Medicine and Health Sciences Commons^™

Retrieval Of Large Balloon Fragments During Transcatheter Pulmonary Valve Implantation Using A Novel Retrieval System, Anne C Taylor, Mohamed Ali H Ghandour, Asra Khan, Srinath T Gowda, Flora Nunez-Gallegos, Lynn F Peng, Jamil A Aboulhosn, Daniel S Levi, Doff B Mcelhinney, Athar M Qureshi

Faculty, Staff and Students Publications

The removal of balloon fragments from the pulmonary artery without damaging the pulmonary and tricuspid valves can be difficult. Four cases during transcatheter pulmonary valve replacement are described in which a novel retrieval system was used to facilitate safe removal. (Level of Difficulty: Advanced.)

Knowledge, Attitude, And Perception Of The General Public Towards The Impact Of Hemoglobinopathies On Pregnancy Outcomes: A Questionnaire-Based Study, Kumud Dhankhar, Kiran Dahiya, Shobha Chikkavaddaragudi Ramachandra, Akila Prashant

International Journal of Health and Allied Sciences

Background: Knowledge about hemoglobinopathies in the general public is unsatisfactory. Many couples are unaware of their carrier status and a child can be born with unexpected, serious hemoglobinopathies. Willingness to participate in screening programs can substantially help in bringing down the birth of children affected with hemoglobinopathies. To understand the knowledge, attitude, and perception of the general public on various hemoglobinopathies and their pregnancy outcomes we performed a questionnaire-based descriptive survey. Methods: After obtaining approval from the institutional ethics committee and validation by an expert panel we circulated a Google form consisting of 21 questions via WhatsApp which was made …

Post-Acute Sequelae Of Covid-19 (Pasc) In Pediatrics: Factors That Impact Symptom Severity And Referral To Treatment., Catherine Soprano, Ryan Ngo, Casey A Konys, Ashley Bazier, Katherine Salamon

Department of Medicine Faculty Papers

The post-acute sequelae of COVID-19 (PASC) is a complex condition. While there are emerging studies on its effects in adults, there is scarce research regarding the long-term effects of COVID-19 infection among youth. Several researchers have likened long-haul COVID-19 to chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) and postural orthostatic tachycardia syndrome (POTS). In adults, the prognosis for these diagnoses is less promising than that in youth; however, there is currently very little information available on the presentation of youth with PASC. A better understanding of the specific symptom presentation for youth diagnosed with PASC is necessary. Retrospective chart reviews were conducted …

Antiphosphatidylserine/Prothrombin Antibodies In Childhood Sle Are Associated With Lupus Anticoagulant Positivity, S. Chugh, R. Pilania, A. Kler, C. Hans, Narender Kumar, S. Singh, J. Ahluwalia

School of Medicine Faculty Publications

ISTH 2023 Congress, June 24 - 28, 2023, Montreal, Canada

Effects Of Covid-19 Vaccination On Pro-Coagulant State Of A Protein S-Deficient Pediatric Patient: New Hope For Treatment Of The Deficiency, Diana Polania-Villanueva, M. Kumar, Lekha Thangada, Jone Garai, Jovanny Zabaleta, Rinku Majumder

School of Medicine Faculty Publications

ISTH 2023 Congress, June 24 - 28, 2023, Montreal, Canada

Mucopolysaccharidosis Iva: Current Disease Models And Drawbacks, Andrés Felipe Leal, Carlos Javier Alméciga-Díaz, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Mucopolysaccharidosis IVA (MPS IVA) is a rare disorder caused by mutations in the N-acetylgalactosamine-6-sulfate-sulfatase (GALNS) encoding gene. GALNS leads to the lysosomal degradation of the glycosaminoglyccreasans keratan sulfate and chondroitin 6-sulfate. Impaired GALNS enzymes result in skeletal and non-skeletal complications in patients. For years, the MPS IVA pathogenesis and the assessment of promising drugs have been evaluated using in vitro (primarily fibroblasts) and in vivo (mainly mouse) models. Even though value information has been raised from those studies, these models have several limitations. For instance, chondrocytes have been well recognized as primary cells affected in MPS IVA and responsible for …

Left Juxtaposition Of The Right Atrial Appendage: Pitfalls In Diagnosis, Kanya Singhapakdi, Wesam Sourour, Thomas Kimball

School of Medicine Faculty Publications

Several congenital anomalies of the right atrial appendage (RAA) have been described including aneurysm, herniation (in association with a pericardial defect), and left juxtaposition. The left juxtaposition of the RAA (LJRAA), first described by Birmingham in 1893 and subsequently introduced by Dixon in 1954, is usually associated with complex cardiac malformations such as obstruction of the left ventricular outflow tract. In this case report, we will describe an unusual variant of LJRAA in the absence of any other cardiac defects, which was initially misinterpreted as an aortic dissection. The correct diagnosis was made after careful reinterpretation and the use of …

