Medical Specialties Commons^™

Household Income, Psychosocial Stressors, And Risk Factors For Sleep-Related Infant Deaths, Jeffrey D. Colvin, Isabella Zaniletti, Carolyn Ahlers-Schmidt, Vicki Collie-Akers, Christy Schunn, Rosemary Nabaweesi, Debbie Cheney, Rachel Y. Moon

Posters

Background: Sleep-related infant deaths (e.g., SIDS, accidental suffocation, undetermined) are the leading cause of postneonatal mortality. Infants from low income families have higher rates of sleep-related deaths. Other risk factors for sleep-related death include nonsupine sleep position, bedsharing, maternal smoking, sleeping in separate room, soft bedding, and breastfeeding <8 weeks. Little is known about how these risk factors vary by income or if psychosocial stressors, social services, and education of parents from healthcare providers about risk factors influence these risks among low income families.


Objective: Aim 1: Describe differences in risk factors for sleep-related infant death by family income. Aim 2: Among low income families, describe differences in risk factors by psychosocial stressors, services, and risk-factor education.


Design/Methods: We analyzed 2016-2017 CDC Pregnancy Risk Assessment Monitoring Study (PRAMS) data in 47 states. PRAMS questions mothers of …

Simulation Based Clinical Systems Testing In The Pediatric Emergency Department To Prepare For Covid-19 Pandemic, Christopher S. Kennedy, Marc Sycip, Lisa Ell, Shautonja Woods

Posters

Background: The COVID-19 prompted pediatric emergency departments (PED) to prepare for a surge in patients. In response, guidelines developed represent “work as imagined” and may not reflect work as done. In situ simulations could identify gaps and help to mitigate errors. Simulation-based clinical systems tests (SbCSTs) can detect latent safety threats (LSTs) in systems design.


Objective: Our aim was to use SbCSTs combined with rapid cycle training to test hospital system modifications for ED preparation. This method represenst a new application of SbCSTs. The research questions were in 2 frames: 1. Can rapidly deployed SbCSTs identify LSTs and recommendations for …

A Smarter Way To Round, Charles Maloy, Adrienne G. Deporre, Erica Adams, Jessica Olson, Amber Hunley, Darcy K. Weidemann

Posters

Background: Communication is a key driver of health care outcomes. Poor communication practices contribute to sentinel safety events, poor family/patient experiences, and delays in care. Use of checklists in the healthcare setting is important to the development of high reliability and is increasingly common, however, the development and implementation of medical checklists for the standard patient floor are inadequately described.


Objective: To develop a sustainable rounding checklist for multidisciplinary discussion of patient safety measures and clinical plans.


Design/Methods: Key stakeholders in the rounding process (subspecialty and general pediatrics physicians, resident physician, bedside nurses, and nursing leadership) created a rounding checklist …

A Deleterious Ephb4 Mutation Suppresses Prox1 Expression And Disrupts Lymphatic Development In Neonatal Non-Immune Hydrops, Gangaram Akangire, Heather Menden, Sheng Xia, Atif Ahmed, Venkatesh Sampath

Posters

Background: Non-immune hydrops fetalis is often a fatal condition and in 20% of cases related to lymphatic anomalies. Maldevelopment of lymphatic valves, atretic or absence of central lymphatic vessels has been described in hydrops fetalis, but the genetic basis of these anomalies remains undefined. Recent reports suggest that Ephrin type B receptor 4 (EPHB4) gene has a significant role in embryonic development of lymphatic system. To test the hypothesis that EPHB4 mutations can cause hydrops fetalis, we combined whole exome sequencing (WES) with functional and pathological analysis of an infant who succumbed to neonatal hydrops.


Objective: To discover the possible …

A Novel Way To Describe Variation In Antibiotic Use, Adrienne G. Deporre, Troy Richardson, Jason Newland, Mark Neuman

Posters

Background: A broader understanding of antibiotic variation can inform stewardship efforts. Diversity indices are frequently calculated in ecologic studies to measure the variety of species in a given habitat, however they have not yet been used to study variation in antibiotic use in the healthcare setting.


Objective: To develop a novel assessment of antibiotic variation, based on the breadth of an antibiotic’s associated conditions as well as its use, measured by days of therapy (DOT).


