Pediatrics Commons^™

Increasing Palliative Care Team Involvement In Pediatric Hematopoietic Stem Cell Transplant Patients, Meagan Vacek, Lisa Tarbell, Melissa List, Erin Hall, Jennifer Linebarger, Kelstan Ellis, Gina Jones, Joel Thompson

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Background: Palliative care facilitates communication, helps with physical and psychological symptom management, and assists in goals of care and advance care planning discussions.1 Multiple organizations, including the American Academy of Pediatrics and American Society of Clinical Oncology encourage palliative care engagement and their involvement with hematopoietic stem cell transplant (HSCT) patients has been shown to be beneficial as HSCT is associated with a high degree of morbidity and possible mortality.2-3 Objective: To increase the number of PaCT consults for patients receiving HSCT for our targeted diagnoses from 48% to 75% by December 2023. Methods: Chart review was conducted for patients …

Speeding Up Time: An Old Back In A Young Athlete, Carey Wagoner, Greg Canty

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Case of a 16 year old female competitive dancer with chronic back pain. Previous diagnosis of mechanical low back pain being treated by PT without improvement. Further workup demonstrated bilateral spinal synovial cysts at L4/L5 facet joints. Patient underwent IR guided aspiration and steroid injection with resolution of her symptoms and ultimately returned to dance.

Something's Gnawing At Me, Catharine Kral, Brian Harvey

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17-year-old female presented to Sports Medicine clinic with a chief complaint of bilateral hip pain and difficulty walking who was found to have bilateral ovarian teratoma.

Hearing Loss In Children With 22q11.2 Deletion Syndrome, Lori Yaktine, Amanda Moore, Jamie Hamm, Blaine Crowley, Meghan Tracy, Janelle R. Noel-Macdonnell Phd, Kim Graiser, Bernice Marrow, Hansoo Song, Srivats Narayanan, Nikita Raje, Donna Mcdonald-Mcginn, Jill M. Arganbright

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22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome, with most recent published prevalence of 1:2,140 live births. Hearing loss is common in children with 22q11.2DS and has been reported to occur in 32-60% of people. Hearing loss is typically conductive in nature secondary to chronic eustachian tube dysfunction and chronic otitis media with effusion (COME). Close monitoring of hearing and the middle ears is recommended to detect COME and the need for ear tubes. Additionally, there is a high prevalence of speech delay in children with 22q11.2DS, stressing the importance of close audiometric evaluation and medical intervention as …

Anterior Hip Pain In Adolescent Female Wrestler, Melanie Kennedy

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No abstract provided.

An Increased Failure Rate Of Asparaginase Desensitization With Calaspargase Pegol, Jillian Fry, Erin M. Guest, Keith J. August

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Background: In late 2022, pegaspargase (SS-PEG) became unavailable to patients younger than 22 years in the United States, leaving calaspargase pegol (SC-PEG) as the only available long-acting asparaginase formulation. SC-PEG has been compared to SS-PEG in two randomized, pediatric clinical trials and found to have similar rates of adverse events with comparable rates of event-free survival. Our institution uses a desensitization protocol for patients who experience a hypersensitivity reaction to asparaginase. Here we review our experience with desensitization since the transition to SC-PEG from SS-PEG. Objectives: To compare the rate of hypersensitivity reactions, success of desensitization protocols, and need for …

Diagnosis Of Mosaic Rasopathy In A Child With Rhabdomyosarcoma, Meagan Vacek, Paige Johnson, Midhat S. Farooqi, Kristi M. Canty, Dihong Zhou, Brendan Lanpher, Wendy Allen-Rhoades, Erin M. Guest

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Background: Phacomatosis pigmentokeratotica (PPK) is a subtype of epidermal nevus syndrome characterized by the co-existence of a sebaceous nevus and a speckled lentiginous nevus and described in approximately 30 cases in literature. PPK is now recognized as a mosaic RASopathy due a postzygotic mutation in the Ras-Raf-MEK-ERK pathway. RAS variants are also known to contribute to tumorigenesis, in some pediatric cancers, including rhabdomyosarcoma. Objective: Describe the presentation and evaluation of a child with pelvic rhabdomyosarcoma and evolving skin lesions found to have a rare mosaic-RASopathy. Design/Method: Case Report Results: The patient is a former 32-week premature female who presented to …

