Medicine and Health Sciences Commons^™

Ethically Addressing Vaccine Hesitancy In Pediatric Populations, Tommie L. James Iii

Theses and Graduate Projects

Vaccines are highly regarded in the scientific and medical community for their efficacy in the prevention of disease; yet the prevalence of misinformation and mistrust surrounding vaccines is growing in communities around the world. Specifically observed in the United States (US) as an increase in vaccination exemption rates, this trend of vaccine hesitancy is leading to an increasing number of parents and caregivers to either refuse or delay vaccinations from the recommended schedule. As a result, an increasing number of outbreaks of diseases such as measles and pertussis, thought to be on the verge of elimination, have spiked. While health …

Discrete Left Ventricle Outflow Tract Obstruction In Children: Incidence And Predictors Of Recurrence. A Multi-Center Study, Gadah M. Alqurashi, Rema S. Almohanna, Kamal Mk Ayoub, Arwa A. Alkhuraiji, Najla A. Almasoud, Amjad R. Alsubaie, Alaa M. Althubaiti, Abdullah A. Al Sehly

Journal of the Saudi Heart Association

Objectives: The purpose of this study is to measure the incidence of recurrence of discrete subaortic stenosis (DSS) after primary resection in two major cardiac centers in Saudi Arabia and to identify risk factors associated with recurrence.

Methods: Data on 234 patients who were diagnosed with DSS and underwent surgical resection between 1999 and 2018 were retrospectively reviewed. Patient demographics as well as echocardiographic, surgical, and pathological data were compared between patients with recurrence and non-recurrence.

Results: The overall recurrence incidence after primary resection was 44.87% (N=105). Most patients were male (59%). The median age at the …

Reducing Emotional Distress For Childhood Hypoglycemia In Parents (Redchip): Protocol For A Randomized Clinical Trial To Test A Video-Based Telehealth Intervention., Susana R. Patton, Andrew Mcconville, Arwen M. Marker, Alexandra D. Monzon, Kimberly A A. Driscoll, Mark A. Clements

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Despite the introduction of new insulin analogs, insulin pumps, and continuous glucose monitoring (CGM), young children with type 1 diabetes mellitus (T1D) remain vulnerable to episodes of hypoglycemia because of their unpredictable eating and activity patterns and high degree of insulin sensitivity. Caregivers and young children living with T1D learn to fear hypoglycemia because it is uncomfortable, unpredictable, and dangerous. Up to 60% of caregivers of young children with T1D report moderate to severe levels of fear of hypoglycemia, and caregiver fear of hypoglycemia relates to lower quality of life for families and suboptimal child glycemic control. Yet, until …

Interstage Home Monitoring For Infants With Single Ventricle Heart Disease: Education And Management: A Scientific Statement From The American Heart Association., Nancy A. Rudd, Nancy S. Ghanayem, Garick D. Hill, Linda M. Lambert, Kathleen A. Mussatto, Jo Ann Nieves, Sarah Robinson, Girish S. Shirali, Michelle M. Steltzer, Karen Uzark, Nancy A. Pike, American Heart Association Council On Cardiovascular And Stroke Nursing; Council On Lifelong Congenital Heart Disease And Heart Health In The Young; Council On Arteriosclerosis, Thrombosis And Vascular Biology; Council On Clinical Cardiology; And Council On Lifestyle And Cardiometabolic Health

Manuscripts, Articles, Book Chapters and Other Papers

This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in-hospital readiness for …

Circus In Motion: A Multimodal Exergame Supporting Vestibular Therapy For Children With Autism, Oscar Peña, Franceli L. Cibrian, Monica Tentori

Engineering Faculty Articles and Research

Exergames are serious games that involve physical exertion and are thought of as a form of exercise by using novel input models. Exergames are promising in improving the vestibular differences of children with autism but often lack of adaptation mechanisms that adjust the difficulty level of the exergame. In this paper, we present the design and development of Circus in Motion, a multimodal exergame supporting children with autism with the practice of non-locomotor movements. We describe how the data from a 3D depth camera enables the tracking of non-locomotor movements allowing children to naturally interact with the exergame . A …

Development Of An Lc-Esi-Ms/Ms Method For Determination Of A Novel Pyrrolomycin (Mp-1) And Application To Pre-Clinical Adme Studies, Wafaa N. Aldhafiri