Design/Methods: We assessed antibiotic use in children aged 60 days-18 years hospitalized at a US Children’s hospital included in the Pediatric Health Information System (PHIS) …

Parental Health Literacy And Acute Care Utilization In Children With Medical Complexity, Emily J. Goodwin, Joy L. Solano, Jessica L. Bettenhausen, Ryan Coller, Adrienne G. Deporre, Rupal Gupta, Kayla R. Heller, Lauren Jones, Leah Jones, Kathyrn Kyler, Ingrid Larson, Laura Plencner, Margaret Queen, Timothy Ryan Smith, Tyler Smith, Jacqueline M. Walker, Margaret Wright, Isabella Zaniletti, Jeffrey D. Colvin

Posters

Background: Inadequate health literacy, defined as inadequate ability to find, understand and use health information is associated with poor health outcomes and high health care costs. Children with medical complexity (CMC) have high rates of acute care utilization. Understanding parental health literacy in CMC and its relationship to acute care utilization may inform interventions designed to lower utilization.


Objective: To examine parental health literacy for CMC and determine its association with acute care utilization.


Design/Methods: In this single-site cross-sectional study, 250 parents of CMC completed a self-administered survey (response rate of 66.1%). CMC were included if they had a complex …

Medical Neglect: Trends In Reporting Practices And Patient Characteristics, Danielle Horton, Emily Killough, Mary Moffatt

Posters

Background: Medical neglect has significant consequences on child health and well-being. Improved processes for defining, recognizing, and reporting medical neglect are needed, however there is limited published data on the subject. Insight into patient characteristics and current reporting practices will help identify areas for intervention to improve outcomes for children at risk for medical neglect.


Objective: To describe characteristics of children identified for concern of medical neglect and trends in reporting of cases to child protective services (CPS).


Design/Methods: We performed a 1-year retrospective descriptive study of subjects < 18 years of age at a single, tertiary-care, academic pediatric institution for whom a Patient at Risk (PAR) assessment was completed exclusively for the concern of medical neglect. The PAR is a unique institutional process by which health professionals and social workers document concerns for child maltreatment. Medical records were reviewed for data regarding demographics, medical history, health care utilization and psychosocial risk factors. The role of the initiating provider and healthcare setting in which the PAR was recorded were also obtained.


Results: Of 270 children with a PAR completed for medical neglect concerns, …

Educating Providers: Timely Post-Operative Pain Management In A Level Iv Neonatal Intensive Care Unit (Nicu), Jamesia Donato, Darian Younger, Rebecca Palmer, Denise Smith, Alexandra Oschman, Meredith Kopp, Daphne Reavey, Eugenia K. Pallotto

Posters

Background: NICU patients often encounter painful procedures and can have significant short and long-term consequences from inadequately treated pain. Ensuring adequate pharmacological and non-pharmacological pain management while minimizing associated risks is paramount. Our NICU is part of the Children’s Hospitals Neonatal Consortium “Erase Pain” collaborative to improve management of postop pain in neonates.


Objective: SMART

Aim: Increase the percentage of patients receiving acetaminophen within one hour after surgery to greater than 70% by July 2020.


Design/Methods: A multidisciplinary team developed pain treatment algorithms and postop order plans to standardize the approach to postop pain management. Providers were educated about their …

Outpatient Antimicrobial Stewardship Programs In Children’S Hospitals: Status, Needs, Barriers, Rana El Feghaly, Elizabeth Monsees, Alaina N. Burns, Brian Lee, Ann L. Wirtz, Adam L. Hersh, Jason Newland

Posters

Background: Antimicrobial stewardship programs (ASPs) are an essential tool to combat the increasing threat of antibiotic resistance. ASPs traditionally reside in acute care settings with a focus on inpatient prescribing. However, in 2016, the Centers for Disease Control and Prevention affirmed the importance of outpatient ASP through its 4 core elements. Incorporation of these elements requires time, personnel, and funding, which may not be available in many institutions.


Objective: This study aimed to evaluate the current state of outpatient ASP in a large network of children’s hospitals and inform a programming agenda.


Design/Methods: This cross-sectional study used an investigator-developed survey …

Prenatal Diagnosis Of An Uncommon Form Of A Hypoplastic Left Heart Syndrome Variant., Anmol Goyal, Kelsey Pinnick, Rita France, Maria Kiaffas

Posters

Background: Mitral valve dysplasia syndrome (MVDS) is a rare form of congenital heart disease, similar to hypoplastic left heart syndrome (HLHS). Prenatal identification is important for counseling, delivery planning and postnatal management.

