECMO cannulation. Chest CT done on ECMO day 22 showed development of right sided PAAs. Patient had an episode of significant pulmonary hemorrhage. Angiogram demonstrated two fusiform right PAAs, one of which had ruptured and was bleeding into the lung parenchyma. Patient had …

Congenital Mitral Valve Regurgitation, The Dilemma Of Repair Vs Replacement., Bianca Cherestal

Research Days

Background: Congenital mitral regurgitation is a rare condition and can be challenging to manage when presenting in the neonatal period

Objectives/Goal: Two week old male presented with poor weight gain, murmur and cardiomegaly on chest X-ray. Echocardiogram showed moderate to severe mitral regurgitation (MR) and suprasystemic pulmonary hypertension (PHN) (fig 1 a, b). The mitral valve (MV) leaflets were thickened and tethered with failure of central coaptation. PHN was classified as WHO I and II (due to persistent PHN of newborn and MR respectively). Inhaled nitric oxide, Enalapril and Furosemide were initiated. Cardiac catheterization revealed PVRi of 8.9 WU x …

Online Echocardiography Modules For Pediatric Cardiology Fellows, Sarah Studyvin, Doaa Aly, Tyler Johnson, Laura Kuzava, Alison Samrany, Nitin Madan, Sanket Shah

Research Days

Background: Adequate echocardiographic (echo) education is integral to successful cardiology training. Online education is proven to be effective in other specialties, but pediatric echo training data are lacking.

Objectives/Goal: Our aim was to design and assess the role of a novel online module-based curriculum to enhance pediatric cardiology fellows’ echo education.

Methods/Design: Four interactive modules, focused on principles and interpretation of a normal echo, were delivered to fellows. Content included high quality 2D and 3D echo clips as well as interactive quizzes and could be accessed from a phone, tablet, or computer. All fellows completed pre- and post-tests, validated by …

The Impact Of Early Tracheostomy On Neurodevelopmental Outcome Of Infants With Severe Bronchopulmonary Dysplasia Exposed To Postnatal Steroids, Amjad Taha

Research Days

Background: BPD is associated with long-term neurodevelopmental impairment. Tracheostomy is performed in 5-12% of severe BPD for prolonged ventilation. There is evidence that chronic ventilation with tracheostomy in severe BPD may facilitate neurodevelopment and lead to improved outcome. However, there is no consensus on the optimal timing of tracheostomy. A large multicenter study of infants with tracheostomy performed at <120 days of life had better neurodevelopmental outcomes at 18-22 months of age. Use of steroids postnatally to ameliorate the severity of BPD had been controversial due to its negative effect on neurodevelopment. To date, no data has specifically evaluated the impact of early tracheostomy on neurodevelopmental outcome of infants with severe BPD who are exposed to postnatal steroids.

Objectives/Goal: To compare the cognitive, language and motor scores among 3 groups of severe BPD infants who received early vs late vs no tracheostomy. Secondly, evaluate if postnatal steroids had an additive negative effect on neurodevelopmental outcomes.

Methods/Design: IRB approved retrospective …

Efficacy Of Using Antibiotic Cocktail In Medically Refractory Colitis/Inflammatory Bowel Disease, David Aaron Simon

Research Days

Background: Antibiotic cocktail targeting intestinal bacteria may offer a promising approach in certain patients with medically refractory IBD. It may find a place in serving as a bridge to more effective long-term medical therapies and in some cases help in achieving clinical remission in patients with refractory disease as an adjunctive therapy. Its main attractions include efficacy in about 50% of medically refractory patients and a non-immunosuppressive adjunct in treatment of children who may otherwise suffer from complications of untreated disease or heavy immunosuppression. The main drawbacks are lack of understanding of true mechanism of action, potential development of drug …

Bar Removal Following Minimally Invasive Pectus Excavatum Repair – Does Removal At 2 Years Affect Recurrence Or Satisfaction Rates?, Wendy Jo Svetanoff

Research Days

Background: Some patients with pectus excavatum require bar removal despite having the bar in for less than three years after minimally invasive placement (MIRPE). It is unknown whether early removal is associated with a higher recurrence rate or lower cosmetic satisfaction.

Objectives/Goal: The aim of this study was to review post-operative outcomes, specifically recurrence rate, and patient satisfaction in patients who underwent bar removal prior to three years.