Late-Gadolinium Enhancement Is Common In Older Pediatric Heart Transplant Recipients And Is Associated With Lower Ejection Fraction, Andrew A Lawson, Kae Watanabe, Lindsay Griffin, Christina Laternser, Michael Markl, Cynthia K Rigsby, Melanie Sojka, Joshua D Robinson, Nazia Husain

Faculty, Staff and Students Publications

BACKGROUND: Chronic graft failure and cumulative rejection history in pediatric heart transplant recipients (PHTR) are associated with myocardial fibrosis on endomyocardial biopsy (EMB). Cardiovascular magnetic resonance imaging (CMR) is a validated, non-invasive method to detect myocardial fibrosis via the presence of late gadolinium enhancement (LGE). In adult heart transplant recipients, LGE is associated with increased risk of future adverse clinical events including hospitalization and death. We describe the prevalence, pattern, and extent of LGE on CMR in a cohort of PHTR and its associations with recipient and graft characteristics.

METHODS: This was a retrospective study of consecutive PHTR who underwent …

See Me, Hear Me, Know Me: Perspectives On Diet And Physical Activity Influences Among Teens Living In Rural Texas Communities, Debbe Thompson, Julie Miranda, Chishinga Callender, Jayna M Dave, Godlove Appiah, Salma M A Musaad

Faculty, Staff and Students Publications

Teens in rural communities are at greater risk of obesity than teens in urban areas. Diet and physical activity influence obesity risk. Understanding their perspectives is an important step in intervention design. This qualitative investigation explored teen perspectives on how living in a rural community influenced their diet and physical activity choices. Forty parent-teen pairs were recruited. Data collection included surveys and telephone interviews. This paper reports teen perspectives identified in the first interview. Thematic analysis was used to code and analyze the data. Findings revealed that the primary factor driving teens' diet and physical activity behaviors was the teens …

Clinical Factors Associated With Need For Neurosurgical Care In Young Children With Imaging For Macrocephaly: A Case Control Study, Jessica F. Rohde, Jeffrey Campbell, Julie Barbera, Elena Taylor, Ashok Ramachandra, Christopher Gegg, Andrea Scherer, Joseph Piatt

Department of Pediatrics Faculty Papers

BACKGROUND: Macrocephaly is present in 2.3% of children with important neurosurgical conditions in the differential diagnosis. The objective of this study was to identify clinical associations with actionable imaging findings among children with head imaging for macrocephaly.

METHODS: We conducted a case-control study of head imaging studies ordered for macrocephaly among children 24 months and younger in a multistate children's health system. Four neurosurgeons reviewed the images, determining cases to be a 'concern' if neurosurgical follow-up or intervention was indicated. Electronic health records were reviewed to collect patient-level data and to determine if surgery was performed. Controls were matched 3:1 …

Neglected Tropical Disease Vaccines: Hookworm, Leishmaniasis, And Schistosomiasis, Peter J Hotez, Maria Elena Bottazzi, Paul M Kaye, Bruce Y Lee, Karl Philipp Puchner

Faculty, Staff and Students Publications

No abstract provided.

Tuberculosis Screening In Migrants To The Eu/Eea And Uk, Niklas Köhler, Anca Vasiliu, Anna Maria Mandalakas, Leonardo Martinez, Christoph Lange

Faculty, Staff and Students Publications

No abstract provided.

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

Posters

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

Posters

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

Posters

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 …

Anticoagulation Therapies And Vte Risk Assessment, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.

Hyperleukocytosis/Leukapheresis, Children's Mercy Kansas City

Clinical Pathways

Hyperleukocytosis is a life-threatening oncology emergency, defined as a white blood cell (WBC) count ≥ 100,000/µL and can occur in up to 20% of patients with acute myeloid or lymphoid leukemias (Abla et al., 2016). Hyperleukocytosis may lead to leukostasis and subsequent end organ damage, commonly affecting the central nervous system, respiratory and renal systems, and can also cause tumor lysis syndrome (TLS), another oncology emergency, and lead to metabolic derangements and further renal dysfunction (Porcu et al., 2000). Treatment of hyperleukocytosis includes supportive care, early initiation of chemotherapy and/or leukapheresis (Zhang et al., 2021). There are no definitive guidelines …

Birth Brachial Plexus Injury (Bbpi): Management Of Shoulder Subluxation/Dislocation, Children's Mercy Kansas City

Clinical Pathways

BBPI most often, but not always, occurs during the delivery process as the infant descends through the birth canal and the shoulder becomes lodged on the mother’s pelvic bone. As contraction begins, the infant’s neck and shoulder are stretched which can cause tears or ruptures of the nerves within the brachial plexus thus impacting the sensation and movement of the affected upper extremity. Incidence of brachial plexus injuries in the United States is 0.4 to 4 in 1,000 live births (Abzug, et al., 2018; Foad et al., 2008; Hoeksma et al., 2004; Waters, 1997). Although many of these infants will …