Case: A 39 year-old woman underwent fetal echocardiography at ~34 weeks gestation for evaluation of fetal cardiomegaly and hydrops revealing: biatrial enlargement, mild-to-moderate mitral and tricuspid valve insufficiency, echobright mitral valve apparatus, biventricular dilation, severe LV fibroelastosis (EFE) and systolic dysfunction, mild hypoplasia of aortic valve annulus and aortic arch, and a thick restrictive atrial septum (RAS) with left to right flow (Fig 1a-c).

Decision‐making: Although critical aortic …

Peripartum Antibiotics Induce Dysbiosis And Predispose The Neonatal Gut Towards Inflammation, Alain Cuna, Marianne N. Nsumu, Venkatesh Sampath

Posters

Background: Broad-spectrum antibiotics in preterm infants have been associated with necrotizing enterocolitis (NEC), but the exact mechanisms that explain this association remain poorly defined.


Objective: To examine the impact of peripartum antibiotics on gut microbiota and intestinal inflammation in the developing gut.


Design/Methods: C57BL6 dams were administered broad spectrum antibiotics mixed with sterile drinking water from E15 to postnatal day (P)14 (Fig 1). Gut microbiota were analyzed by targeted 16S PCR for total bacterial density and relative abundance of major bacterial phyla. Effects of antibiotics on inflammatory TLR-signaling and injury in the neonatal gut were evaluated with PCR and histology. …

Response To Dexamethasone Predicts Diagnosis Of Severe (Type 2) Bronchopulmonary Dysplasia Or Death, Christopher R. Nitkin, Keith Feldman, Alain Cuna, Alexandra Oschman, William E. Truog, Michael Norberg, Jane Taylor, Tamorah Lewis

Posters

Background: Bronchopulmonary dysplasia (BPD) is the most common respiratory morbidity after preterm birth but requires diagnosis at 36 weeks postmenstrual age (PMA). Dexamethasone is often used to treat infants at high-risk of BPD. The ability for earlier prediction of BPD, based on steroid response, could be useful as a surrogate marker for new therapies.


Objective: To construct a model that predicts severe BPD or death at 36 weeks PMA based on clinical response to dexamethasone.


Design/Methods: Retrospective chart review of preterm infants treated with dexamethasone between 2010-2020 at a Level IV NICU with data collected on demographics, age of steroid …

Characterization Of Comorbidities In Patients With A Dual Diagnosis Of Down Syndrome And Autism Spectrum Disorder Using Cerner Health Facts, Michael Slogic, Earl F. Glynn, Cy Nadler, Meredith Dreyer, Sarah T. Edwards

Presentations

Background: Up to 19% of patients with Down syndrome (DS) meet diagnostic criteria for autism spectrum disorder (ASD) (Channell, et al, 2019). While the medical and psychological comorbidities for patients with DS or ASD are well characterized, outcomes for patients with a dual diagnosis (DS-ASD) are poorly understood. Large DS-ASD cohorts and comparison samples are needed to fill this gap, as well as methods for grouping and analyzing complex diagnostic phenotypes. Objective: Our objective is to utilize Cerner Health Facts, a multi-institutional healthcare database, to identify large populations with DS, ASD, and DS-ASD, allowing for characterization and comparison of their …

Investigation Of Alpl Variant States And Clinical Outcomes: An Analysis Of Adults And Adolescents With Hypophosphatasia Treated With Asfotase Alfa., Priya S. Kishnani, Guillermo Del Angel, Shanggen Zhou, Eric T. Rush

Manuscripts, Articles, Book Chapters and Other Papers

Background: Hypophosphatasia (HPP), a rare metabolic disease, can be inherited in an autosomal recessive (biallelic) or an autosomal dominant (monoallelic) manner. Most of the severe, early-onset, frequently lethal HPP in infants is acquired through recessive inheritance; less severe, later-onset, typically nonlethal HPP phenotypes are acquired through either dominant or recessive inheritance. HPP's variable clinical presentation arises from >400 identified ALPL pathogenic variants with likely variable penetrance, especially with autosomal dominant inheritance. This post hoc analysis investigated the relationship between ALPL variant state (biallelic and monoallelic) and clinical outcomes with asfotase alfa in HPP.

