Medicine and Health Sciences Commons^™

Bioactive Metabolites Of Omega-6 And Omega-3 Fatty Acids Are Associated With Inflammatory Cytokine Concentrations In Maternal And Infant Plasma At The Time Of Delivery, Rebecca Slotkowski, Matthew Van Ormer, Anum Akbar, Taija Hahka, Maranda Thompson, Rebekah Rapoza, Arzu Ulu, Melissa Thoene, Elizabeth Lyden, Maheswari Mukherjee, Ana G. Yuil-Valdes, Sathish Kumar Natarajan, Tara Nordgren, Corrine K. Hanson, Ann Anderson-Berry

Journal Articles: Pediatrics

Background & aims

Inflammation is necessary for a healthy pregnancy. However, unregulated or excessive inflammation during pregnancy is associated with severe maternal and infant morbidities, such as pre-eclampsia, abnormal infant neurodevelopment, or preterm birth. Inflammation is regulated in part by the bioactive metabolites of omega-6 (n-6) and omega-3 (n-3) fatty acids (FAs). N-6 FAs have been shown to promote pro-inflammatory cytokine environments in adults, while n-3 FAs have been shown to contribute to the resolution of inflammation; however, how these metabolites affect maternal and infant inflammation is still uncertain. The objective of this study was to predict the influence of …

The Impact Of Iron Supplementation On The Preterm Neonatal Gut Microbiome: A Pilot Study, Matthew Van Ormer, Maranda Thompson, Melissa Thoene, Jean-Jack Riethoven, Sathish Kumar Natarajan, Corrine K. Hanson, Ann Anderson-Berry

Journal Articles: Pediatrics

OBJECTIVE: The gastrointestinal microbiome in preterm infants exhibits significant influence on optimal outcomes-with dysbiosis shown to substantially increase the risk of the life-threatening necrotizing enterocolitis. Iron is a vital nutrient especially during the perinatal window of rapid hemoglobin production, tissue growth, and foundational neurodevelopment. However, excess colonic iron exhibits potent oxidation capacity and alters the gut microbiome-potentially facilitating the proliferation of pathological bacterial strains. Breastfed preterm infants routinely receive iron supplementation starting 14 days after delivery and are highly vulnerable to morbidities associated with gastrointestinal dysbiosis. Therefore, we set out to determine if routine iron supplementation alters the preterm gut …

Evaluating The Impact Of Routine Enteral Iron Fortification On Gastrointestinal Dysbiosis In Premature Infants, Matthew Van Ormer

Theses & Dissertations

The gastrointestinal microbiome in preterm infants bears significant influence on optimal outcomes – dysbiosis is shown to substantially increase the risk of the life-threatening necrotizing enterocolitis, whereas optimal microbiome composition improves nutrient digestion and immune function. Iron is a vital nutrient especially during the perinatal window of rapid hemoglobin production, tissue growth, and foundational neurological development. However, excess colonic iron exhibits potent oxidation capacity and alters the competitive environment of microbes – potentially facilitating the proliferation of pathological bacterial strains in the gut. Of note, breastfed preterm infants routinely receive iron fortification starting at 14 days of life and are …

Evaluation Of Vitamin E Isoforms In Placental Tissue And Their Relationship With Maternal Dietary Intake And Plasma Concentrations In Mother-Infant Dyads, Ishani Jhamb, Alyssa Freeman, Michelle R. Lotfi, Matthew Van Ormer, Corrine K. Hanson, Ann Anderson-Berry, Melissa K. Thoene

Journal Articles: Pediatrics

α-tocopherol is a vitamin E isoform with potent antioxidant activity, while the γ-tocopherol isoform of vitamin E exerts more pro-inflammatory effects. In maternal-fetal environments, increased plasma α-tocopherol concentrations are associated with positive birth outcomes, while higher γ-tocopherol concentrations are linked with negative pregnancy outcomes. However, little is known about tocopherol concentrations in placental tissue and their role in modulating placental oxidative stress, a process that is implicated in many complications of pregnancy. The objectives of this research are to evaluate the concentrations of α- and γ-tocopherol in placental tissue and assess relationships with maternal and umbilical cord plasma concentrations. A …

Something Smells Fishy: How Lipid Mediators Impact The Maternal-Fetal Interface And Neonatal Development, Maranda Thompson, Arzu Ulu, Maheswari Mukherjee, Ana G. Yuil-Valdes, Melissa K. Thoene, Matthew Van Ormer, Rebecca Slotkowski, Teri Mauch, Ann Anderson-Berry, Corrine K. Hanson, Tara M. Nordgren, Sathish Kumar Natarajan

