Medical Specialties Commons^™

A Pediatric Case Of Treatment-Related Myelodysplastic Syndrome While On Therapy For Pre-B All, Sara Mcelroy

Research Days

Background: Treatment-related myelodysplastic syndrome (t-MDS) is a known, but rare, late effect of cancer therapy, specifically radiation, alkylating agents or topoisomerase II inhibitors. When secondary to treatment with alkylating agents, t-MDS typically occurs 4 to 7 years after therapy, and common cytogenetics include chromosomes 5 and 7 abnormalities. Treatment in children with t-MDS is typically allogeneic stem cell transplant, but the prognosis remains poor.

Objectives/Goal: To describe a rare case of a pediatric patient who developed t-MDS while receiving treatment for Pre-B Cell Acute Lymphoblastic Leukemia (pre-B ALL) and to outline the treatment regimen that he received.

Methods/Design: Case Report …

Therapeutic Plasma Exchange In Critically Ill Pediatric Patients With Leukemia, Sarah E. Mc Dermott, Chandni Dargan

Research Days

Background: Therapeutic plasma exchange (TPE) has well-documented applications in the adult population, outlined by the American Society of Apheresis (ASFA) 2019 guidelines. Limited data exists regarding the use of TPE in critically ill pediatric patients, however these reports rarely include patients with oncological diseases. Care for these patients poses certain clinical considerations including the safety of continuing chemotherapy, delayed clearance of chemotherapy secondary to end organ damage and mechanical clearance (i.e., continuous renal replacement therapy (CRRT) circuitry), and ability to tolerate chemotherapy once recovered. We aim to highlight the potential benefits of TPE in their acute management, so that it …

Atrial Standstill In A Pediatric Patient With Scn5a Mutation Following Procainamide Challenge, Anmol Goyal

Research Days

Background: Atrial standstill (AS) is a rare arrhythmia characterized by absence of electrical and mechanical atrial activity associated with SCN5A channelopathy.

Case: An 18 year old male with structurally normal heart, frequent sinus pauses, nonsustained atrial tachycardia and high-grade block was found to have SCN5A mutation c.3823G>A (p.Asp1275Asn). An electrophysiology study (EPS) with high density voltage mapping of the right atrium was done (Fig 1a). Nonsustained multifocal atrial tachycardia was induced without ablative targets (Fib 1b). Procainamide challenge was negative for Brugada, however induced AS (Fig 1c-d). No atrial capture could be achieved at maximal output. Empiric atrial lead …

Reconsidering Perioperative Antibiotic Use In Elective Laparoscopic Cholecystectomy, Kayla B. Briggs

Research Days

Background: As rates of antimicrobial resistance increase, the use of prophylactic perioperative antibiotics (PPA) has been questioned in cases with a low risk of surgical site infection (SSI). In laparoscopic cholecystectomy performed for biliary dyskinesia, hyperkinetic gallbladder, and gallbladder polyps, the use of PPA varies with no widely accepted practice pattern.

Objectives/Goal: We examined institutional PPA usage and SSI rates for elective laparoscopic cholecystectomy to determine if PPAs are indicated.

Methods/Design: Following IRB approval, children/2010 and 8/2020 for symptomatic cholelithiasis, biliary dyskinesia, hyperkinetic gallbladder, and/or gallbladder polyps were included. Several surgeons changed practice to selective PPA use in 2016, decreasing …

Do Males With 45,X/46,Xy Mosaicism Have Turner Syndrome, Michelle Knoll

Research Days

Background: Patients with 45,X/46,XY mosaicism can have genitalia spanning the range from typical female to typical male, and the sex assigned at birth is often based on the appearance of the genitalia. According to current nomenclature, only the subgroup assigned female are diagnosed with Turner syndrome (TS) (Gravholt et al, 2017). Clinical practice guidelines recommend that girls with TS be screened for several comorbidities, including short stature, cardiac and renal anomalies, and autoimmune conditions, but there are no recommendations for screening of boys with 45,X/46,XY mosaicism.