Methods: Data were pooled from two phase …

Recalibration Of The Renal Angina Index For Pediatric Septic Shock., Natalja L. Stanski, Hector R. Wong, Rajit K. Basu, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Scott L. Weiss, Michael T. Bigham, Parag N. Jain, Adam Schwarz, Riad Lutfi, Jeffrey Nowak, Geoffrey L. Allen, Neal J. Thomas, Jocelyn R. Grunwell, Michael Quasney, Bereketeab Haileselassie, Lakhmir S. Chawla, Stuart L. Goldstein

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Sepsis-associated acute kidney injury (AKI) is a common diagnosis in children that is associated with poor outcomes. The lack of therapeutic options once present makes early identification of at-risk patients essential. The renal angina index (RAI) has been previously validated to predict severe AKI in heterogeneous populations of critically ill children. The performance of this score specifically in children with septic shock is unknown.

Methods: A secondary analysis of a multicenter, prospective, observational study of 379 children with septic shock to determine the ability of the RAI to predict severe AKI at day 3, and to assess for the …

Global Trends In Norovirus Genotype Distribution Among Children With Acute Gastroenteritis., Jennifer L. Cannon, Joseph Bonifacio, Filemon Bucardo, Javier Buesa, Leesa Bruggink, Martin Chi-Wai Chan, Tulio M. Fumian, Sidhartha Giri, Mark D. Gonzalez, Joanne Hewitt, Jih-Hui Lin, Janet Mans, Christian Muñoz, Chao-Yang Pan, Xiao-Li Pang, Corinna Pietsch, Mustafiz Rahman, Naomi Sakon, Rangaraj Selvarangan, Hannah Browne, Leslie Barclay, Jan Vinjé

Manuscripts, Articles, Book Chapters and Other Papers

Noroviruses are a leading cause of acute gastroenteritis (AGE) among adults and children worldwide. NoroSurv is a global network for norovirus strain surveillance among children(genotype and polymerase type) and uploaded 1,325 dual-typed sequences to the NoroSurv web portal during 2016-2020. More than 50% of submitted sequences were GII.4 Sydney[P16] or GII.4 Sydney[P31] strains. Other common strains included GII.2[P16], GII.3[P12], GII.6[P7], and GI.3[P3] viruses. In total, 22 genotypes and 36 dual types, including GII.3 and GII.20 viruses with rarely reported polymerase types, were detected, reflecting high strain diversity. Surveillance data captured in NoroSurv enables the monitoring of trends in norovirus strains …

Critical Need For Pharmacologic Treatment Options In Nafld: A Pediatric Perspective., Chance S. Friesen, Sherwin S. Chan, Jonathan B. Wagner, Chelsea Hosey-Cojocari, Iván L Csanaky, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

Nonalcoholic fatty liver disease (NAFLD) affects up to 70% of children with obesity and has become the number one etiology for liver transplant in the United States. Early, effective intervention is critical to prevent disease progression into adulthood. Yet, it is seldom achieved through lifestyle modification alone. Thus, children must be included in NAFLD pharmacology trials, which, to date, continue to focus primarily on adult populations. This commentary serves as a call to action.

Real-Time Gastrointestinal Infection Surveillance Through A Cloud-Based Network Of Clinical Laboratories., Juliana M. Ruzante, Katherine Olin, Breda Munoz, Jeff Nawrocki, Rangaraj Selvarangan, Lindsay Meyers

Manuscripts, Articles, Book Chapters and Other Papers

Acute gastrointestinal infection (AGI) represents a significant public health concern. To control and treat AGI, it is critical to quickly and accurately identify its causes. The use of novel multiplex molecular assays for pathogen detection and identification provides a unique opportunity to improve pathogen detection, and better understand risk factors and burden associated with AGI in the community. In this study, de-identified results from BioFire® FilmArray® Gastrointestinal (GI) Panel were obtained from January 01, 2016 to October 31, 2018 through BioFire® Syndromic Trends (Trend), a cloud database. Data was analyzed to describe the occurrence of pathogens causing AGI across United …

Health-Related Quality Of Life Across Recent Pediatric Obesity Classification Recommendations., William R. Black, Kelsey B. Borner, Marshall T. Beauchamp, Ann M. Davis, Meredith Dreyer, Brooke Sweeney, Sarah Hampl