Methods/Design: A retrospective review was performed of patients who underwent MIRPE between October 2006 and June 2017 and had bar removal less than 3 years after repair. Demographics, reason for bar removal, …

Constrictive Pericarditis After Repair Of A Ruptured Sinus Of Valsalva, Sarah Studyvin, Laura Kuzava

Research Days

Background: Constrictive pericarditis is an uncommon complication of cardiac surgery. We report a patient who developed constrictive pericarditis after ruptured sinus of Valsalva (RSOV) repair.

Methods: A 23-year-old male presented with exertional dyspnea one year after RSOV repair. TTE showed a small, circumferential effusion with thickened pericardium, ventricular septal bounce, left atrial enlargement, diastolic hepatic flow reversal, and trivial mitral regurgitation without stenosis. He underwent cardiac catheterization, which revealed elevated filling pressures (RVEDP 16 mmHg, LVEDP 18 mmHg), RVEDP/RVSP ratio < 0.5, and a low cardiac index (1.65 L/min/m2). Cardiac MRI confirmed pericardial thickening with paradoxic septal motion, dilated pulmonary veins and retrograde flow in the SVC.

Results: Pericardiectomy of thickened and adherent pericardium was performed. The central venous pressure decreased from 23 to 7 mmHg and …

Prenatal Diagnosis Of An Uncommon Form Of A Hypoplastic Left Heart Syndrome Variant., Anmol Goyal

Research Days

Background: Mitral valve dysplasia syndrome (MVDS) is a rare form of congenital heart disease, similar to hypoplastic left heart syndrome (HLHS). Prenatal identification is important for counseling, delivery planning and postnatal management.

Case: A 39 year-old woman underwent fetal echocardiography at ~34 weeks gestation for evaluation of fetal cardiomegaly and hydrops revealing: biatrial enlargement, mild-to-moderate mitral and tricuspid valve insufficiency, echobright mitral valve apparatus, biventricular dilation, severe LV fibroelastosis (EFE) and systolic dysfunction, mild hypoplasia of aortic valve annulus and aortic arch, and a thick restrictive atrial septum (RAS) with left to right flow (Fig 1a-c).

Decision‐making: Although critical aortic …

The Role Of Fractionated Exhaled Nitric Oxide (Feno) In Eosinophilic Esophagitis And The Relationship With Downstream Eosinophils, Panamdeep Kaur

Research Days

Background: Eosinophilic Esophagitis (EoE) is a chronic immune mediated disease of the esophagus characterized by symptoms of esophageal dysfunction and eosinophilic-predominant inflammation of the esophagus. It can lead to feeding difficulties, failure to thrive, and esophageal stricturing. Currently, the gold standard for diagnosis and assessing response to therapy is upper endoscopy and histopathological analysis of biopsies. A noninvasive, cost-effective, and low risk alternative that can aid in the management of EoE is not currently available. Previous studies assessing correlation of fractionated exhaled nitric oxide (FeNO) with degree of esophageal eosinophilic inflammation were low powered but noted a trend for association. …

Metreleptin And Metformin Use In An Infant With Congenital Generalized Lipodystrophy Secondary To Agpat2 Mutation, Cintya Schweisberger

Research Days

Background: Congenital generalized lipodystrophy (CGL) is a rare inherited disease characterized by widespread loss of subcutaneous fat and severe metabolic abnormalities. Metreleptin, a synthetic analog of leptin, is a treatment modality that has been shown to decrease fasting triglycerides, fasting glucose, and HbA1c. Metformin use in infants has only been described in a few case reports of CGL and Donohue syndrome (insulin receptor mutation), and there is no established dosing for this age group. We report metreleptin, insulin, and metformin use in an infant with type 1 CGL who presented with marked hypertriglyceridemia, hypoleptinemia, hyperglycemia, and transaminitis.