Access To Pediatric Neurology Training And Services Worldwide:A Survey By The International Child Neurology Association, Jo M Wilmshurst, Coriene Catsman- Berrevoets, Donald L. Gilbert, Lakshmi Nagarajan, Pauline Samia, Esra Serdaroglu Serdaroglu, Chahnez Triki, Jorge Vidaurre, Biju Hameed

Paediatrics and Child Health, East Africa

Pediatric neurology is the medical subspecialty responsible for diagnosing and managing diseases and disorders of the nervous system in childhood and adolescence. In many, but not all, regions of the world, the discipline of pediatric neurology is recognized as a specialty or subspecialty of either neurology or pediatrics. Significant knowledge and competencies in this area are necessary to be effective in clinical practice. The need for this is driven by the high burden of disease from neurologic conditions in children and the effect on their families. As the first part of a multistaged project under the auspices of the International …

World Allergy Organization (Wao) Diagnosis And Rationale For Action Against Cow's Milk Allergy (Dracma) Guidelines Update – X – Breastfeeding A Baby With Cow's Milk Allergy, Vicki Mcwilliam, Merryn Netting, Evelyn Volders, Debra Palmer, Ignacio Ansotegui, Stefania Arasi, Amal H. Assa'ad, Sami L. Bahna, Antonio Bognanni, Rose Kamenwa

Paediatrics and Child Health, East Africa

Cow’s milk allergy is rare in exclusively breastfed infants. To support the continuation of breastfeeding an infant after diagnosis with a cow’s milk allergy, it is critical to examine the evidence for and against any form of cow’s milk elimination diet for lactating mothers. In this narrative review, we highlight the lack of high-quality evidence, hence subsequent controversy, regarding whether the minuscule quantities of cow’s milk proteins detectable in human milk cause infant cow’s milk allergy symptoms. Current clinical practice recommendations advise a 2–4 week trial of maternal cow’s milk dietary elimination for: a) IgE-mediated cow’s milk allergy only if …

Multimodal Analysis Of Secondary Cerebellar Alterations After Pediatric Traumatic Brain Injury, Finian Keleher, Hannah M Lindsey, Rebecca Kerestes, Houshang Amiri, Robert F Asarnow, Talin Babikian, Brenda Bartnik-Olson, Erin D Bigler, Karen Caeyenberghs, Carrie Esopenko, Linda Ewing-Cobbs, Christopher C Giza, Naomi J Goodrich-Hunsaker, Cooper B Hodges, Kristen R Hoskinson, Andrei Irimia, Marsh Königs, Jeffrey E Max, Mary R Newsome, Alexander Olsen, Nicholas P Ryan, Adam T Schmidt, Dan J Stein, Stacy J Suskauer, Ashley L Ware, Anne L Wheeler, Brandon A Zielinski, Paul M Thompson, Ian H Harding, David F Tate, Elisabeth A Wilde, Emily L Dennis

Faculty, Staff and Student Publications

Importance: Traumatic brain injury (TBI) is known to cause widespread neural disruption in the cerebrum. However, less is known about the association of TBI with cerebellar structure and how such changes may alter executive functioning.

Objective: To investigate alterations in subregional cerebellum volume and cerebral white matter microstructure after pediatric TBI and examine subsequent changes in executive function.

Design, setting, and participants: This retrospective cohort study combined 12 data sets (collected between 2006 and 2020) from 9 sites in the Enhancing Neuroimaging Genetics Through Meta-Analysis Consortium Pediatric TBI working group in a mega-analysis of cerebellar structure. Participants with TBI or …

Blood Culture Versus Antibiotic Use For Neonatal Inpatients In 61 Hospitals Implementing With The Nest360 Alliance In Kenya, Malawi, Nigeria, And Tanzania: A Cross-Sectional Study, Sarah Murless-Collins, Kondwani Kawaza, Nahya Salim, Elizabeth Molyneux, Msandeni Chiume, Jalemba Aluvaala Aluvaala, William Macharia, Veronica Chinyere Ezeaka, Opeyemi Odedere, Donat Shamba

Paediatrics and Child Health, East Africa

Background: Thirty million small and sick newborns worldwide require inpatient care each year. Many receive antibiotics for clinically diagnosed infections without blood cultures, the current ‘gold standard’ for neonatal infection detection. Low neonatal blood culture use hampers appropriate antibiotic use, fuelling antimicrobial resistance (AMR) which threatens newborn survival. This study analysed the gap between blood culture use and antibiotic prescribing in hospitals implementing with Newborn Essential Solutions and Technologies (NEST360) in Kenya, Malawi, Nigeria, and Tanzania.