Journal Articles: Pediatrics

Normal pregnancy relies on inflammation for implantation, placentation, and parturition, but uncontrolled inflammation can lead to poor maternal and infant outcomes. Maternal diet is one modifiable factor that can impact inflammation. Omega-3 and -6 fatty acids obtained through the diet are metabolized into bioactive compounds that effect inflammation. Recent evidence has shown that the downstream products of omega-3 and -6 fatty acids may influence physiology during pregnancy. In this review, the current knowledge relating to omega-3 and omega-6 metabolites during pregnancy will be summarized.

Ventricular Tachycardia In A Pediatric Patient With High-Risk Thrombotic Thrombocytopenia Purpura, Taylor J. Kratochvil, Jeffrey A. Robinson

Journal Articles: Pediatrics

An 8-year-old previously healthy male was diagnosed with thrombotic thrombocytopenic purpura (TTP) and increased serum cardiac troponin I. Telemetry recorded non-sustained ventricular tachycardia, without ST-segment changes or other abnormalities on serial electrocardiogram. This case illustrates that cardiac monitoring by telemetry should be considered in high-risk TTP with elevated cardiac troponin.

Retinol And Pro-Vitamin A Carotenoid Nutritional Status During Pregnancy Is Associated With Newborn Hearing Screen Results, Rebecca Slotkowski, Matthew Van Ormer, Anum Akbar, Olivia Paetz, Taija Hahka, Maranda Thompson, Alyssa Freeman, Alexandra Hergenrader, Sarah Sweeney, Zeljka Korade, Thiago Genaro-Mattos, Corrine K. Hanson, Ann Anderson-Berry, Melissa K. Thoene

Journal Articles: Pediatrics

The prenatal period is critical for auditory development; thus, prenatal influences on auditory development may significantly impact long-term hearing ability. While previous studies identified a protective effect of carotenoids on adult hearing, the impact of these nutrients on hearing outcomes in neonates is not well understood. The purpose of this study is to investigate the relationship between maternal and umbilical cord plasma retinol and carotenoid concentrations and abnormal newborn hearing screen (NHS) results. Mother-infant dyads (n = 546) were enrolled at delivery. Plasma samples were analyzed using HPLC and LC-MS/MS. NHS results were obtained from medical records. Statistical analysis …

Plasma Retinol Concentrations And Dietary Intakes Of Mother-Infant Sets In Singleton Versus Twin Pregnancy, Anum Akbar, Sarah Duvall, Matthew Van Ormer, Rebecca Slotkowski, Taija Hahka, Thiago Genaro-Mattos, Zeljka Korade, Corrine K. Hanson, Ann Anderson-Berry, Melissa K. Thoene

Journal Articles: Pediatrics

Vitamin A (retinol) is essential for normal fetal development, but the recommendation for maternal dietary intake (Retinol Activity Equivalent, RAE) does not differ for singleton vs. twin pregnancy, despite the limited evaluation of retinol status. Therefore, this study aimed to evaluate plasma retinol concentrations and deficiency status in mother-infant sets from singleton vs. twin pregnancies as well as maternal RAE intake. A total of 21 mother-infant sets were included (14 singleton, 7 twin). The HPLC and LC-MS/HS evaluated the plasma retinol concentration, and data were analyzed using the Mann-Whitney U test. Plasma retinol was significantly lower in twin vs. singleton …

Omega-3 Polyunsaturated Fatty Acid Levels In Maternal And Cord Plasma Are Associated With Maternal Socioeconomic Status, Alexandra Hergenrader, Matthew Van Ormer, Rebecca Slotkowski, Maranda Thompson, Alyssa Freeman, Olivia Paetz, Sarah Sweeney, Lauren Wegner, Khadijjta Ali, Nicole S. Bender, Ridhi Chaudhary, Melissa K. Thoene, Corrine K. Hanson, Ann Anderson-Berry

Journal Articles: Pediatrics

Omega-3 (n-3) polyunsaturated fatty acids (PUFAs) play a crucial role in fetal growth and neurodevelopment, while omega-6 (n-6) PUFAs have been associated with adverse pregnancy outcomes. Previous studies have demonstrated that socioeconomic status (SES) influences dietary intake of n-3 and n-6 PUFAs, but few studies have evaluated the association between maternal and cord plasma biomarkers of PUFAs and socioeconomic markers. An IRB-approved study enrolled mother-infant pairs (n = 55) at the time of delivery. Maternal and cord plasma PUFA concentrations were analyzed using gas chromatography. Markers of SES were obtained from validated surveys …