Anesthetic And Multidisciplinary Management Of A 2-Staged Liver Transplant​, Kasey Brooks, Lisa Conley, Brandon Layton

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We present a case of a 2 staged liver transplant on a 12-month-old 8kg male with newly diagnosed mitochondrial depletion syndrome causing acute liver failure, refractory lactic acidosis, and cardiopulmonary collapse requiring ECMO. The patient’s past medical history at the time of presentation included history of prematurity at 32 weeks, developmental delay, and a brother with an undiagnosed pancreatic disorder. The patient was brought in by parents to the emergency department with a cough, minimal PO intake for 5 days, and increased fatigue who was found to be URI positive. Initial labs were concerning for anemia, elevated bilirubin, elevated AST/ALT, …

Early Cholestasis And Vitamin K Deficiency Secondary To Biliary Atresia Presenting As Coagulopathy And Thymic Hemorrhage, Abbey Elsbernd, Lauren Amos Md

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Background Bleeding and progressive coagulopathy are established symptoms of early cholestasis, including extrahepatic biliary atresia. We present a patient with bleeding secondary to vitamin K deficiency and cholestasis-induced fat malabsorption with unusual presentation of mediastinal mass. Objective Describe a case of late-onset vitamin K deficiency and cholestasis ¬¬presenting as jaundice and coagulopathy with associated bleeding into thymic structures Design/Method Case report Results A 4-week-old term male infant was brought to the ED with fussiness and bruising. Prior to presentation, he was afebrile and followed regularly with his primary care provider for jaundice below phototherapy thresholds. Infant received vitamin K at …

Single Center Retrospective Comparison Of Bivalirudin And Heparin For Therapeutic Anticoagulation In Pediatric Patients, Clayton Habiger, Shannon L. Carpenter

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Background: The pharmacological choice for continuous anticoagulation therapy in pediatric patients has classically been unfractionated heparin. However, with heparin there are risks of treatment resistance in pediatrics given differences in developmental hemostasis, and contemporary medications such as bivalirudin offer a valuable alternative. Bivalirudin binds to thrombin and has shown encouraging results compared to heparin in pediatric patients receiving ECMO, but no comparison has been made in patients receiving therapeutic anticoagulation. Three years ago, Children’s Mercy Pediatric Hematology department encouraged bivalirudin use over heparin use for therapeutic anticoagulation requiring a continuous infusion. Objectives: Compare clinical and laboratory outcomes in patients receiving …

Transition Skills Lab: A Telehealth Intervention For Improving Healthcare Transition In Youth With Special Health Care Needs, Angela Combs Ma, Alaina Linafelter, Jordan Sevart, Michele H. Maddux

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Introduction: Few interventions exist to provide hands-on practice of key health management tasks to youth with special health care needs (YSHCN). The Transition Skills Lab was adapted from Jerlym Porter’s work and piloted as a virtual intervention in YSHCN. Methods: Sixteen young adults participated in the virtual Transition Skills Lab, during which they practiced three key patient competencies pertaining to health care tasks: reading an insurance card, reading a pill bottle label, and scheduling an initial visit with an adult doctor. Participants were then asked to rate their confidence on skills taught within the intervention on a four-point scale from …

Congenital Heart Defects And Autism: Understanding The Breakdown Of Associated Risk Factors In A Clinically Referred Sample, Elizabeth Raines, Amanda Strasser, Amanda Manderfeld, Paul Glasier, Elizabeth J. Willen

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Background: Children with Congenital Heart Disease (CHD) have higher odds of developing social difficulties and/or an Autism Spectrum Disorder (AuSD) than the general population (i.e., ~10% vs. ~1%). However, there is a paucity of nuanced understanding of specific drivers of the increased rates of AuSD in extant literature. The purpose of this study is to identify the rates of co-occurring cardiac, neurological, and genetic conditions to better understand associated risk factors in a patient sample from a medium-size children’s hospital. Methods: Our population includes a clinically referred sample of children (i.e., medical history of CHD and neurodevelopmental risk) under 18 …