Clinical Case: A …

Neonatal Renal Failure In The Setting Of Anorectal Malformation: A Case Report And Literature Review., Wendy Jo Svetanoff, Asma Ahmed, Richard J. Hendrickson, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Anorectal malformations (ARMs) can occur in isolation or in association with other anomalies, most commonly those of the genitourinary systems. Morbidity and mortality are highest among patients who develop end-stage renal disease (ESRD) either from severe congenital anomalies (dysplastic kidneys) or from repeated infections in those who have vesicoureteral reflux or persistent recto-urinary fistulas. We describe our management strategy for a patient born with an ARM and bilateral dysplastic kidneys to highlight the nuances and complex decision-making considerations required in taking care of this complex patient population. Our patient is a male twin born at 32 weeks' gestational age who …

Survival Following Relapse In Children With Acute Myeloid Leukemia: A Report From Aml-Bfm And Cog., Mareike Rasche, Martin Zimmermann, Emma Steidel, Todd Alonzo, Richard Aplenc, Jean-Pierre Bourquin, Heidrun Boztug, Todd Cooper, A S. Gamis, Robert B. Gerbing, Iveta Janotova, Jan-Henning Klusmann, Thomas Lehrnbecher, Nora Mühlegger, Nils V. Neuhoff, Naghmeh Niktoreh, Lucie Sramkova, Jan Stary, Katharina Waack, Christiane Walter, Ursula Creutzig, Michael Dworzak, Gertjan Kaspers, Edward Anders Kolb, Dirk Reinhardt

Manuscripts, Articles, Book Chapters and Other Papers

Post-relapse therapy remains critical for survival in children with acute myeloid leukemia (AML). We evaluated survival, response and prognostic variables following relapse in independent cooperative group studies conducted by COG and the population-based AML-BFM study group. BFM included 197 patients who relapsed after closure of the last I-BFM relapse trial until 2017, while COG included 852 patients who relapsed on the last Phase 3 trials (AAML0531, AAML1031). Overall survival at 5 years (OS) was 42 ± 4% (BFM) and 35 ± 2% (COG). Initial high-risk features (BFM 32 ± 6%, COG 26 ± 4%) and short time to relapse (BFM …

Evaluation Of The Hypothesis That Viral Meningitis Is A Mimic Of Abusive Head Trauma, Danielle Horton

Research Days

Background: Viral meningitis (VM) has been proposed as an alternative cause of subdural hemorrhage (SDH) in young children diagnosed with abusive head trauma (AHT). It has been proposed that VM may mimic symptoms and clinical features of AHT, resulting in an incorrect diagnosis of abuse.

Objectives/Goal: We aim to evaluate the hypothesis that VM is a mimic of AHT by comparing the history of present illness (HPI) and initial clinical presentation of young children with proven VM to those with subdural hemorrhage and concomitant suspicious injuries (SDH + CSIs) and to those with SDH and no CSIs (SDH only). We …

Local Implementation And Standardization Of The Pediatric Severe Traumatic Brain Injury Guidelines, Michelle Brown

Research Days

Problem Statement/Question: Severe pediatric Traumatic Brain Injury (TBI) remains a significant cause of morbidity and mortality. Evidence-based guidelines leave gaps in how to best implement and deliver standardized care on the local hospital level.

Background/Project Intent (Aim Statement): We aim to improve clinical outcomes for severe TBI patients (Glasgow Coma Scale ≤8) with standardization of care according to updated evidence-based guidelines. We will target early guideline adherence (initial 72 hours of PICU admission) with a focus on 1) targeted temperature management (TTM40mmHg).

Methods (include PDSA cycles): The Children’s Mercy Hospital Pediatric ICU Severe TBI Guidelines were updated after the 3rd …

Simulation Based Clinical Systems Testing Of A Pediatric Ed To Improve Staff And Process Readiness For Pediatric Hypoglycemia, Kevin Meilak Md

Research Days

Background/Project Intent (Aim Statement): Hypoglycemia is the most common metabolic disorder in children in pediatric emergency department (ED) settings 1 . Children may present with nonspecific symptoms, or asymptomatically2 . So identification/treatment is very challenging. There is evidence in the adult literature that there is wide variability in the treatment of hypoglycemia3 . Currently Children’s Mercy does not have a standardized approach to the treatment of hypoglycemia not associated with diabetes mellitus. From observation in the clinical setting we believe there are gaps regarding the identification and treatment of hypoglycemia not associated with diabetes mellitus. Delayed recognition and under treatment …

Optimization Of Perioperative Prophylaxis In Penicillin-Allergic Labeled Patients, Katie A. Vandervelde

Research Days

Problem Statement/Question: A penicillin (PCN)-allergy label is life-long and has been shown to result in more costly, less effective, and broader-spectrum antibiotics being utilized for surgical prophylaxis. At CMH, 6.6% of surgical patients receiving perioperative antibiotics are labeled PCN-allergic. A common myth is that approximately 10% of patients with a PCN-allergy history will experience an allergic reaction if administered a cephalosporin, whereas recent observational studies have found crossreactivity rates between 0.17-0.7%. Most of these patients receive a cefazolin alternative unnecessarily. Currently, no process exists to identify and clarify PCN-allergic patients preoperatively. Data from November 2018-December 2019 found ~2.33 patients per …