Testicular Torsion Education: Improving Awareness Among Boys And Their Caregivers Regarding The Urgency Of Evaluating Scrotal Pain, Abby Taylor

Capstone Experience

Introduction: Testicular torsion is a urologic emergency that requires prompt medical evaluation and surgical intervention. The length of time from onset of the torsion to evaluation in the emergency department is often delayed due to lack of knowledge of the urgency of the situation amongst boys and their caregivers. We hypothesize that an animated educational video will increase awareness of the condition and prompt urgent evaluation in the emergency department.

Methods: A 5-minute animated educational video was created following a boy, Danny, as he develops testicular torsion. Four population groups: boys, parents, school nurses, and pediatricians, completed group-specific pre- and …

Nutrition Support Practices For Infants Born <750 Grams Or <25 Weeks Gestation: A Call For More Research, Melissa K. Thoene, Ann Anderson-Berry

Journal Articles: Pediatrics

With advances in medical care and efforts to care for continually smaller and younger preterm infants, the gestational age of viability has decreased, including as young as 21 or 22 weeks of gestation [...].

Postoperative Breath-Holding Spells Requiring Cardiopulmonary Resuscitation After Cardiopulmonary Bypass, Rebecca Kreis, Jeffrey A. Robinson, Ali N. Ibrahimiye, Laura Ortmann

Journal Articles: Pediatrics

Breath-holding spells are common in childhood and can be associated with bradycardia and pulselessness. This report details severe breath-holding spells complicating postoperative management after atrial septal defect closure. The patient required cardiopulmonary resuscitation despite the use of a temporary pacemaker to prevent bradycardia. After multiple episodes of chest compressions, the decision was made to not intervene immediately to pulselessness and the patient was able to recover without further intervention.

Omega-6 And Omega-3 Fatty Acid-Derived Oxylipins From The Lipoxygenase Pathway In Maternal And Umbilical Cord Plasma At Delivery And Their Relationship With Infant Growth, Maranda Thompson, Arzu Ulu, Ana G. Yuil-Valdes, Maheswari Mukherjee, Melissa K. Thoene, Matthew Van Ormer, Rebecca Slotkowski, Elizabeth Lyden, Ann Anderson-Berry, Corrine K. Hanson, Tara M. Nordgren, Sathish Kumar Natarajan

Journal Articles: Pediatrics

Omega-3 and omega-6 fatty acids are important for neonatal development and health. One mechanism by which omega-3 and omega-6 fatty acids exert their effects is through their metabolism into oxylipins and specialized pro-resolving mediators. However, the influence of oxylipins on fetal growth is not well understood. Therefore, the objective of this study was to identify oxylipins present in maternal and umbilical cord plasma and investigate their relationship with infant growth. Liquid chromatography-tandem mass spectrometry was used to quantify oxylipin levels in plasma collected at the time of delivery. Spearman's correlations highlighted significant correlations between metabolite levels and infant growth. They …

Post-Transplant Hepatic Graft Fibrosis In Pediatric Liver-Inclusive Transplant Recipients, Hanh D. Vo

Theses & Dissertations

Hepatic graft fibrosis is a common histologic finding following pediatric liver transplant (LT) that may affect the long-term graft outcome. Hence, it is essential to identify hepatic graft fibrosis at a stage where fibrosis is not yet clinically apparent but has the potential for progression to initiate appropriate intervention and prevent its progression. Liver biopsy is the gold standard for fibrosis staging, but it is invasive, requires general anesthesia in children, and has potential complications. Our long-term goal is to implement non-invasive tests to assess and monitor the progression of hepatic graft fibrosis in liver-inclusive transplant children. The work presented …

Glad To Be Out. I Was Running Out Of Womb: A Study Of Omega-6 And Omega-3 Fatty Acid Metabolites At Delivery And Their Relationship To Infant Growth, Prematurity, And Admission To The Neonatal Intensive Care Unit, Maranda Thompson