Parents’ Perception Of An Intensive Interdisciplinary Pain Treatment For Amplified Pain Syndrome, Jacqueline Pabis Phd, Sabrina Ung, Jenny Scheurich, Dustin Wallace Phd

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Introduction: Intensive interdisciplinary pain treatment (IIPT) is an effective treatment for youth impaired by chronic pain (Claus et al., 2022). IIPT programs incorporate parents in treatment, as parental distress has been found to impact health and functioning in youth with chronic pain. Research has suggested parent satisfaction may be related to increased child adherence, and in turn, reduced pain (Gorodzinsky et al., 2012). There is limited research on parental satisfaction with IIPT and the role of parent-provider communication. Method: Parents (n=99) of children in an IIPT program completed questionnaires related to treatment satisfaction, parent-provider communication (e.g., provider listened carefully, provider …

Pediatric Site Response To Rapid Implementation Of Decentralized Clinical Trials, Abby Kietzman, An-Lin Cheng

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In 2020, the COVID-19 pandemic drastically altered the way clinical research is conducted through a widespread adoption of telemedicine practices. Using telehealth/telemedicine services allowed researchers to conduct “decentralized clinical trials”, or DCTs, for all or portions of study protocol procedures while maintaining safety measures implemented for pandemic control. However, current review of available literature details the explosion in use of decentralized clinical trials, resulting in gaps in evidence-based outcomes and lack of standardization across research entities. While sponsors and contract research organizations (CROs) tend to lead the charge around adoption of DCTs, research sites are hesitant to make the transition …

Double Aortic Arch With Atresia Of The Left Aortic Arch Proximal To The Left Common Carotid Artery In A Patient With Phace Syndrome – A Management Conundrum, Mohamed Aashiq Abdul Ghayum, Anmol Goyal, Aliessa P. Barnes, Sanket Shah

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Background Cardiac anomalies in PHACE syndrome (P: posterior fossa anomalies, H: hemangiomas, A: arterial lesions, C: cardiac abnormalities, E: abnormalities of the eye) often consists of complex aortic arch anomalies with double aortic arch (DAA) being an uncommon association. Atresia of the proximal segment of the left arch in DAA is extremely rare and can pose a diagnostic and management conundrum due to formation of a loose vascular ring and asymptomatic nature as seen in our case. Case Description A 5-year-old male, diagnosed with PHACE syndrome, at a different center was noted to have an incidental finding on head imaging …

Transposition Of The Great Arteries With Intramural Left Main Coronary Artery: Salient Imaging Findings And Description Of Two Operative Techniques, Joshua Holbert, Manasa Gadiraju, Sanket Shah, Edo Bedzra

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Background: Transposition of the great arteries (TGA) is a common cyanotic critical congenital heart disease, with a feasible prenatal diagnosis. Arterial switch operation (ASO) with/without VSD closure is the preferred surgical approach with added challenge when an intramural coronary artery (IMC) is present. ASO is technically quite demanding with IMC, and incidence of post-operative complications and mortality are potentially higher. We present our recent TGA-IMC experience focusing on salient features identified on echocardiography, CT and invasive angiograms, as well as variations in ASO surgical techniques for repair. Methods: Our echo imaging acquisition required high frequency transducers and shallow image depth …

Importance Of Tropical Medicine, Chia-Chi Angela Fu, A Clinton White, Miguel Cabada, Ryan Northup

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Overviews the importance of tropical medicine to society and global health.

Safe Sleep Education For Local Refugee And Immigrant Populations, Sara Jones

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Provide Safe Sleep education to local refugee and immigrant populations through their resettlement agency Jewish Vocational Services (JVS) with resources available in their first language.

Himalayan Health Exchange: Kargiakh Medical Expedition, Rachel Oldfather

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Medical expedition to remote Himalayan villages involving high-altitude treks, improvised clinics, and immersion in the local Buddhist culture.

Global Health Elective - Philippines, Hannah Ho

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Provides an overview of experiences working in the Philippines as a pediatric resident.

Expanding The Winter Warmth, Sarah Gwazdacz

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Reviews the efforts to expand collection of winter apparel for refugee families in Kansas City.