Tnfaip3: Evolving Clinical Picture From Autoinflammatory Syndrome To Autoimmune Lymphoproliferative Syndrome, Esosa Adah Md

Research Days

Background: Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder of the immune system that can affect children and adults. The most frequent presentation is benign lymphoproliferation limited to lymphoid organs such as the lymph nodes, liver, and spleen which result in splenomegaly and lymphadenopathy, two prominent features in ALPS patients. ALPS can lead to anemia, thrombocytopenia, and neutropenia which increase risks of infection and hemorrhage. Most cases are caused by either a germline or somatic mutation in the FAS, FASLG, or CASP10 genes that are involved in cell apoptosis. Tumor Necrosis Factor Alpha, Induced Protein 3 (TNFAIP3) is a …

A Case Of Metabolic Genetic Emergency, Elizabeth Loughman

Research Days

Background: Newborn screening (NBS) is one of the largest public health initiatives to improve infant morbidity and mortality, and this has been shown to detect and lead to prompt treatment of uncommon but rapidly fatal conditions. While NBS has changed outcomes of diseases, it is still imperative that prompt and early recognition of abnormal results and medical interventions remain the mainstay of care.

Objective: To describe a case of inborn error of metabolism in a 4-day old asymptomatic infant, where early recognition and treatment affected outcome.

Design/Methods: This case is a 4-day old female, former 36-week gestational age infant who …

Association Between Blood Pressure And Esa Dose In Pediatric Patients On Dialysis, Heather A. Morgans

Research Days

Background: Hypertension is a reported side effect of Erythropoiesis Stimulating Agents (ESAs), with a mechanism of action related to elevated hematocrit levels and direct vasopressor effects. Limited information exists on the relationship between ESA dosage and hypertension in children receiving maintenance dialysis.

Objectives/Goal: The primary aim of this study was to determine whether there is a significant association between ESA dose and blood pressure (BP) in pediatric patients on dialysis. The secondary aim was to determine covariates in relation to ESA dose.

Methods/Design: Data from the International Pediatric Dialysis Network (IPDN) database was used to retrospectively analyze the association between …

Setting A Threshold For Discharge Antibiotics In Children With Perforated Appendicitis: A Study Update, Kayla B. Briggs

Research Days

Background: To address concerns of antibiotic overutilization, normal white blood cell count (WBC) for age was previous l criteria for discontinuing antibiotics at discharge in perforated appendicitis. Retrospective review suggested a decrease in threshold for additional antibiotic administration to a discharge WBC(IAA).

Objectives/Goal: The purpose of our study is to determine if lowering the threshold for additional antibiotic administration decreased the rate of IAA after laparoscopic appendectomy for perforated appendicitis in children who were not discharged with additional antibiotics.

Methods/Design: Following IRB approval, prospective data was retrospectively reviewed. Children with perforated appendicitis identified during laparoscopic appendectomy at our institution between …

Standardized Point-Of-Care Ultrasound (Pocus) Set-Up For Cardiac Evaluation During Covid-19 Pandemic In A Pediatric Population., Anmol Goyal

Research Days

Background: The novel coronavirus SARS-CoV2 is known to cause significant cardiac dysfunction, effusion, thromboembolism and coronary dilation. Currently no pediatric specific guidelines exist from ACC or ASE for use of POCUS. Our objective was to identify ideal pediatric appropriate POCUS equipment and standardize its use.

Methods: A Phillips Lumify probe paired with Android-Samsung tablet and a Butterfly iQ probe paired with an iPad were considered. A workflow algorithm and cleaning instructions were created. If the POCUS scan answered the clinical question, then a detailed echo was deferred. If the POCUS scan revealed findings requiring comprehensive evaluation, a follow-up echo was …

C-Reactive Protein Values To Predict Sepsis-Induced Inflammatory Response In Premature Infants, Megan H. Tucker

Research Days

Background: C-reactive protein (CRP) is an inflammatory marker that has been recognized as a biomarker of the systemic inflammatory response in preterm neonates. We hypothesized that initial and peak CRP values would correlate with the degree of sepsis-induced acute lung injury (ALI) as measured by the pulmonary severity score (PSS).