Manuscripts, Articles, Book Chapters and Other Papers

Extreme body mass index (BMI) values (i.e., above the 97th and below the 3rd percentiles) are inaccurately represented on the Centers for Disease Control and Prevention's growth curves, which may limit the utility of BMI percentile and BMI z-score for capturing changes in clinical outcomes for patients at extreme weights. Modeling child obesity severity based upon the percentage of BMI in excess of the 95th percentile (BMI95pct) has been proposed as an improved metric to better capture variability in weight at extreme ends of growth curves, which may improve our understanding of relationships between weight status and changes in clinical …

The Prevalence Of Hyperpalatable Baby Foods And Exposure During Infancy: A Preliminary Investigation., Kai Ling Kong, Tera L. Fazzino, Kaitlyn M. Rohde, Katherine S. Morris

Manuscripts, Articles, Book Chapters and Other Papers

Objective: To characterize the prevalence of hyperpalatable foods (HPF) among baby foods in the U.S. and examine the prevalence of HPF exposure and consumption from both baby food and adult food sources among infants aged 9-15 months.

Methods: A U.S. baby food database as well as baby foods from three 24-h dietary recalls of 147 infants were used to identify baby foods as HPF per previous publication. HPF exposure was defined as intake of any HPF during the 3-day measurement period. To determine the extent of HFP consumption, % kilocalorie (kcal) intake from HPF was characterized.

Results: Only 12% of …

Efficacy Of Weight Reduction On Pediatric Nonalcoholic Fatty Liver Disease: Opportunities To Improve Treatment Outcomes Through Pharmacotherapy., Chance S. Friesen, Chelsea Hosey-Cojocari, Sherwin S. Chan, Iván L. Csanaky, Jonathan B. Wagner, Brooke Sweeney, Alec Friesen, Jason D. Fraser, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

Obesity is the single greatest risk factor for nonalcoholic fatty liver disease (NAFLD). Without intervention, most pediatric patients with NAFLD continue to gain excessive weight, making early, effective weight loss intervention key for disease treatment and prevention of NAFLD progression. Unfortunately, outside of a closely monitored research setting, which is not representative of the real world, lifestyle modification success for weight loss in children is low. Bariatric surgery, though effective, is invasive and can worsen NAFLD postoperatively. Thus, there is an evolving and underutilized role for pharmacotherapy in children, both for weight reduction and NAFLD management. In this perspective article, …

Hydronephrosis Classifications: Has Utd Overtaken Apd And Sfu? A Worldwide Survey., Santiago Vallasciani, Anna Bujons Tur, John Gatti, Marcos Machado, Christopher S. Cooper, Marie Klaire Farrugia, Huixia Zhou, Mohammed El Anbari, Pedro-José Lopez

Manuscripts, Articles, Book Chapters and Other Papers

Objective: To collect baseline information on the ultrasonographic reporting preferences.

Method: A 13-multiple choice questionnaire was designed and distributed worldwide among pediatric urologists, pediatric surgeons, and urologists. The statistical analysis of the survey data consisted of 3 steps: a univariate analysis, a bivariate and a multivariate analysis.

Results: Three hundred eighty participants responded from all the continents. The bivariate analysis showed the significant differences in the geographical area, the years of experience and the volume of cases. Most of the physicians prefer the SFU and APD systems because of familiarity and simplicity (37 and 34%, respectively). Respondents noted that their …

Leveraging Institutional Support To Build An Integrated Multidisciplinary Care Model In Pediatric Inflammatory Bowel Disease., Jennifer Verrill Schurman, Craig A. Friesen

Manuscripts, Articles, Book Chapters and Other Papers

While the biopsychosocial nature of inflammatory bowel disease (IBD) is now well accepted by clinicians, the need for integrated multidisciplinary care is not always clear to institutional administrators who serve as decision makers regarding resources provided to clinical programs. In this commentary, we draw on our own experience in building successful integrated care models within a division of pediatric gastroenterology (GI) to highlight key considerations in garnering initial approval, as well as methods to maintain institutional support over time. Specifically, we discuss the importance of making a strong case for the inclusion of a psychologist in pediatric IBD care, justifying …

Delta-Like 4 Is Required For Pulmonary Vascular Arborization And Alveolarization In The Developing Lung., Sheng Xia, Heather Menden, Nick Townley, Sherry M. Mabry, Jeffrey J. Johnston, Michael F. Nyp, Daniel P. Heruth, Thomas Korfhagen, Venkatesh Sampath