Theses & Dissertations

Normal pregnancy relies on inflammation for implantation, placentation, and parturition, but uncontrolled it can lead to poor infant outcomes. Maternal diet is one modifiable factor that can impact inflammation. Omega-3 and omega-6 fatty acids have been shown to be metabolized into bioactive metabolites that effect inflammation. The presence and importance of these metabolites at the time of delivery are not well understood. Therefore, the purpose of this dissertation was to first quantify omega-3 and omega-6 metabolites in maternal plasma, cord plasma, and placental tissue. Then the relationships between metabolites and infant outcomes, including birth weight percentile, length percentile, head circumferences …

The Ecology Of Mental Health And The Impact Of Barriers On Mental Health Service Utilization, Alisha Aggarwal

Theses & Dissertations

Mental health has emerged as a major public health concern in recent times with several disparities and barriers related to adequate health service utilization. Different age groups experience diverse symptoms and face distinct barriers in accessing and utilizing healthcare. Policies like Mental Health Parity and Addiction Equity Act and the Patient Protection and Affordable Care Act were launched to improve insurance coverage and delivery of mental health services. However, a large gap of unmet needs exists for adequate mental health service utilization in the country. This dissertation aims to describe the financial and non-financial barriers to mental health service utilization …

Derivation And Validation Of A General Predictive Model For Long Term Risks For Mortality And Invasive Cardiovascular Interventions In Congenital Heart Disease, David A. Danford

Capstone Experience

Introduction. Accurate assessment of prognosis is a key driver of clinical decision making in congenital heart disease (CHD), but is complicated because CHD represents such a diverse collection of conditions. The aim of this investigation is to derive, validate, and calibrate multivariable predictive models for time to surgical or catheter-mediated intervention (INT) in CHD and for time to death in CHD. Methods. 4108 unique subjects were prospectively and consecutively enrolled, and randomized to derivation and validation cohorts. Total follow up was 26,578 patient-years, with 102 deaths and 868 INTs. Accelerated failure time multivariable predictive models for the outcomes, based on …

Proof Of Concept: The Use Of Renal Biomarkers In Critically Ill Pediatric Patients For Therapeutic Drug Monitoring, Christopher Lee Shaffer

Theses & Dissertations

Acute kidney injury (AKI) is a serious and common complication in critically ill pediatric patients. The incidence of pediatric AKI continues to increase, especially in patients who undergo surgical correction of congenital heart defects. Serum creatinine and urine output are the most commonly used tools to assess renal function, with international guidelines standardizing AKI-definitions based upon these parameters. However, changes in serum creatinine can occur 24 hours or later after a renal insult event, delaying the diagnosis and potential interventions to reverse injury. It is critical to identify endogenous renal biomarkers within the pediatric population that are both timely and …

Association Of New And/Or Progressive Multiple Organ Dysfunction Syndrome With Mortality And New Morbidity Among Children Encountering Septic Shock, Rachel L. Waworuntu

Capstone Experience

Despite reductions in sepsis mortality, there remains a substantial morbidity burden among children who survive sepsis. Furthermore, the majority develop multiple organ ‘group has historically been analyzed as a single cohort. Using the Life After Pediatric Sepsis Evaluation (LAPSE) investigation database, we studied the association of new and progressive multiple organ dysfunction syndrome (NPMODS) with morbidity and mortality among children with septic shock. We hypothesized that NPMODS represents a distinct phenotype based on evolving organ dysfunction. There was evidence of an association but it did not reach the 0.05 level of significance. We also hypothesized and confirmed that children with …

Capacity Building For A New Multicenter Network Within The Echo Idea States Pediatric Clinical Trials Network, Robert D. Annett, Scott Bickel, John C. Carlson, Kelly Cowan, Sara Cox, Mark J. Fisher, J. Dean Jarvis, Alberta S. Kong, Jessica S. Kosut, Kurtis R. Kulbeth, Abbot Laptook, Pearl A. Mcelfish, Mary M. Mcnally, Lee M. Pachter, Barbara A. Pahud, Lee A. Pyles, Jennifer Shaw, Kari Simonsen, Jessica Snowden, Christine B. Turley, Andrew M. Atz

Journal Articles: Pediatrics

Introduction: Research capacity building is a critical component of professional development for pediatrician scientists, yet this process has been elusive in the literature. The ECHO IDeA States Pediatric Clinical Trials Network (ISPCTN) seeks to implement pediatric trials across medically underserved and rural populations. A key component of achieving this objective is building pediatric research capacity, including enhancement of infrastructure and faculty development. This article presents findings from a site assessment inventory completed during the initial year of the ISPCTN. Methods: An assessment inventory was developed for surveying ISPCTN sites. The inventory captured site-level activities designed to increase clinical trial research …