Objectives/Goal: We sought to determine if boys with 45,X/46,XY mosaicism exhibited a similar rate …

Changes In Family Functioning During The First Year Of Pediatric Cancer Treatment: A Mixed-Method Pilot Study, Carolyn Bates

Research Days

Background: A new diagnosis of pediatric cancer presents a sudden and tremendous stressor for families of all backgrounds and circumstances (Long & Marsland, 2011; Bemis et al., 2015). Although many families are resilient in the context of new cancer diagnosis, approximately 33% are at risk for negative outcomes due to psychosocial distress and difficulties with effective coping (Kazak et al., 2020; Pai et al., 2008), supporting the need for systematic assessment of psychosocial health as a standard of care in pediatric oncology (Kazak et al., 2015). Alongside heightened stress within the first year of diagnosis, parents may relax family rules …

Impact Of Covid-19 On Families With A Child In Cancer Treatment, Mirae J. Fornander

Research Days

Background: A new diagnosis of pediatric cancer diagnosis is a sudden and tremendous stressor to families (Long & Marsland, 2011). The COVID-19 pandemic presented an acute, universal stressor that impacted daily life around the world (Brooks et al., 2020). The impact of the COVID-19 pandemic on children with cancer and their families is not yet known.

Objectives/Goal: The current study is a pilot investigation of COVID-19 exposure and impact among a sample of families with a child being treated for cancer.

Methods/Design: Primary caregivers (N=22) of a child (M age=7.4, SD=4.6) who was diagnosed with cancer within the last 18 …

Pharmacogenetic Testing In Patients With Autism Spectrum Disorder Evaluated In The Children’S Mercy Hospital Goldiloks© Clinic, Rachel Goodson

Research Days

Background: Autism Spectrum Disorder (ASD) affects 1 in 54 children in the United States. Children with ASD are more likely to be diagnosed with co-occurring mental health disorders. There is currently little research guiding medication choice and dosing in patients with ASD. Children with ASD are at increased risk for reduced clinical response and adverse reaction (including due to polypharmacy). Pharmacogenomics is an approach for medication decision making that leverages an individual’s genetic information and clinical presentation to make informed choices, but no studies have specifically investigated the outcomes of PGX for patients with ASD. Understanding the clinical and genetic …

Outcomes Of Infants With Severe Rop At Risk Of Treatment With Avastin Compared To Laser Surgery, Stephen Doxey

Research Days

Background: Retinopathy of prematurity (ROP) is a well-known complication occurring in the most premature infants leading to visual impairment and in the most severe cases associated with retinal detachment and childhood blindness. Factors contributing the development of ROP include chronic hypoxia and disordered vascular genesis primarily through the vascular endothelial growth factor (VEGF) signaling pathway. Infants at risk of developing ROP are screened with serial eye exam and treated with either laser surgery or intraocular injects of bevacizumab (Avastin) to prevent progression of ROP and preserve vision. Bevacizumab, an inhibitor of VEGF signaling, may have positive effects on preventing ROP …

Hyperglycemia Requiring Insulin Among Pediatric Patients Diagnosed With Hemophagocytic Lymphohistiocytosis, Cintya Schweisberger

Research Days

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder marked by massive cytokine release due to macrophage and T-cell activation. Hallmarks of the diagnosis include fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogemia, and elevations in ferritin and soluble IL-2 receptor. Given HLH is associated with critical illness, elevation in inflammatory markers, and treated with glucocorticoids, the development of hyperglycemia during its course is not unexpected. However, detailed descriptions of the severity of hyperglycemia and strategies in insulin management among HLH patients are lacking. We describe 10 years’ experience at a single tertiary pediatric health center with HLH patients who developed insulin dependent …

A Screen Of Fda-Approved Drugs Identifies Inhibitors Of Protein Tyrosine Phosphatase 4a3 (Ptp4a3 Or Prl-3), Dylan R. Rivas, Mark Vincent C. Dela Cerna, Caroline N. Smith, Shilpa Sampathi, Blaine G. Patty, Donghan Lee, Jessica S. Blackburn

Molecular and Cellular Biochemistry Faculty Publications

Protein tyrosine phosphatase 4A3 (PTP4A3 or PRL-3) is highly expressed in a variety of cancers, where it promotes tumor cell migration and metastasis leading to poor prognosis. Despite its clinical significance, small molecule inhibitors of PRL-3 are lacking. Here, we screened 1443 FDA-approved drugs for their ability to inhibit the activity of the PRL phosphatase family. We identified five specific inhibitors for PRL-3 as well as one selective inhibitor of PRL-2. Additionally, we found nine drugs that broadly and significantly suppressed PRL activity. Two of these broad-spectrum PRL inhibitors, Salirasib and Candesartan, blocked PRL-3-induced migration in human embryonic kidney cells …

Mindset, Medicine And You, Gregory A. Jaffe, Md, Msed

Department of Family & Community Medicine Presentations and Grand Rounds

Objectives

• Describe the differences between a fixed and growth mindset
• State phrases that encourage growth mindset
• Apply growth mindset techniques to medical education

When Total Joint Arthroplasty After Septic Arthritis Can Be Safely Performed, Timothy L. Tan, Chi Xu, Feng-Chih Kuo, Elie Ghanem, Jaiben George, Noam Shohat, Ji-Ying Chen, Mel S Lee, Carlos Higuera, Javad Parvizi

Rothman Institute Papers

Background: Patients undergoing total joint arthroplasty (TJA) following septic arthritis are at higher risk for developing periprosthetic joint infection (PJI). Minimal literature is available to guide surgeons on the optimal timing of TJA after completing treatment for prior native joint septic arthritis. This multicenter study aimed to determine the optimal timing of TJA after prior septic arthritis and to examine the role of preoperative serology in predicting patients at risk for developing PJI.

