Not-So-Apparent Mixing Lesions: Late Presentation Of Cardioembolic Stroke, 2021 Children's Mercy Kansas City
Not-So-Apparent Mixing Lesions: Late Presentation Of Cardioembolic Stroke, Amulya Buddhavarapu
Background: Multiple levels of inter-atrial shunting can rarely present late in life with dyspnea and embolic stroke from effects of bidirectional shunting.
Case: A 55-year-old male with history of a patent foramen ovale, atrial fibrillation and embolic stroke presented with worsening fatigue and dyspnea at rest. Echocardiogram showed mildly reduced ventricular function with severe right heart dilation. On transesophageal echo, the coronary sinus (CS) was severely dilated with a persistent left superior vena cava (LSVC). A stress test showed no perfusion defects. CT angiography showed an absence of right SVC and a single LSVC connecting to the CS. There was ...
Congenital Mitral Valve Regurgitation, The Dilemma Of Repair Vs Replacement., 2021 Children's Mercy Kansas City
Congenital Mitral Valve Regurgitation, The Dilemma Of Repair Vs Replacement., Bianca Cherestal
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 ...
Constrictive Pericarditis After Repair Of A Ruptured Sinus Of Valsalva, 2021 Children’s Mercy Hospital
Constrictive Pericarditis After Repair Of A Ruptured Sinus Of Valsalva, Sarah Studyvin, Laura Kuzava
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 ...
Umbilical Access In Laparoscopic Surgery In Infants Less Than 3 Months: Single Institution Retrospective Review, 2021 Children's Mercy Hospital
Umbilical Access In Laparoscopic Surgery In Infants Less Than 3 Months: Single Institution Retrospective Review, James Fraser
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 ...
Pediatric Pulmonary Artery Aneurysms Causing Pulmonary Hemorrhage: A Case Of Transcatheter Intervention While On Va Ecmo Support, 2021 Children's Mercy Kansas City
Pediatric Pulmonary Artery Aneurysms Causing Pulmonary Hemorrhage: A Case Of Transcatheter Intervention While On Va Ecmo Support, Igor Areinamo
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.
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 ...
Improving Thromboprophylaxis In Hospitalized Pediatric Inflammatory Bowel Disease Patients- A Quality Improvement Project, 2021 Children's Mercy Hospital
Improving Thromboprophylaxis In Hospitalized Pediatric Inflammatory Bowel Disease Patients- A Quality Improvement Project, Amy Issa, Panam Kaur
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 ...
Development Of A Gme Wellness Program At One Sponsoring Institution: One Year’S Journey, 2021 Lehigh Valley Health Network
Development Of A Gme Wellness Program At One Sponsoring Institution: One Year’S Journey, Despina Tsarouhis Bs, Margaret A. Hadinger Edd, Joseph E. Patruno Md, Carmine Pellosie Do, Mph, Mba, Daniel Relles Md, Kevin M. Correll Mdiv, Mapc, Richard Drabic Lcsw, Johnathon Dallarosa Md, Lekha Racharla Do
Department of Education
No abstract provided.
Local Implementation And Standardization Of The Pediatric Severe Traumatic Brain Injury Guidelines, 2021 Children's Mercy Kansas City
Local Implementation And Standardization Of The Pediatric Severe Traumatic Brain Injury Guidelines, Michelle Brown
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 ...
Simulation Based Clinical Systems Testing Of A Pediatric Ed To Improve Staff And Process Readiness For Pediatric Hypoglycemia, 2021 Children's Mercy Hospital GME
Simulation Based Clinical Systems Testing Of A Pediatric Ed To Improve Staff And Process Readiness For Pediatric Hypoglycemia, Kevin Meilak Md
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, 2021 Children's Mercy Kansas City
Optimization Of Perioperative Prophylaxis In Penicillin-Allergic Labeled Patients, Katie A. Vandervelde
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 ...
Phenotypic Characterization Of Jarid2-Related Intellectual Disability: A Case Series, 2021 Children's Mercy Kansas City
Phenotypic Characterization Of Jarid2-Related Intellectual Disability: A Case Series, Maxime Cadieux-Dion
Background: In recent years, wide implementation of research and clinical next generation sequencing has led to an astonishing number of novel disease-gene assertions. Recently, loss of function variants in JARID2 were reported in 16 patients with a clinically distinct neurodevelopmental phenotype that consisted of neurodevelopmental delay, intellectual disability (ID), learning disability, autism and behavioral abnormalities. Dysmorphic features were seen in most patients and included high anterior hairline, deep-set eyes, full lips, broad forehead, bulbous nasal tip, or depressed nasal bridge. Cleft lip/palate was observed in only 1/16 patients. Most cases were de novo, with only one inherited case ...