Reach, Effectiveness, Adoption, And Maintenance Of Mobile Electronic Clinical Decision Support Tools Deployed As Part Of National Quality Improvement Projects, Ellen K. Kerns

Theses & Dissertations

Electronic clinical decision support (ECDS) tools are often developed within quality improvement (QI) projects to increase adherence with the latest clinical practice guidelines. However, the potential reach and maintenance of ECDS use beyond the time and location of their associated project are very limited. Deploying ECDS using a mobile app (mECDS) has shown the potential to be a viable method of overcoming these limitations. However, it is unclear what pattern the reach and adoption of such a tool might follow and what effect this use has on clinical practice. Our team developed an app which contained two different mECDS tools …

Early Systolic Dysfunction And Impact Of Gene Mutation Severity In Marfan Syndrome, Aaron Clem, Lois J. Starr, David Danford, Mary Craft, Ling Li, Jason Christensen, Anji Yetman

Child Health Research Institute Pediatric Research Forum

Background

Marfan syndrome is caused by a mutation in the fibrillin-1 gene that manifests with a variety of features including aortic root dilation. Recent research has identified a primary cardiomyopathy in patients with Marfan syndrome, hypothesized to be due to the presence of abnormal fibrillin-1 in the myocardium. Controversy over the nature and significance of this cardiomyopathy remains. Echocardiographic measurement of the first-phase of ejection (defined as the beginning of systole to peak aortic valve flow) may be more sensitive to systolic dysfunction and provide useful clinical information. The purpose of this study is to:

1. Assess systolic dysfunction in patients …

Do I Add Value? A Mixed Methods Study Comparing Medical Students’ Experiences With Patient Documentation, Shannon Haines, Kari Nelson, Kaeli Samson, Nathan Gollehon

Child Health Research Institute Pediatric Research Forum

Background

Clinical documentation is an essential skill for practicing physicians, yet many medical students are not prepared for the documentation demands required during residency.^1,2 Recent CMS rule changes now allow medical students’ notes to be used for billing.³ Previous studies have shown positive perceptions of medical students whose notes were used in an outpatient setting.⁴ We utilized Constructivist Learning Theory to frame our work, where students construct their knowledge as faculty and residents facilitate the process.⁵

Objectives

To explore if using student documentation for billing impacts student perceptions and experiences during the pediatric clerkship.

Methods

Pediatric …

Novel Mouse Model For Analysis Of Macrophage Function In Neuroblastoma, Gabrielle L. Brumfield, Shelby M. Knoche, Alaina C. Larson, Brittany Poelaert, Benjamin T. Goetz, Poomy Pandey, Don W. Coulter, Joyce C. Solheim

Child Health Research Institute Pediatric Research Forum

Background: Neuroblastoma is the third most common childhood cancer and accounts for 12% of cancer-associated deaths in children under the age of 15. Patients with high risk neuroblastoma have a poor 5-year survival rate of less than 50%. Neuroblastoma tumors treated with the histone deacetylase inhibitor (HDACi) vorinostat have increased infiltration of macrophages with upregulated immune cell-surface receptors. Neuroblastoma cells release VEGF and M-CSF, which may alter intratumoral macrophage populations. VEGF has also been implicated in alteration of amyloid precursor protein family processing. Our lab demonstrated that amyloid precursor protein 2 (APLP2), a member of the amyloid precursor protein …

Fit For Life: Educating About A Healthy Lifestyle In Omaha Elementary Schools, Emilee Greager, Mary Conklin, Jordan Nustad, Tyler Bendrick, Haley Bremer, Tavishi Chopra, Jane Driano, Connor Eggleston, Lily Foley, Jeffrey Kepple, Ryan Leuty, Taylor Mattice, Alison Mause, Elizabeth Mause, Molly Olsen, Breanne Parets, Heerali Patel, Thomas Pennington, Miranda Prints, Eva Schaible, Christopher Toivonen, Grace Vitek, Claire Willman, Allen Zheng, Kody Moffatt

Child Health Research Institute Pediatric Research Forum

Background: The prevalence of childhood obesity is increasing across the country. Health education at a young age is critical for children to establish healthy habits. The Fit for Life program is put together by Creighton medical students to inspire elementary students to lead a healthy lifestyle. The curriculum integrates physical exercise, real organ demonstrations, emotional wellness exercises, and nutritious meal building to model and encourage healthy habits.

