Medicine and Health Sciences Commons^™

Incidence Of Initial Renal Replacement Therapy Over The Course Of Kidney Disease In Children., Derek K. Ng, Matthew B. Matheson, Bradley A. Warady, Susan R. Mendley, Susan L. Furth, Alvaro Muñoz

Manuscripts, Articles, Book Chapters and Other Papers

The Chronic Kidney Disease in Children Study, a prospective cohort study with data collected from 2003 to 2018, provided the first opportunity to characterize the incidence of renal replacement therapy (RRT) initiation over the life course of pediatric kidney diseases. In the current analysis, parametric generalized gamma models were fitted and extrapolated for RRT overall and by specific treatment modality (dialysis or preemptive kidney transplant). Children were stratified by type of diagnosis: nonglomerular (mostly congenital; n = 650), glomerular-hemolytic uremic syndrome (HUS; n = 49), or glomerular-non-HUS (heterogeneous childhood onset; n = 216). Estimated durations of time to RRT after …

Anticipation, Accompaniment, And A Good Death In Perinatal Care., Bryanna S. Moore, Brian S. Carter, Bryan Beaven, Katie House, Joel House

Manuscripts, Articles, Book Chapters and Other Papers

The ethics of perinatal care, and the experiences of families who receive such care, remains a nascent area of inquiry. It can be hard to see how existing "good death" constructs apply to the experiences of fetal patients and their families. In this paper, we explore two themes raised by a case at our fetal health center: anticipation and accompaniment. In this case, a mother presented to our fetal health center; her unborn son, our fetal patient, was diagnosed with life-threatening hypoplastic left heart syndrome and endocardial fibroelastosis. The parents were told that their son's life expectancy, upon birth, was …

Transanal-Only Swenson-Like Pull-Through For Late Diagnosed Hirschsprung Disease., Rebecca M. Rentea, Devin R. Halleran, Hira Ahmad, Elias Maloof, Richard J. Wood, Marc A. Levitt

Manuscripts, Articles, Book Chapters and Other Papers

Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A transanal-only pull-through is a well-described approach but in the newborn period risks a situation whereby the transition zone is higher than the sigmoid. We present our experience with the unique patient population of older HD patients in whom the transition zone was reliably reachable via a single-stage transanal approach, performed in prone position. Patients between 2 and 6 years of age with a rectal or sigmoid …

Dr. Ahmed Abdelmoity To Receive Endowed Chair, Children's Mercy Hospital

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Former Children’s Mercy CEO Rand O’Donnell, PhD, and his wife, Melva, are establishing an endowed chair position that will be awarded to Ahmed Abdelmoity, MD, FAAP, Division Director of Child Neurology, Section Chief of Epilepsy and Neurophysiology. Dr. Abdelmoity will become our 31st endowed chair at Children’s Mercy.

Unraveling Complexity About Childhood Obesity And Nutritional Interventions: Modeling Interactions Among Psychological Factors., Keith Feldman, Gisela M B Solymos, Maria Paula De Albuquerque, Nitesh V. Chawla

Manuscripts, Articles, Book Chapters and Other Papers

As the global prevalence of childhood obesity continues to rise, researchers and clinicians have sought to develop more effective and personalized intervention techniques. In doing so, obesity interventions have expanded beyond the traditional context of nutrition to address several facets of a child's life, including their psychological state. While the consideration of psychological features has significantly advanced the view of obesity as a holistic condition, attempts to associate such features with outcomes of treatment have been inconclusive. We posit that such uncertainty may arise from the univariate manner in which features are evaluated, focusing on a particular aspect such as …

An Electronic Medical Records-Based Approach To Identify Idiosyncratic Drug-Induced Liver Injury In Children., Tracy L. Sandritter, Jennifer Goldman, Clayton J Habiger, James F. Daniel, Jennifer Lowry, Ryan T. Fischer

Manuscripts, Articles, Book Chapters and Other Papers

Drug-induced liver injury (DILI) is the leading cause of liver failure in the United States and the most common cause of drug recall. As opposed to the recognized direct toxicity of super-therapeutic acetaminophen or chemotherapeutic agents in children, limited data exists for pediatric populations on the incidence of idiosyncratic DILI (iDILI) that may develop independently of drug dose or duration of administration. To improve the detection of adverse drug reactions at our hospital, we utilized electronic medical records-based automated trigger tools to alert providers of potential iDILI. Clinical criteria concerning for iDILI were defined as serum ALT > 5x or serum …