Functional Outcomes Of Infants With Chiari Ii Malformation With Tracheostomy And Home Ventilator Dependence, 2021 Children's Mercy Kansas City
Functional Outcomes Of Infants With Chiari Ii Malformation With Tracheostomy And Home Ventilator Dependence, Abbigayle Carlson
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 ...
Activation Of Aryl Hydrocarbon Receptor Signaling In Human Trophoblasts Alters Markers Of Growth And Differentiation, 2021 Children's Mercy Kansas City
Activation Of Aryl Hydrocarbon Receptor Signaling In Human Trophoblasts Alters Markers Of Growth And Differentiation, Asmaa Alsousi
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 ...
Bivalirudin And Thrombolytic Therapy: A Novel Successful Treatment Of Severe Aortic Arch Thrombosis In A Term Neonate, 2021 Children's Mercy Kansas City
Bivalirudin And Thrombolytic Therapy: A Novel Successful Treatment Of Severe Aortic Arch Thrombosis In A Term Neonate, Amjad Taha
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 ...
A Case Of Metabolic Genetic Emergency, 2021 Children's Mercy Kansas City
A Case Of Metabolic Genetic Emergency, Elizabeth Loughman
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 ...
Association Between Blood Pressure And Esa Dose In Pediatric Patients On Dialysis, 2021 Children's Mercy Hospital
Association Between Blood Pressure And Esa Dose In Pediatric Patients On Dialysis, Heather A. Morgans
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 ...
Evaluation Of A Symptom-Based Algorithm For Managing Battery Ingestions In Children, 2021 Children's Mercy Hospital
Evaluation Of A Symptom-Based Algorithm For Managing Battery Ingestions In Children, James Fraser
Background/Objectives/Goal: Complications from battery ingestion in the pediatric population are becoming more severe due to the emergence of stronger and smaller batteries. We previously established a symptom-focused, evidence-based algorithm for battery ingestion and seek to evaluate this protocol to investigate its safety and potential cost benefit. (Figure 1)
Methods/Design: Following IRB approval, all radiographs performed for foreign body ingestion between 2017-2020 were reviewed. All button battery ingestions were included to evaluate an institutional symptom-based algorithm emphasizing observation over intervention.
Results: Review of 2,237 foreign bodies series demonstrated 44 button battery ingestions. Median age at ingestion was ...
Mutant P53 Degradation By Potential Hsp40/J-Domain Protein Inhibitors Derived From A Natural Compound Plumbagin, 2021 Children's Mercy Hospital
Mutant P53 Degradation By Potential Hsp40/J-Domain Protein Inhibitors Derived From A Natural Compound Plumbagin, Mohamed A.A. Alalem
Background/Rationale: Accumulation of mutant p53 (mutp53) in cancer cells plays a crucial role in enhanced malignant properties by mutp53 oncogenic gain of function i(GOF). Given that mutp53 is required for cancer cells’ abilities to maintain the oncogenicity, promoting mutp53 degradation is a promising new avenue for anticancer treatment. Recently, we identified a key role of DNAJA1, a member of J-domain proteins (JDPs/HSP40) family, in protecting mutp53, mainly the unfolded type, from degradation. Inhibiting the activity of DNAJA1 or JDPs may lead to reduced cancer progression through inducing mutp53 degradation; however, there is no JDPs/HSP40-specific inhibitors that ...
Educational Impact Of Supervising Fellow Visits In The Emergency Department, 2021 Children's Mercy Kansas City
Educational Impact Of Supervising Fellow Visits In The Emergency Department, Grace E. Arends Md
Problem Statement/Question: After meeting certain criteria, pediatric emergency medicine (PEM) fellows were previously permitted to independently manage and discharge patients. These encounters anecdotally fostered autonomy but missed educational opportunities. We have previously reported that fellow positive perception of education and autonomy was low (22% and 11%). Additionally, the absence of an attending’s attestation on these patient charts prevented the collection of a professional service fee, an average potential billed amount of $125.67 per unit of service or total of $620,307 in 2019.
Background/Project Intent (Aim Statement): After key stakeholder involvement, we recognized attending supervision for ...
Smart Rounding: Development Of A Nurse-Driven Rounding Checklist As A Sustainable Intervention For Improved Care Communication, 2021 Internal Medicine/Pediatrics Residency, UMKC
Smart Rounding: Development Of A Nurse-Driven Rounding Checklist As A Sustainable Intervention For Improved Care Communication, Charles I. Maloy
Background/Project Intent: Use of checklists in the healthcare setting is increasingly common and is important to the development of high reliability and standard avenues for communication. At our tertiary care freestanding pediatric hospital, there was no widely used rounding checklist that discussed items important to quality care and patient safety outside of the intensive care unit. The development and implementation of medical checklists for the standard patient floor are inadequately described. We will develop and implement a rounding checklist with at least 80% daily checklist use sustained over 6 months. Team members will report improved multidisciplinary discussion of patient ...