Objectives/Goal: 1) Determine if confirmed (CF) sepsis events are associated with higher initial and peak CRP values than rule out (RO) sepsis events. 2) Investigate if initial and/or peak CRP correlates with severity of sepsis-induced ALI as measured by the PSS.

Methods/Design: In this retrospective case control study, we included …

Kernicterus Spectrum Disorders Diagnostic Toolkit: Validation Using Retrospective Chart Review, Vijaya Dasari

Research Days

Background: Kernicterus Spectrum Disorders (KSDs) result from hyperbilirubinemia-induced brain injury. While rare, KSDs can result in devastating neurologic sequelae affecting motor function and tone, causing dystonia and choreoathetosis, and/or auditory neuropathy spectrum disorders. Families of children with undiagnosed neurodevelopmental disorders and a history of neonatal hyperbilirubinemia often question this potential diagnosis. We developed a Toolkit (KSD-TK) questionnaire in an effort to provide a standardized method to predict the likelihood of KSDs.

Objectives/Goal: This study compares the accuracy of the KSD-TK to clinical diagnoses made by our Children’s Mercy Hospital Kernicterus Center of Excellence (KCOE). The primary objective of this study …

Bivalirudin And Thrombolytic Therapy: A Novel Successful Treatment Of Severe Aortic Arch Thrombosis In A Term Neonate, Amjad Taha

Research Days

SUMMARY An early term infant with uncomplicated perinatal history was found to have a large thrombus in the aortic arch after he failed regular newborn critical congenital heart defect (CCHD) screen. He responded well to Bivalirudin thrombolytic and tissue-Plasminogen activator (tPA) combination therapy, with a significant resolution of the thrombus. The infant tolerated hospital admission well with no significant complications. He was discharged home on daily aspirin at two weeks of life. To our knowledge, the combination therapy approach with Bivalirudin and tPA is the first one reported in the literature in the neonatal age group.

BACKGROUND Aortic arch thrombosis …

Functional Outcomes Of Infants With Chiari Ii Malformation With Tracheostomy And Home Ventilator Dependence, Abbigayle Carlson

Research Days

Background: Chiari II malformation is relatively common with an incidence of about 1 in 1000 live birth and is associated with reduced life expectancy. Characteristic features are beaked midbrain, downward displacement of the tonsils, and cerebellar vermis, and spinal myelomeningocele that can be associated with hydrocephalus needing ventriculoperitoneal shunt (VP shunt). Mortality is about 50-70% if not recognized early. Survivors have high morbidity and develop neurologic abnormalities and significant developmental delays. Long-term outcome is poorly understood.

Objective: Not much has been published that describes the outcome of infant with Chiari II malformation that received tracheostomy for long-term ventilator support. Our …

Diagnosis And Management Of Otitis Media With Effusion In Pediatric Urgent Care Clinics, Ashley Deschepper

Research Days

Background: Otitis media with effusion (OME)’s clinical presentation is often confused with acute otitis media (AOM) by providers. Despite OME guidelines recommending watchful waiting with no antibiotics, rates of antibiotic use remain elevated. In our pediatric urgent care clinics (UCCs), an estimated 50% of patients diagnosed with OME receive antibiotic prescriptions.

Objectives/Goal: To determine the provider diagnosis validity and the rates of antibiotics prescribed among pediatric OME patients evaluated in 3 UCCs within a pediatric healthcare system.

Methods/Design: We randomly selected 75% of encounters for children age 0-18 years who had a billing diagnosis of OME in 2019. Charts were …

Activation Of Aryl Hydrocarbon Receptor Signaling In Human Trophoblasts Alters Markers Of Growth And Differentiation, Asmaa Alsousi

Research Days

Background: It is estimated that 1.7% of pregnant women smoke during their pregnancy globally, with the highest levels observed in Europe at 8.1%, and lowest in Africa at 0.8. The association of maternal cigarette smoking with increased risk of poor birth outcomes such as preterm birth, congenital anomalies, and neonatal mortality is well-established. In addition, evidence suggests that intrauterine exposure to maternal smoking impacts the risk of developing diseases later in life; however, we still do not understand the exact mechanism(s) leading to these outcomes. Once components of cigarette smoke (CS) cross the placenta and enter the fetal compartment, several …