Msto1 Mutations Cause Mtdna Depletion, Manifesting As Muscular Dystrophy With Cerebellar Involvement., S Donkervoort, R Sabouny, P Yun, L Gauquelin, K R Chao, Y Hu, I Al Khatib, A Töpf, P Mohassel, B B Cummings, R Kaur, D Saade, S A Moore, L B Waddell, M A Farrar, J K Goodrich, P Uapinyoying, S H S Chan, A Javed, M E Leach, P Karachunski, J Dalton, L Medne, A Harper, C Thompson, Isabelle Thiffault, S Specht, R E Lamont, Carol J. Saunders, H Racher, F P Bernier, D Mowat, N Witting, J Vissing, R Hanson, Keith A. Coffman, Meagan K. Hainlen, J S Parboosingh, A Carnevale, G Yoon, R E Schnur, Care4rare Canada Consortium, K M Boycott, J K Mah, V Straub, A Reghan Foley, A M Innes, C G Bönnemann, T E Shutt

Manuscripts, Articles, Book Chapters and Other Papers

MSTO1 encodes a cytosolic mitochondrial fusion protein, misato homolog 1 or MSTO1. While the full genotype-phenotype spectrum remains to be explored, pathogenic variants in MSTO1 have recently been reported in a small number of patients presenting with a phenotype of cerebellar ataxia, congenital muscle involvement with histologic findings ranging from myopathic to dystrophic and pigmentary retinopathy. The proposed underlying pathogenic mechanism of MSTO1-related disease is suggestive of impaired mitochondrial fusion secondary to a loss of function of MSTO1. Disorders of mitochondrial fusion and fission have been shown to also lead to mitochondrial DNA (mtDNA) depletion, linking them to the mtDNA …

Pneumothorax: What’S A Pediatric Pulmonologist To Do?, Terrence W. Carver

Manuscripts, Articles, Book Chapters and Other Papers

A pneumothorax is a collection of air in the pleural space commonly associated with respiratory symptoms. Patients may be relatively asymptomatic but may present with life-threatening distress. Confirmation of a clinically suspected pneumothorax is most often by chest x-ray when patients have been stabilized. Pneumothoraces are first categorized as primary, secondary, or iatrogenic and informs the evaluation and treatment plan. However, despite management guidelines in the literature, their applicability for pediatric patients is limited. Understanding of the historical risk factors including the acute clinical course and how radiographs, both chest X-ray and CT chest illuminate which patients are at highest …

The Early "Unnatural" History Following Surgical Repair Of Ventricular Septal Defects., Sathish M. Chikkabyrappa, Justin T. Tretter, Arpan R. Doshi, Sujatha Buddhe, Puneet Bhatla, Achi Ludomirsky

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed.

Methods: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed.

Results: The majority of …

Elevated Glucose Acts Directly On Osteocytes To Increase Sclerostin Expression In Diabetes, Donna M. Pacicca, Tammy Brown, Dara Watkins, Karen Kover, Yun Yan, Matthew Prideaux, Lynda Bonewald

Manuscripts, Articles, Book Chapters and Other Papers

© 2019, The Author(s).

Bone quality in diabetic patients is compromised, leading to weaker bones and increased fracture risk. However, the mechanism by which this occurs in diabetic bone remains to be fully elucidated. We hypothesized that elevated glucose and glucose variation would affect the function of osteocytes, essential regulators of bone homeostasis and quality. To first test this hypothesis, we used the IDG-SW3 osteocyte-like cell line to examine the effects of glucose levels on osteocyte function and viability in vitro. We confirmed our in vitro findings using the in vivo streptozotocin-induced (STZ) diabetic rat model and ex-vivo cultured osteocytes …

Gamna-Gandy Bodies Present As A Spleen Mass In A Patient With Hereditary Spherocytosis, Chandni Dargan, Sahibu Sultan M Habeebu, Grace S. Mitchell, Weijie Li

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Adapting The Diabetes Prevention Program For Low And Middle-Income Countries: Protocol For A Cluster Randomised Trial To Evaluate 'Lifestyle Africa'., Delwyn Catley, Thandi Puoane, Lungiswa Tsolekile, Ken Resnicow, Kandace Fleming, Emily A. Hurley, Joshua M. Smyth, Mara Z. Vitolins, Estelle V. Lambert, Naomi Levitt, Kathy Goggin

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Low and middle-income countries like South Africa are experiencing major increases in burden of non-communicable diseases such as diabetes and cardiovascular conditions. However, evidence-based interventions to address behavioural factors related to these diseases are lacking. Our study aims to adapt the CDC's National Diabetes Prevention Program (DPP) within the context of an under-resourced urban community in Cape Town, South Africa.