Manuscripts, Articles, Book Chapters and Other Papers

The molecular mechanisms by which endothelial cells (ECs) regulate pulmonary vascularization and contribute to alveolar epithelial cell development during lung morphogenesis remain unknown. We tested the hypothesis that delta-like 4 (DLL4), an EC Notch ligand, is critical for alveolarization by combining lung mapping and functional studies in human tissue and DLL4-haploinsufficient mice (Dll4+/lacz). DLL4 expressed in a PECAM-restricted manner in capillaries, arteries, and the alveolar septum from the canalicular to alveolar stage in mice and humans. Dll4 haploinsufficiency resulted in exuberant, nondirectional vascular patterning at E17.5 and P6, followed by smaller capillaries and fewer intermediate blood vessels at P14. Vascular …

Heartburn In Children And Adolescents In The Presence Of Functional Dyspepsia And/Or Irritable Bowel Syndrome Correlates With The Presence Of Sleep Disturbances, Anxiety, And Depression., Jennifer Colombo, Amanda D. Deacy, Jennifer Verrill Schurman, Craig A. Friesen

Manuscripts, Articles, Book Chapters and Other Papers

ABSTRACT: The aim of this study was to assess the relationship of heartburn in pediatric patients with functional dyspepsia (FD) and irritable bowel syndrome (IBS) with gastrointestinal symptoms, sleep disturbances, and psychologic distress.The overlap in symptoms of FD, IBS, and gastroesophageal reflux disease (GERD) predicts greater symptom severity and decreased quality of life and presents opportunities for improved diagnostic classification and personalized therapeutics.A cross-sectional observational study of 260 pediatric patients with abdominal pain was conducted. Patients completed standardized questionnaires assessing clinical symptoms, sleep quality, and psychologic symptoms during routine clinical care. Questionnaire data were compared for patients reporting heartburn and …

Attention Deficit Hyperactivity Disorder: Treatment, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Attention Deficit Hyperactivity Disorder: Assessment, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Reliability Of The Cutting Alignment Scoring Tool (Cast) To Assess Trunk And Limb Alignment During A 45-Degree Side-Step Cut., Lauren S. Butler, Eryn K. Milian, Amie Deverna, Kevin Latz, Henry B. Ellis, Alexa R. Martinez, Kristin Hayden, Christian Gerstenkorn, Sara C. Carpenito, Charles W. Wyatt, Dai Sugimoto

Manuscripts, Articles, Book Chapters and Other Papers

Background: Three-dimensional (3D) motion analysis is considered the gold standard for evaluating human movement. However, its clinical utility is limited due to cost, operating expertise, and lengthy data processing time. Numerous qualitative scoring systems have been introduced to assess trunk and lower extremity biomechanics during functional tasks. However, the reliability of qualitative scoring systems to evaluate cutting movements is understudied. Purpose/Hypotheses: To assess the inter-rater and intra-rater reliability of the Cutting Alignment Scoring Tool (CAST) among sports medicine providers and to evaluate rater agreement of each component of the CAST. The hypotheses were: 1) there would be good-to-excellent inter-rater and …

Comorbidities And Complications In Adults With Pyruvate Kinase Deficiency., Audra N. Boscoe, Yan Yan, Elizabeth Hedgeman, Eduard J. Van Beers, Hanny Al-Samkari, Wilma Barcellini, Stefan W. Eber, Bertil Glader, Hassan M. Yaish, Satheesh Chonat, Mukta Sharma, Kevin H M Kuo, Ellis J. Neufeld, Heng Wang, Madeleine Verhovsek, Sujit Sheth, Rachael F. Grace

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Pyruvate kinase (PK) deficiency is caused by PKLR gene mutations, leading to defective red blood cell glycolysis and hemolytic anemia. Rates of comorbidities and complications by transfusion history and relative to the general population remain poorly quantified.

METHODS: Data for patients aged ≥ 18 years with two confirmed PKLR mutations were obtained from the PK deficiency Natural History Study (NCT02053480). Frequencies of select conditions were compared with an age- and sex-matched cohort from a general insured US population without PK deficiency.

RESULTS: Compared with the matched population (n = 1220), patients with PK deficiency (n = 122) had significantly …

Mechanical Support Of Superior Cavopulmonary (Glenn) Physiology To Heart Transplantation, Edo K S Bedzra, Aliessa P. Barnes, Brian Birnbaum, James D. St. Louis

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.