Theses & Dissertations

A rapid, selective, and sensitive liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS) method was developed and validated for quantitation of a novel Pyrrolomycin (MP-1) in mouse plasma. MP-1 was extracted from plasma utilizing a structural analog (PL-3) as the internal standard (IS). Analyte separation was achieved using a Waters Acquity UPLC®BEH C18 column (1.7 µm, 100 x 2.1 mm) protected with Acquity UPLC C18 guard column. Mobile phase consisted of 0.1% acetic acid in water (10%) and methanol (90%) at a total flow rate of 0.25 mL/min. The mass spectrometer was operated at unit resolution in the multiple reaction …

Pediatric Sjs-Ten: Where Are We Now?, Michele Ramien, Jennifer Goldman

Manuscripts, Articles, Book Chapters and Other Papers

Stevens-Johnson syndrome and toxic epidermal necrolysis are rare severe blistering skin reactions triggered by medications or infections. Over the last 5 to 10 years, a number of important publications have advanced understanding of these diseases and their response to treatment. Importantly, a subset of patients with disease triggered by infection has been identified as having Mycoplasma pneumoniae-induced rash and mucositis, suggesting a reconsideration of the diagnostic paradigm. We present an update on pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis in the broader context of cutaneous adverse drug reactions and focus on challenges and recent advances in diagnosis, management, and prevention.

Management Practices For West Syndrome In South Asia: A Survey Study And Meta-Analysis, Priyanka Madaan, Prem Chand, Kyaw Linn, Jithangi Wanigasinghe, Mimi Lhamu Mynak, Prakash Poudel, Raili Riikonen, Amit Kumar, Pooja Dhir, Sandeep Negi, Jitendra Kumar Sahu

Department of Paediatrics and Child Health

Objectives: Considering the dearth of literature on West syndrome (WS) from South Asian countries, this study aimed to evaluate the management practices in South Asia by an online survey and meta-analysis.
Methods: An online questionnaire was sent to 223 pediatric neurologists/pediatricians in India, Pakistan, Myanmar, Sri Lanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta-analysis.
Results: Of 125 responses received (response rate: 56%), around 60% of responders observed male preponderance and an approximate lead-time-to-treatment (LTTT) of 4-12 weeks. The commonest etiology observed was a static structural insult (88.6% of responders). Most commonly used first-line drug …

Fatal Case Of Newborn Lassa Fever Virus Infection Mimicking Late Onset Neonatal Sepsis: A Case Report From Northern Nigeria, Taofik Oluwaseun Ogunkunle, Surajudeen Oyeleke Bello, Chinwe Immaculata Anderson, Rashida Musa, Rasaq Olaosebikan, Abdulazeez Imam

Department of Pharmacology and Experimental Therapeutics Faculty Papers

BACKGROUND: Lassa fever is a zoonotic viral infection endemic to the West Africa countries. It is highly fatal during pregnancy and as such reports of neonatal onset Lassa fever infections are rare in scientific literature. We report a fatal case of Lassa fever in a 26-day-old neonate mimicking the diagnosis of late-onset neonatal sepsis.

CASE PRESENTATION: The patient is a 26-day-old neonate who was admitted with a day history of fever, poor feeding, pre-auricular lymphadenopathy and sudden parental death. He was initially evaluated for late onset neonatal sepsis. He later developed abnormal bleeding and multiple convulsions while on admission, prompting …

Stress And Negative Affect As Mediators In The Association Between Parental Social Support And Lung Function In Adolescents With Asthma, Amber Osorno, Eric Sternlicht, Pornchai Tirakitsoontorn, Azucena Talamantes, Anchalee Yuengsrigul, Zeev N. Kain, Brooke Jenkins

SURF Posters and Papers

Asthma is the leading chronic condition amongst children in the United States as 7.5% of children are diagnosed with asthma. Studies have shown that positive social support is associated with positive asthma management. A strong social support system predicts good management of asthma symptoms; however, current literature has not yet examined how social support impacts lung function as opposed to merely symptom management. Stress and negative affect have been revealed to be associated with worse asthma control, as well as exacerbation of symptoms. Stressful situations, such as the death of a family member, unemployment, and familial tensions lead to worsening …

The Spectrum Of Primary Immunodeficiencies At A Tertiary Care Hospital In Pakistan, Sonia Qureshi, Fatima Mir, Samina Junejo, Khalid Saleem, Samreen Zaidi, Abdullah B. Naveed, Khalil Ahmad, Farah Naz Qamar