Methods: A total of 207 TJAs were performed after prior septic arthritis from 2000 to 2017 at 5 institutions. Laboratory values, prior treatment, time from the initial …

Lessons Learned After 760 Neurointerventions Via The Upper Extremity Vasculature: Pearls And Pitfalls., Ahmad Sweid, Joshua H. Weinberg, Omaditya Khanna, Somnath Das, Julie Kim, Darcy Curtis, Batoul Hammoud, Kareem El Naamani, Rawad Abbas, Shyam Majmundar, Kalyan C. Sajja, Nohra Chalouhi, Fadi Al Saiegh, Nikolaos Mouchtouris, Elias Atallah, M. Reid Gooch, Nabeel A. Herial, Stavropoula Tjoumakaris, Victor Romo, Robert H. Rosenwasswer, Pascal Jabbour

Department of Neurosurgery Faculty Papers

BACKGROUND: The radial approach has been gaining more widespread use by neurointerventionalists fueled by data from the cardiology literature showing better safety and overall reduced morbidity.

OBJECTIVE: To present our institution's experience with the radial approach for neuroendovascular interventions in 614 consecutive patients who underwent a cumulative of 760 procedures.

METHODS: A retrospective analysis was performed and identified neuroendovascular procedures performed via the upper extremity vasculature access site.

RESULTS: Amongst 760 procedures, 34.2% (260) were therapeutic, and 65.7% (500) were nontherapeutic angiograms. Access sites were 71.5% (544) via a conventional radial artery, 27.8% (211) via a distal radial artery, 0.5% …

Genome Sequence Of A Virulent African Swine Fever Virus Isolated In 2020 From A Domestic Pig In Northern Vietnam, Quang Lam Truong, Thi Lan Nguyen, Thi Hoa Nguyen, Jishu Shi, Hiep Lai Xuan Vu, Thi Lan Huong Lai, Van Giap Nguyen

Nebraska Center for Virology: Faculty Publications

This study reports the genome sequence of an isolated African swine fever (ASF) virus (VNUA-ASFV-05L1/HaNam) obtained at the fourth passage on pulmonary alveolar macrophages. The virus was isolated during a typical acute ASF outbreak in pigs in a northern province of Vietnam in 2020.

Polycystic Ovarian Syndrome For The Pcp, Madeline Taskier, Md

Department of Family & Community Medicine Presentations and Grand Rounds

Objectives

• Define PCOS
• Outline varying diagnostic criteria
• Briefly review pathophysiology and diagnostic approach
• Review primary care management of PCOS symptoms
• Summarize role of PCP for clinical monitoring
• Explore influence of PCOS on fertility and pregnancy

Long-Term Safety Of Growth Hormone Treatment In Childhood: Two Large Observational Studies: Nordinet Ios And Answer., Lars Sävendahl, Michel Polak, Philippe Backeljauw, Joanne C Blair, Bradley S Miller, Tilman R Rohrer, Anita Hokken-Koelega, Alberto Pietropoli, Nicky Kelepouris, Judith L. Ross

Department of Pediatrics Faculty Papers

CONTEXT: Growth hormone (GH) treatment has a generally good safety profile; however, concerns about increased mortality risk in adulthood have been raised.

OBJECTIVE: This work aims to assess the long-term safety of GH treatment in clinical practice.

METHODS: Data were collected from 676 clinics participating in 2 multicenter longitudinal observational studies: the NordiNet International Outcome Study (2006-2016, Europe) and ANSWER Program (2002-2016, USA). Pediatric patients treated with GH were classified into 3 risk groups based on diagnosis. Intervention consisted of daily GH treatment, and main outcome measures included incidence rates (events/1000 patient-years) of adverse drug reactions (ADRs), serious adverse events …

The Power Of Outpatient Botulinum Injections In Preventing Hirschsprung Associated Enterocolitis, Wendy Jo Svetanoff

Research Days

Background: Hirschsprung associated enterocolitis (HAEC) is a potentially life-threatening complication, where the child often presents with obstructive symptoms. Failure of the internal anal sphincter (IAS) to relax is thought to play a role in these symptoms. Botulinum toxin (BT) injections assist in temporarily relieving obstructive symptoms due to IAS achalasia in patients with Hirschsprung’s disease following surgical pull-through. The use of BT may play a role in preventing HAEC episodes related to IAS dysfunction.