Methods/analysis: The new intervention (Lifestyle Africa) consists of 17 weekly sessions delivered by trained community health workers (CHWs). In addition to educational and cultural adaptations of DPP content, the programme adds novel components of text messaging and …

Nearly Complete Genome Sequences Of 17 Enterovirus D68 Strains From Kansas City, Missouri, 2018, Suman B. Pakala, Yi Tan, Ferdaus Hassan, Annie Mai, Robert H. Markowitz, Meghan H. Shilts, Seesandra V. Rajagopala, Rangaraj Selvarangan, Suman R. Das

Manuscripts, Articles, Book Chapters and Other Papers

Here, we report 17 nearly complete genome sequences of enterovirus D68 (EV-D68) isolated from Kansas City, MO, in 2018. Phylogenetic analysis suggests that these strains belong to subclade B3, similar to the ones that caused the 2016 epidemics in the United States but different from the 2014 outbreak B1 strains.

Pain, Functional Disability, And Their Association In Juvenile Fibromyalgia Compared To Other Pediatric Rheumatic Diseases., Mark Connelly, Jennifer E Weiss, Carra Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Severe pain and impairments in functioning are commonly reported for youth with juvenile fibromyalgia. The prevalence and impact of pain in other diseases commonly managed in pediatric rheumatology comparatively have been rarely systematically studied. The objective of the current study was to determine the extent to which high levels of pain and functional limitations, and the strength of their association, are unique to youth with juvenile primary fibromyalgia syndrome/JPFS) relative to other pediatric rheumatic diseases.

METHODS: Using data from 7753 patients enrolled in the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry, we compared the levels and …

Failure To Rescue As An Outcome Metric For Pediatric And Congenital Cardiac Catheterization Laboratory Programs: Analysis Of Data From The Impact Registry., Michael L. O'Byrne, Kevin F. Kennedy, Natalie Jayaram, Lisa J. Bergersen, Matthew J. Gillespie, Yoav Dori, Jeffrey H. Silber, Steven M. Kawut, Jonathan J. Rome, Andrew C. Glatz

Manuscripts, Articles, Book Chapters and Other Papers

Background Risk-adjusted adverse event (AE) rates have been used to measure the quality of pediatric and congenital cardiac catheterization laboratories. In other settings, failure to rescue (FTR) has demonstrated utility as a quality metric. Methods and Results A multicenter retrospective cohort study was performed using data from the IMPACT (Improving Adult and Congenital Treatment) Registry between January 2010 and December 2016. A modified FTR metric was developed for pediatric and congenital cardiac catheterization laboratories and then compared with pooled AEs. The associations between patient- and hospital-level factors and outcomes were evaluated using hierarchical logistic regression models. Hospital risk standardized ratios …

Pharmacometabolomics Of Respiratory Phenotypic Response To Dexamethasone In Preterm Infants At Risk For Bronchopulmonary Dysplasia., Tamorah R. Lewis Md Phd, Prabhakar Chalise, Cheri Gauldin, William E. Truog

Manuscripts, Articles, Book Chapters and Other Papers

A prospective cohort study was performed in preterm infants less than 32 weeks gestation at birth who were treated with dexamethasone for developing or established bronchopulmonary dysplasia (BPD). Respiratory phenotype (Respiratory Severity Score (RSS)), serum, and urine metabolomics were assessed before and after treatment. Ten infants provided nine matched serum and nine matched urine samples. There was a significant decrease in RSS with steroid treatment. Serum gluconic acid had the largest median fold change (140 times decreased, P = 0.008). In metabolite set enrichment analysis, in both serum and urine, the urea cycle, ammonia recycling, and malate-aspartate shuttle pathways were …

Standardizing Aminoglycoside Induced Ototoxicity Monitoring, Claire Elson, Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier

Posters

Aminoglycoside (AG) antibiotics are essential for the treatment of cystic fibrosis (CF) lung infections. Pseudomonas aeruginosa. Nontuberculous mycobacteria. Monitoring is critical secondary to potential nephrotoxicity and ototoxicity. Children’s Mercy Kansas City (CMKC), standardized nephrotoxicity monitoring in 2016. Observed variable ototoxicity monitoring practices. Prevalence of ototoxicity, 2016 CFF Patient Registry, 1.1% in pediatric patients (less than or equal to 18 years). 2.2% in pediatric and adult population. National Institute of Deafness and Other Communication Disorders, 13% in US population equal to or less than 12 years old. A standardized AG induced ototoxicity monitoring algorithm (AIOA) was developed and implemented at CMKC …

How To Use Peanut Ige Levels To Predict Challenge Outcomes In The Pediatric Population, Nalin U.G., Jay Portnoy, Jodi Shroba, Salman Aljubran

Posters

Introduction: The accepted clinical practice is to consider oral challenge when peanut IgE levels are 2.0 kUA/L or less in patients with a history of a major reaction or less than 5.0 kUA/L in patients with only sensitization. Certain populations are predisposed to having increased IgE levels, and these patients are often not offered challenges due to elevated numbers.

Methods: We reviewed unidentified patients information from January 2017 to July 2019 on peanut IgE levels and oral challenge outcomes through REDCap database. A ROC using SPSS from IBM was used and the points from the ROC curve were used to …

Sirna Targeting And Treatment Of Gastrointestinal Diseases., Rachel Chevalier

Manuscripts, Articles, Book Chapters and Other Papers

RNA interference via small interfering RNA (siRNA) offers opportunities to precisely target genes that contribute to gastrointestinal (GI) pathologies, such as inflammatory bowel disease, celiac, and esophageal scarring. Delivering the siRNA to the GI tract proves challenging as the harsh environment of the intestines degrades the siRNA before it can reach its target or blocks its entry into its site of action in the cytoplasm. Additionally, the GI tract is large and disease is often localized to a specific site. This review discusses polymer and lipid-based delivery systems for protection and targeting of siRNA therapies to the GI tract to …

The Pediatrician's Role In Eliminating Racial And Ethnic Disparities In Sleep-Related Infant Deaths., Jeffrey D. Colvin, Rachel Y. Moon

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Outcomes Of Hematopoietic Cell Transplantation In Patients With Germline Samd9/Samd9l Mutations., Ibrahim A. Ahmed, Midhat S. Farooqi, Mark T. Vander Lugt, Jessica Boklan, Melissa Rose, Erika D. Friehling, Brandon Triplett, Kenneth Lieuw, Blachy Davila Saldana, Christine M. Smith, Jason R. Schwartz, Rakesh K. Goyal

Manuscripts, Articles, Book Chapters and Other Papers

Germline mutations in SAMD9 and SAMD9L genes cause MIRAGE (myelodysplasia, infection, restriction of growth, adrenal hypoplasia, genital phenotypes, and enteropathy) (OMIM: *610456) and ataxia-pancytopenia (OMIM: *611170) syndromes, respectively, and are associated with chromosome 7 deletions, myelodysplastic syndrome (MDS), and bone marrow failure. In this retrospective series, we report outcomes of allogeneic hematopoietic cell transplantation (HCT) in patients with hematologic disorders associated with SAMD9/SAMD9L mutations. Twelve patients underwent allogeneic HCT for MDS (n = 10), congenital amegakaryocytic thrombocytopenia (n = 1), and dyskeratosis congenita (n = 1). Exome sequencing revealed heterozygous mutations in SAMD9 (n = 6) or SAMD9L (n = …

Current Practice Patterns In The Diagnosis And Management Of Sleep-Disordered Breathing In Infants., Rachana Kombathula, David G. Ingram Md, Zarmina Ehsan Md

Manuscripts, Articles, Book Chapters and Other Papers

STUDY OBJECTIVES: Currently, there are no universally accepted guidelines for diagnosis and management of sleep-disordered breathing (SDB) in infants. The purpose of this study was to survey pediatric sleep medicine providers regarding their current practice patterns for diagnosis and management of SDB in infants.

METHODS: An anonymous, web-based survey with 71 questions was distributed via the PEDSLEEP and Ped-Lung listserv, which serve as a hub of communication for pediatric sleep and pulmonary medicine providers worldwide.

RESULTS: Fifty-four providers from eight countries completed the survey. Ninety-six percent of providers reported performing sleep studies in infants with 53% performing more than 30 …

Aap To Honor Dr. Jackson With Lifetime Achievement Award, Children's Mercy Hospital

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Mary Anne Jackson, MD, FAAP, FPIDS, FIDSA, is a rarity among physicians. Her curriculum vitae reads like a virtual “Who’s Who” in pediatric infectious diseases. She’s served as an associate editor of the American Academy of Pediatrics’ Red Book, a musthave reference for pediatricians across the nation.