Methods: Over four weeks, medical students taught the 4^th and 5^th graders of two Omaha schools over live video while they followed along with hands-on activities. Before and after the program, …

Postnatal Diagnosis Of Dandy Walker Malformation With Encephalocele, Heerali Patel

Child Health Research Institute Pediatric Research Forum

Encephalocele is a defect of the neural tube. Dandy Walker malformation (DWM) is a defect involving agenesis or hypoplasia of the cerebellar vermis, cystic dilation of the fourth ventricle, and enlargement of the posterior fossa. This is a case of a female preterm infant born at 36 weeks’ gestation who presented with DWM and an encapsulated occipital encephalocele at birth. The pregnancy was complicated by poorly controlled maternal diabetes. Prenatal ultrasound indicated a central nervous system anomaly. Amniocentesis did not show chromosomal abnormality. The infant also presented with polydactyly of the right thumb. MRI showed evidence of soft tissue within …

Evaluation Of Brain Cholesterol Metabolism After Neonatal Hypoxic-Ischemic Brain Injury, Amanda Dave, Luke Allen, Zeljka Korade, Eric Peeples

Child Health Research Institute Pediatric Research Forum

Introduction

Neonatal hypoxic-ischemic brain injury (HIBI), resulting from impaired cerebral blood flow and oxygen delivery to the brain, affects at least 1.5 per 1,000 live births each year in the United States. Altered levels of cholesterol and cholesterol metabolites have been identified in brain tissue and in serum after adult brain injury such as traumatic brain injury and stroke. We hypothesized that there would be temporal and brain region-specific alterations in cholesterol and sterol precursors after neonatal HIBI.

Methods

Postnatal day 9 CD1 mouse pups were anesthetized with isoflurane and randomized to HIBI induced by carotid artery ligation or controls …

Perceptions Of Pediatricians On The Sars-Cov2 Vaccine, Karl Khandalavala, Kari Simonsen, Jeffrey A. Robinson

Child Health Research Institute Pediatric Research Forum

Introduction: The novel SARs-CoV2 coronavirus has infected approximately 30,000,000 people in the USA. To date, approximately 146 million doses of SARs-CoV2 vaccines have been administered. Perceptions of rapid vaccine development have resulted in reported vaccine hesitancy and refusal. Though not currently FDA approved for use in pediatric populations, but pediatric vaccination may contribute to herd immunity. Previous study demonstrates that physician recommendations significantly impact perceptions of routine pediatric immunizations among parents. Given this historical data, this study aimed to describe the strength of recommendation by pediatricians for the SARs-CoV2 vaccine for pediatric patients and their parents.

Methods: A …

Food Protein Induced Enterocolitis Syndrome Triggers At Children’S Hospital, Rachel Nelson, Russell Hopp

Child Health Research Institute Pediatric Research Forum

Background

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food disease characterized by delayed vomiting and diarrhea, often at the first ingestion of the food. The clinical phenotype of FPIES, including trigger food and disease course, varies by region. A literature review of the most common FPIES foods in children in the United States and worldwide includes cow’s milk, soy, oats, rice, and egg.

Objective:

We sought to survey the most common FPIES foods seen at Children’s hospital in the past five years.

Methods:

A data search of the EPIC electronic medical record using FPIES codes ICD-10 (K52.21) and ICD-9 …

Femoral And Tibial Indications For Initial And Reoperation Surgeries With Fassier-Duval Intramedullary Rods For Children With Osteogenesis Imperfecta, Adriano Dellapolla, Kaeli Samson, Tinotenda Sekeramayi, Arianna Dalamaggas, Megan Gillespie, Vincent Eaton, Elizabeth Strudthoff, Maegen Wallace

Child Health Research Institute Pediatric Research Forum

Background: Osteogenesis imperfecta (OI) is a genetic connective tissue disorder affecting quantity and integrity of collagen type I, which is integral for the strength of osseus tissue. OI has a heterogenous molecular inheritance pattern – divided into four major subgroups (I-IV). Defects in collagen protein products lead to poor development of skeletal structures and increased fracture rates. Children with OI suffer from multiple fractures and bone deformities often requiring surgical intervention with osteotomies and intramedullary telescoping rods, most often with Fassier-Duval (FD) rods. Our study–updated cohort from 2018 paper Azzam et al.–looked at the relationship between initial and reoperation indications …