Healthcare In Under-Resourced Areas, Gabrielle Fleming, Ryan Northup, Bill Houseworth, Lori Housworth

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Describes pediatric resident's experience working in the inpatient unit, ER, PICU, and NICU in Cambodia.

Ada Deficiency: Improvement In Immune Function After Enzyme Replacement Therapy, Esosa Adah, Nikita Raje

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Introduction: Adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID) is characterized by severe lymphocytopenia affecting T-, and B-,lymphocytes, with non-immunological manifestations including neurodevelopmental deficits, sensorineural deafness, and skeletal abnormalities. Treatment modalities include enzyme replacement therapy(ERT) and/or autologous gene therapy or hematopoietic stem cell treatment. We report a 9-month-old female with ADA deficient T-B-NK+ SCID treated with ERT while awaiting stem cell treatment. Case Description: A 9-month-old female with a history of failure to thrive, hypotonia and loss of developmental milestones had genetic testing done that showed variants ADA c.911 T>G, c.302G>A. She was admitted for further work up. Lymphocyte …

Bowel Cleanout Prior To Intrathecal Baclofen Pump Implantation: Effects On Acute Post- Operative Gastrointestinal Complications, Amanda Lindenberg, Brittni Walton, Sathya Vadivelu, Matthew J. Mclaughlin

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Background: Intrathecal baclofen (ITB) pumps have been used for management of spasticity and dystonia There is limited data on immediate post-operative complications such as GI adverse events after surgery for ITB pump related procedures. This study aimed to compare patients who underwent a pre-operative bowel cleanout to those who did not and the relationship with post-operative nausea, vomiting and constipation requiring an escalation of their bowel regimen. Design: Retrospective cohort study. Setting: Pediatric acute care hospital. Participants: All patients who underwent new ITB pump and/or catheter placement from January 2015-December 2022. Interventions: Preoperative bowel clean out. Main Outcome Measure: Quantity …

Increasing Annual Eye Exam Completion For Children With Cystic Fibrosis Receiving Cftr Modulators, Paula Capel, Claire Elson, Stephanie Duehlmeyer, Jessica Haynes, Amanda Conway, Christopher M. Oermann

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Background: CFTR modulators are life-changing therapy for people with CF. During preclinical studies of ivacaftor, cataracts were identified in juvenile rat pups. Subsequently, noncongenital cataracts have been reported in children receiving CFTR modulator therapy [1], so baseline and annual slit lamp eye examinations for cataract development are recommended for children with CF (aged <18). At Children’s Mercy Kansas City (CMKC), our standard practice is to conduct baseline and annual ophthalmologic examinations to assess for cataracts in children with CF prescribed CFTR modulator therapy, but there was no formal process for tracking adherence to this standard and ensuring that examinations were completed. A quality improvement project was undertaken in May 2022 to determine the rate of completion of annual eye exams and increase adherence to our annual monitoring standard.

Methods: To improve adherence to annual monitoring standards, a quality improvement taskforce including CF care team nurses and pharmacists was developed. A database was used to document baseline and follow-up eye examinations. A standardized communication process was developed and deployed that included an eye exam brochure (Figure …

Investigating The Association Between Mast Cell Activation Syndrome And Irritable Bowel Syndrome, Ishaan Jakhar, Maggie Urschler, Medha Singh, Suman Sahil, Hassan Ahmad

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Investigating the Association between Mast Cell Activation Syndrome and Irritable Bowel Syndrome Introduction Mast Cell Activation Syndrome (MCAS) involves the inappropriate release of chemical mediators resulting in varied episodic symptoms. Irritable Bowel Syndrome (IBS) is a common functional abdominal disorder where mucosal immune alterations play a key role. Since mast cells function as gatekeepers for the intestinal interface, we examined what proportion of patients diagnosed with MCAS also had IBS along with comorbid risk factors. Methods All patients in our study were 18 years of age or older. We used Cerner Healthfacts to gather our cohorts of patients with MCAS, …

Evaluating A Newborn Infant With Pancytopenia For Whim Syndrome, Shayan Sazdar, Thao Le, Nikita Raje