Objectives/Goal: Our aim was to determine the effect of scheduled outpatient Botulinum injections on the development of HAEC.

Methods/Design: A retrospective review between July 2010 – …

Iv Amiodarone Vs Iv Sotalol Use In Postoperative Junctional Ectopic Tachycardia (Jet): A Randomized Study, Tanner Isaacson

Research Days

Background: Junctional ectopic tachycardia (JET) is one of the most common post-operative arrhythmias encountered following congenital heart surgery. For many, amiodarone is the pharmacologic treatment of choice. While effective, amiodarone has been associated with dose-related adverse effects. Sotalol has been approved for intravenous use in pediatric patients in the United States to treat both supraventricular and ventricular arrhythmias, although pediatric data remains limited. Sotalol has class III antiarrhythmic properties with some mild β-adrenergic blocking effects, similar to amiodarone. Data shows a lower rate of cardiovascular collapse and adverse events with sotalol in patients with congenital heart disease. At present, it …

Long-Term Outcomes And Satisfaction Rates After Costal Cartilage Resection For Slipping Rib Syndrome, James Fraser

Research Days

Background/Objectives/Goal: Slipping rib syndrome (SRS) is a challenging and underdiagnosed condition that has proven to be an elusive diagnosis due to an extensive differential with presenting symptoms that mimic a myriad of alternative diagnoses of both thoracic and abdominal pathology. The diagnosis of SRS is recognized in a patient with consistent, progressive, often debilitating unilateral or bilateral chest wall and/or upper abdominal pain with intermittent clicking or rib slipping. SRS frequently does not resolve with conservative management, including physical therapy, rest, and NSAIDs, as well as intercostal injections, and even opioid medications. Workup can be extensive including multiple subspecialty referrals …

Multimodal Pain Control In Common Neonatal Surgeries: Post-Operative Pain Protocol Reduces Opiate Exposure And Side Effects, Jamesia Donato

Research Days

Background: Opioids are the primary post-operative (post-op) analgesic in neonates. Side effects include hypotension, apnea and ileus. Previous studies show IV acetaminophen decreases opiate need in specific populations including cleft palate repair, cardiac surgery. The effect of IV acetaminophen as part of a post-op pain algorithm is not known in common neonatal surgical procedures.

Objectives/Goal: To determine if (a) a post-op pain algorithm including IV acetaminophen and (b) IV acetaminophen exposure reduces cumulative opioid requirements in neonates undergoing common surgeries in the NICU.

Methods/Design: This is a retrospective cohort study comparing cumulative post-operative opiate exposure between infants with common surgeries …

The Effect Of Covid-19 On Pediatric Orthopaedic Trauma Volume At A Level 1 Pediatric Trauma Center In The Midwestern United States, Kirsten Norrell

Research Days

Background: School and playground closures and the cancellation of extracurricular activities in the Kansas City region on March 17, 2020 due to the COVID-19 pandemic created an unprecedented situation for children and parents. Early reports in the literature on adult and pediatric trauma volume suggested a decrease in orthopaedic injuries during the pandemic. This is the first study to report how the pandemic affected orthopaedic trauma volume at a Level 1 pediatric trauma center during the entire five months since school and activity closures went into effect.

Objectives/Goal: The aim of this study was to compare the volume and type …

Pre-Hospital Traumatic Cardiopulmonary Arrest In Children At A Level 1 Pediatric Trauma Center, Kayla B. Briggs

Research Days

Background: The survival of traumatic cardiopulmonary arrest (TCA) requiring pre-hospital cardiopulmonary resuscitation (P-CPR) is abysmal across age groups.

Objectives/Goal: We aim to describe the patterns of injury, pre-hospital interventions, and outcomes of children suffering from TCA leading to P-CPR at our institution to support standardized guidelines addressing the termination of prolonged resuscitation attempts in children.

Methods/Design: Following IRB approval, retrospective review was conducted to identify children ages 0-17 years who suffered TCA leading to P-CPR at our institution between 5/1/2009–3/1/2020. For analysis, patients were stratified into those still undergoing CPR at arrival and those who attained pre-hospital return of spontaneous …

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 …