Department of Paediatrics and Child Health

Background: Primary Immunodeficiency Disorders (PIDs) are well-known disorders in the West. but the recognition and diagnosis of these disorders is challenging in developing countries. We present the spectrum of PIDs seen at a tertiary care center in Pakistan, identified using clinical case definitions and molecular methods.
Methods: A retrospective chart review of children suspected to have PID was conducted at the Aga Khan University Hospital (AKUH) Karachi, Pakistan from 2010 to 2016. Data on demographics, clinical features, family history of consanguinity, sibling death, details of laboratory workup done for PID and molecular tests targeted panel next generation sequencing (NGS) or …

Patient Experience In A Pediatric Emergency Department During Covid-19, Beth L. Emerson Md, Erika Setzer

Patient Experience Journal

The COVID-19 pandemic has changed many dynamics in healthcare in the United States. This study explores an increase in patient experience (PE) scores in a pediatric emergency department. Visits were analyzed before and after March 8, 2020, corresponding with the first local case of COVID-19. Changes in the patient population and characteristics of survey responders were analyzed. Overall, the number of daily visits decreased (113 vs 36/day) and survey response rate decreased (3.7 vs 2.8%, p = 0.03), but PE scores increased (87.21 to 93.73, p = 0.002). Comparatively, an increase in patients with higher acuity levels by Emergency Severity …

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From The International Kawasaki Disease Registry., Brian W Mccrindle, Cedric Manlhiot, Jane W Newburger, Ashraf S Harahsheh, Therese M Giglia, Frederic Dallaire, Kevin Friedman, Tisiana Low, Kyle Runeckles, Mathew Mathew, Andrew S Mackie, Nadine F Choueiter, Pei-Ni Jone, Shelby Kutty, Anji T Yetman, Geetha Raghuveer, Elfriede Pahl, Kambiz Norozi, Kimberly E Mchugh, Jennifer S Li, Sarah D De Ferranti, Nagib Dahdah

Paediatrics Publications

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA

Ewing Sarcoma Family Of Tumors-Derived Small Extracellular Vesicle Proteomics Identify Potential Clinical Biomarkers., Glenson Samuel, Jennifer Crow, Jon B. Klein, Michael L. Merchant, Emily Nissen, Devin C. Koestler, Kris Laurence, Xiaobo Liang, Kathleen Neville, Vincent S. Staggs, Atif Ahmed, Safinur Atay, Andrew K. Godwin

Manuscripts, Articles, Book Chapters and Other Papers

Purpose: Ewing Sarcoma Family of Tumors (ESFT), the second most common pediatric osseous malignancy, are characterized by the pathognomonic chromosomal EWS-ETS translocation. Outside of tumor biopsy, no clinically relevant ESFT biomarkers exist. Additionally, tumor burden assessment at diagnosis, monitoring of disease responsiveness to therapy, and detection of disease recurrence are limited to radiographic imaging. To identify new, clinically relevant biomarkers we evaluated the proteome of a subset of ESFT-derived small extracellular vesicles (sEVs).

Materials and methods: We performed the first high quality proteomic study of ESFT-derived sEVs from 5 ESFT cell lines representing the most common EWS-ETS fusion types and …

Fosl1 Is A Novel Mediator Of Endotoxin/Lipopolysaccharide-Induced Pulmonary Angiogenic Signaling., Christopher R. Nitkin, Sheng Xia, Heather Menden, Wei Yu, Min Xiong, Daniel P. Heruth, Shui Qing Ye, Venkatesh Sampath

Manuscripts, Articles, Book Chapters and Other Papers

Systemic sepsis is a known risk factor for bronchopulmonary dysplasia (BPD) in premature infants, a disease characterized by dysregulated angiogenesis and impaired vascular and alveolar development. We have previoulsy reported that systemic endotoxin dysregulates pulmonary angiogenesis resulting in alveolar simplification mimicking BPD in neonatal mice, but the underlying mechanisms remain unclear. We undertook an unbiased discovery approach to identify novel signaling pathways programming sepsis-induced deviant lung angiogenesis. Pulmonary endothelial cells (EC) were isolated for RNA-Seq from newborn C57BL/6 mice treated with intraperitoneal lipopolysaccharide (LPS) to mimic systemic sepsis. LPS significantly differentially-regulated 269 genes after 6 h, and 1,934 genes after …