Plus, she’s recently added the title of Interim Dean, University of Missouri-Kansas City School of Medicine, to her long list of accomplishments. She is also a Professor of Pediatrics and Pediatric Infectious Diseases specialist at Children’s Mercy Kansas City.

The AAP Section on Infectious Diseases will present Dr. Mary Anne Jackson with a …

And The Emmy Goes To... Inside Pediatrics!, Children's Mercy Hospital

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Once again, Inside Pediatrics was awarded an Emmy for excellence in programming at this weekend's celebration of the best in television broadcasting. This is the third win in three seasons for the documentary series featuring Children's Mercy patients, families and staff. The series is now seen in six countries and also airs on Florida PBS

https://www.childrensmercy.org/in-the-community/inside-pediatrics-landing-page/

Clabsi Prevention Through Prevention Huddles And Clabsi Risk Assessment And Prevention Form In Electronic Medical Record, Tara Benton, Barb Haney, Lacey Bergerhofer, Yolanda Ballam, Kaitlyn Hoch

Posters

Our aim is to increase the awareness of high risks for central line associated bloodstream infection (CLABSI) and decrease the CLABSI rate with the implementation of CLABSI prevention huddles and an electronic medical record (EMR) "CLABSI Risk Assessment and Prevention" form.

Safe Sleep In The Nicu, Ashley Mirabile, Barb Haney, Dianne Wilderson, Beckie Palmer, Ashley Domsch, Eugenia K. Pallotto

Posters

Goal: To evaluate a staff education program on approach to improve in the rate of eligible NICU infants in safe sleep in a busy 84-bed level IV NICU.

Outcome Measures; Improve safe sleep compliance for eligible patients to 90% or greater by December 2019.

Process Measures: Audits from bedside RNs showing compliance with safe sleep.

Planned Intervention: In December of 2018 a multidisciplinary work group brainstormed and implemented best practice safe sleep education for staff and parents. September 2019 a Safe Sleep Educational Toolkit was made available for staff.

Results: Safe Sleep compliance has gone from 53% to 90% in …

Improving Care Of The Small Baby, Beckie Palmer, Abdebayo Oshodi, Brandy Huitt, Kaylee Hurt, Pamela Kliethermes, Trudy Koons, Patricia Lanzer, Ashley Mirabile, Allyson Owen, Christian Anthony Schneider, Betsi Anderson, Steven Olsen

Posters

Hierarchy of Aims:

• Global Aim: Create a Small Baby Unit for infants admitted to our NICU that arebirth, in which 90% of our patients are admitted to F pod on day of admission, by March 2019
• Interim Aim: Staff 80% of our micropremie patients with a Small Baby Team nurse from admission until >32 weeks by May 2019.
• Sub Aim: Increase rates of developmentally appropriate care (2 person cares, kangaroo care compliance) by 50% by August 2019.

Improving Malnutrition Identification In The Outpatient Setting, Kristi Thaete, Karen Stephens

Posters

Identification of Pediatric Malnutrition in Outpatient Clinics is the First Step to Allowing Registered Dietitian (RD) Involvement in the Improvement of Patient Care.

Pediatric Surgery Opioid Prescription Follow-Up, Gadison Quick, Nathaniel Aviles, Gabriel Melgarejo, Hunjung Choi, Elizabeth Edmundson

Posters

This quality improvement project is assisting general and orthopedic surgeons in gaining greater clarity when prescribing opioids for specific surgical procedures. Additional surgical services are being added to the project fall of 2019. Opioid prescribing patterns are, and will continue to be, key to all prescribing providers within our organization.

A Multifaceted Approach To Improve Quarterly Visit Rates At A Pediatric Cystic Fibrosis Care Center, Paula Capel, Jessica Banks, Micaela Mckenna, Ashley Andrews, Christopher M. Oermann

Posters

Standard quality improvement methodology was used to improve quarterly visit rates among cystic fibrosis patients at Children's Mercy Kansas City Cystic Fibrosis Center.

• A family-centered, team-based approach was adopted
• A change in culture led to sustained improvement

Improved quarterly visit rates should drive improvement in outcomes including pulmonary function and nutritional status.