Methods: Inpatient data from every newborn admission record (July 2019–August 2022) were included to describe hospital-level blood culture use and antibiotic prescription. Health Facility …

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

Posters

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, …

Early Skeletal Muscle Loss In Adolescent And Young Adult Cancer Patients Treated With Anthracycline Chemotherapy, Savannah V Wooten, Fei Wang, Michael E Roth, Guanshu Liu, J Andrew Livingston, Behrang Amini, Susan C Gilchrist, Michelle Hildebrandt, Eugenie S Kleinerman

Faculty, Staff and Student Publications

BACKGROUND: Early skeletal muscle loss has been observed in adolescent and young adult (AYA) sarcoma patients undergoing treatment. Identification of individuals within the AYA populace that are at greatest risk of anthracycline-induced skeletal muscle loss is unknown. Moreover, investigations which seek out underlying causes of skeletal muscle degradation during chemotherapy are critical for understanding, preventing, and reducing chronic health conditions associated with poor skeletal muscle status.

METHODS: Computed tomography (CT) scans were used to investigate changes in skeletal muscle of 153 AYA sarcoma and Hodgkin lymphoma patients at thoracic vertebra 4 after anthracycline treatment. Images were examined at three time …

Effect Of The Covid-19 Pandemic On The Epidemiology Of Pediatric Traumatic Brain Injury In Brazil, Leopoldo Mandic Ferreira Furtado, José Aloysio Da Costa Val Filho, Victor Da Silva Pereira, Yasmin S Coimbra, Vitor Hugo R Caldas, Anne R Magalhães, Bruna Athayde S De Carvalho, Saulo G Moreira, Antônio L Teixeira, Aline Silva De Miranda

Faculty, Staff and Student Publications

Aim: In response to the coronavirus 2019 disease (COVID-19) pandemic, governments worldwide implemented measures to prevent infection, resulting in restricted school activities, restricted children’s freedom of movement, and increased risk of violence and injuries at home, including traumatic brain injury (TBI), among children. In Brazil, the consequences of the COVID-19 pandemic on the causes, severity, and mortality of pediatric TBI have not yet been investigated. Thus, our study aimed to determine whether the COVID-19 pandemic has affected the epidemiology of pediatric TBI among Brazilian children.

Materials and methods: We investigated the patients with TBI aged <18 years who visited a tertiary trauma center in Brazil in 2019 and 2020. TBI-related variables, such as classification, mechanism, clinical manifestations, need for intervention, morbidity, and mortality, were recorded. Furthermore, we used a nationwide databank to collect information on mortality from external causes of trauma and violence in the pediatric population in 2019 and 2020. The Mann-Whitney test was used to compare quantitative variables related to the mechanisms and severity of TBI in both periods in order to determine the impact of the COVID-19 pandemic.

Results: Of the patients with …

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

Posters

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

Posters

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

Posters

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 …

Building Up A Genomic Surveillance Platform For Sars-Cov-2 In The Middle Of A Pandemic: A True North-South Collaboration, Waqasuddin Khan, Furqan Kabir, Samiah Kanwar, Fatima Aziz, Sahrish Muneer, Adil Kalam, Mehdia Ali, Nadia Ansari, Fyezah Jehan, Muhammad Imran Nisar

Department of Paediatrics and Child Health

Next-generation sequencing technology has revolutionised pathogen surveillance over the last two decades. However, the benefits are not equitably distributed, with developing countries lagging far behind in acquiring the required technology and analytical capacity. Recent declines in the cost associated with sequencing-equipment and running consumables have created an opportunity for broader adoption. During the COVID-19 pandemic, rapid diagnostics development and DNA sequencing revolutionised the ability to diagnose and sequence SARS-CoV-2 rapidly. Socioeconomic inequalities substantially impact the ability to sequence SARS-CoV-2 strains and undermine a developing country's pandemic preparedness. Low- and middle-income countries face additional challenges in establishing, maintaining and expanding genomic …

Can Machine Learning Methods Be Used For Identification Of At-Risk Neonates In Low-Resource Settings? A Prospective Cohort Study, Babar S. Hasan, Zahra Hoodbhoy, Amna Khan, Mariana Nogueira, Bart Bijnens, Devyani Chowdhury

Department of Paediatrics and Child Health

Introduction: Timely identification of at-risk neonates (ARNs) in the community is essential to reduce mortality in low-resource settings. Tools such as American Academy of Pediatrics pulse oximetry (POx) and WHO Young Infants Clinical Signs (WHOS) have high specificity but low sensitivity to identify ARNs. Our aim was assessing the value of POx and WHOS independently, in combination and with machine learning (ML) from clinical features, to detect ARNs in a low/middle-income country.
Methods: This prospective cohort study was conducted in a periurban community in Pakistan. Eligible live births were screened using WHOS and POx along with clinical information regarding pregnancy …