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From The International Kawasaki Disease Registry., Brian W. Mccrindle, Cedric Manlhiot, Jane W Newburger, Ashraf S. Harahsheh, Therese M. Giglia, Frederic Dallaire, Kevin Friedman, Tisiana Low, Kyle Runeckles, Mathew Mathew, Andrew S. Mackie, Nadine F. Choueiter, Pei-Ni Jone, Shelby Kutty, Anji T. Yetman, Geetha Raghuveer, Elfriede Pahl, Kambiz Norozi, Kimberly E Mchugh, Jennifer S. Li, Sarah D De Ferranti, Nagib Dahdah, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA Z score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA Z scores ≥10, …

Hearing Loss In Mucopolysaccharidoses: Current Knowledge And Future Directions., Jeremy Wolfberg, Keerthana Chintalapati, Shunji Tomatsu, Kyoko Nagao

Department of Pediatrics Faculty Papers

Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by a deficiency of one of the enzymes involved in the degradation of glycosaminoglycans. Hearing loss is a common clinical presentation in MPS. This paper reviews the literature on hearing loss for each of the seven recognized subtypes of MPS. Hearing loss was found to be common in MPS I, II, III, IVA, VI, and VII, and absent from MPS IVB and MPS IX. MPS VI presents primarily with conductive hearing loss, while the other subtypes (MPS I, MPS II, MPS III, MPS IVA, and MPS VII) can present with …

A Pilot Study Of The Preliminary Efficacy Of Pain Buddy: A Novel Intervention For The Management Of Children’S Cancer-Related Pain, John F. Hunter, Amanda M. Acevedo, Sergio Gago-Masague, Alexandra Kain, Christine Yun, Lilibeth Torno, Brooke N. Jenkins, Michelle A. Fortier

Psychology Faculty Articles and Research

Objectives

Cancer‐related pain in children is prevalent and undermanaged. Mobile health (mHealth) applications provide a promising avenue to address the gap in pain management in children with cancer. Pain Buddy is a multicomponent mHealth application developed to manage cancer‐related pain in children. The goal of this paper is to present preliminary efficacy data of the impact of Pain Buddy on children's pain severity and frequency.

Methods

In a randomized controlled trial over 60 days, children (N = 48) reported daily pain on a tablet while receiving usual care. Those in the intervention group (N = 20) received remote …

Pediatric Blunt Cerebrovascular Injury: Identifying Common Symptomatologies To Improve Screening Guidelines A Literature Review And Case Study, Natasha Kuklis, Dima Khalil, Sarah A. Jones Sapienza Md

Research Scholars Poster Presentation

No abstract provided.

Characterization Of Microbiology And Associated Antibiotic Usage In The Neonatal Intensive Care Unit, Bridget Lunney, Tibisay Villalobos Md, Faap, Kristin M. Held-Wheatley Pharmd, Bcop

Research Scholars Poster Presentation

No abstract provided.

Using Nsqip-Pediatrics To Target Quality Improvement Initiatives For Appendicitis, Erin Arevalo, Marybeth Browne Md

Research Scholars Poster Presentation

No abstract provided.

Atopic Dermatitis: Infected, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Atopic Dermatitis: Non-Infected, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Consensus Guidelines For Management Of Hyperammonaemia In Paediatric Patients Receiving Continuous Kidney Replacement Therapy., Rupesh Raina, Jirair K Bedoyan, Uta Lichter-Konecki, Philippe Jouvet, Stefano Picca, Nicholas Ah Mew, Marcel C Machado, Ronith Chakraborty, Meghana Vemuganti, Manpreet K Grewal, Timothy Bunchman, Sidharth Kumar Sethi, Vinod Krishnappa, Mignon Mcculloch, Khalid Alhasan, Arvind Bagga, Rajit K Basu, Franz Schaefer, Guido Filler, Bradley A Warady

Paediatrics Publications

Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the …

Guided Imagery And Deep Breathing: Minimizing Anxiety Experienced By Pediatric Cancer Patients, Bethany Grace Aney

Graduate Theses/Dissertations

This particular research study examined the impact of guided imagery and deep breathing as coping tools to reduce the anxiety levels experienced by pediatric cancer patients ages 6-12 years of age at a children’s hospital in Central Pennsylvania. This quantitative study utilized a pretest-posttest design, in addition to utilizing the State-Trait Anxiety Inventory for Children (STAIC™) as the baseline and post-test assessment. The STAIC™ is a questionnaire tool that assesses individuals’ state and trait anxiety through a total of 40 questions. This particular study had a total of five patients, two male and three female that consented to undergoing three …