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Introduction Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare immunodeficiency caused by a gain-of-function mutation in CXCR4. A complication of WHIM syndrome is severe neutropenia, which can result in fatal bacterial infections. Given the rarity of the disease and variability of clinical presentations, WHIM syndrome can be difficult to diagnose and manage. Case Description A full term male infant was hospitalized for hypoxic ischemic encephalopathy after delivery. Physical exam was unremarkable. During his hospitalization, he was found to have progressive pancytopenia. Initial lymphocyte subsets showed a significant T cell lymphopenia of 1325 mm3 CD3+, 920 mm3 CD4+, 390 …

Evaluating Patients With Recurrent Fever And Elevated Igd, Thao Le, Nikita Raje

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Introduction: Hyperimmunoglobulinemia D with periodic fever syndrome (HIDS) often presents within the first year of life. Symptoms include fevers, chills, headache, lymphadenopathy, and abdominal pain. Patients may be misdiagnosed as other autoinflammatory syndromes, resulting in delay of treatment. Case: A 16-year-old female presented for evaluation of recurrent fever. Her fevers started at two months of age, occurred one to two times a month, and were associated with headache, abdominal pain, and fatigue . At 18-months, she was diagnosed with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA). She was treated with prednisone and an adenotonsillectomy but her fevers persisted. . At …

Understanding Macrophage Phagocytosis In Pediatric Leukemia, Molly Leyda, Jacqelyn Nemechek, John Szarejko, Fang Tao, Tykeem Manor, Douglas Myers, John M. Perry

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Macrophages are a diverse and widespread type of innate immune cell which play an important role in homeostasis and defense. In a process called phagocytosis, macrophages engulf dying cells, foreign substances, and pathogens. Importantly, macrophages can present antigens from phagocytosed cells allowing them to initiate an adaptive immune response against remaining cells of the same type. Despite the immunosuppressive nature of the tumor microenvironment, macrophages often infiltrate tumors where they can either promote or inhibit cancer development, though the specific conditions influencing their pro- vs anti-cancer activity remain enigmatic. Recently, macrophages have been brought into the spotlight of immunotherapy research …

Factors Associated With Respiratory Pathogen Panel Utilization In Children Hospitalized With Acute Respiratory Illness — New Vaccine Surveillance Network, Kansas City, 2017–2021, Edward Lyon, Brian R. Lee, Benjamin Clopper, Heidi L. Moline, Rangaraj Selvarangan, Jennifer Schuster

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Background: Respiratory pathogen panels (RPP) are multiplex PCR platforms that detect several respiratory viruses from one specimen. For most children hospitalized with acute respiratory illness (ARI), management is supportive, and detection of a specific virus from RPP does not impact clinical care. Therefore, clinical RPP use is not standardized, and ordering is at the discretion of the clinician. We sought to understand factors associated with RPP utilization among pediatric patients hospitalized with ARI. Methods: From October 2017 to September 2021, participants < 18 years hospitalized with ARI were enrolled at a single site in the New Vaccine Surveillance Network (NVSN). Eligible patients were residents of Jackson County, MO, had one or more ARI symptoms (e.g., cough, fever, nasal congestion) lasting < 14 days, and were enrolled within 48 hours of admission. Parent interviews and medical chart reviews were conducted. All participants had a research RPP, but results were not available to the clinical providers. Clinical providers were able to order a clinical RPP (cRPP), for which they received test results. Characteristics of NVSN enrollees hospitalized with ARI with and without a cRPP are described. Lastly, medical complexity was assessed via the pediatric complex chronic conditions classification system (CCC) then analyzed via chi- square test between groups. Findings: During the study period, 1,038 participants were enrolled, and 555 (53.4%) received a cRPP. Most, 299 (53%), cRPPs were ordered in the emergency department or urgent care before admission. Age was a significant factor associated with cRPP use (Table 1). cRPP participants were more likely to have complex chronic conditions, and/or technology dependence. No difference in cRPP use was observed by race/ethnicity, payer status, or sex. More participants were enrolled in 2020-2021, but the overall usage of cRPP is similar between years (Table 2). Conclusions: In this large cohort of children hospitalized with ARI, medical complexity, technological dependence, and age < 2 months were associated with increased utilization of cRPPs. Understanding the impact of cRPP on clinical care requires further investigation to better understand the utility of these tests.