The Use Of Animal-Assisted Therapeutic Interventions In The Hospital Setting During The Covid-19 Pandemic, Alicia Cesare

Graduate Theses/Dissertations

Handlers of therapy and/or facility dogs working within hospital settings have experienced various barriers and challenges within their practice during the COVID-19 pandemic. Animal-assisted interventions, specifically therapeutic working dogs, are a valued source of support to individuals, communities, hospital settings, and disaster sites during times of community distress. The purpose of this study was to identify barriers and solutions to the continued use of animal-assisted therapeutic interventions in support of patients and families within the hospital setting during the COVID-19 pandemic. This researcher’s position within the research is the knowledge and experience of being a facility dog handler within the …

Congenital Myasthenic Syndrome Caused By A Frameshift Insertion Mutation In, Szabolcs Szelinger, Jonida Krate, Keri Ramsey, Samuel P Strom, Perry B Shieh, Hane Lee, Newell Belnap, Chris Balak, Ashley L Siniard, Megan Russell, Ryan Richholt, Matt De Both, Ana M Claasen, Isabelle Schrauwen, Stanley F Nelson, Matthew J Huentelman, David W Craig, Samuel Yang, Steven A Moore, Kumaraswamy Sivakumar, Vinodh Narayanan, Sampathkumar Rangasamy, Ucla Clinical Genomics Center

Articles, Abstracts, and Reports

Objective: Description of a new variant of the glutamine-fructose-6-phosphate transaminase 1 (GFPT1) gene causing congenital myasthenic syndrome (CMS) in 3 children from 2 unrelated families.

Methods: Muscle biopsies, EMG, and whole-exome sequencing were performed.

Results: All 3 patients presented with congenital hypotonia, muscle weakness, respiratory insufficiency, head lag, areflexia, and gastrointestinal dysfunction. Genetic analysis identified a homozygous frameshift insertion in the GFPT1 gene (NM_001244710.1: c.686dupC; p.Arg230Ter) that was shared by all 3 patients. In one of the patients, inheritance of the variant was through uniparental disomy (UPD) with maternal origin. Repetitive nerve stimulation and single-fiber EMG was consistent …

Intussusception In An Infant As A Manifestation Of Covid-19, Zorays Moazzam, Areej Saleem, Alina Ashraf, Fyezah Jehan, Muhammad Arshad

Medical College Documents

Gastrointestinal manifestations of COVID-19 are rare and have primarily been limited to diarrhea or vomiting. Intussusception is the most common cause of bowel obstruction in infants, with up to 30% of pediatric intussusception cases having a preceding viral illness. We present the rare case of intussusception in a SARS-CoV-2 positive infant. This is the first documented case of survival in a SARS-CoV-2 positive patient presenting with intussusception as the primary manifestation. As our knowledge of this disease evolves, surgeons need to remain suspicious for possible gastrointestinal manifestations of COVID-19.

Respiratory Failure Due To A Large Mediastinal Mass In A 4-Year-Old Female With Blast Cell Crisis: A Case Report., Christian I Wade, Cody J Couperus-Mashewske, Mia E Geurts, Nicholas Derfler, John Ngo, Kyle S Couperus

Articles, Abstracts, and Reports

INTRODUCTION: Symptomatic leukostasis is an exceptionally atypical presentation of blast crisis; and when coupled with an enlarged neoplastic mediastinal mass in a four-year-old female, an extremely rare and challenging pediatric emergency arises.

CASE REPORT: We present a unique case of a four-year-old female who arrived via emergency medical services in cardiopulmonary arrest with clinical and radiographic evidence suggestive of bilateral pneumothoraces, prompting bilateral chest tube placement. Further evaluation revealed a large mediastinal mass and a concurrent white blood cell count of 428,400 per milliliter (/mL) (4,400-12,900/mL), with a 96% blast differential, consistent with complications of T-cell acute lymphoblastic leukemia.

CONCLUSION: …

Drivers Of Trust In Pediatric Health Care: Role Of Family-Centered Care, Alyssa Carlier

Research Scholars Poster Presentation

No abstract provided.