Pediatrics Commons^™

An Uncommon Clinical Presentation Of Relapsing Dilated Cardiomyopathy With Identification Of Sequence Variations In Mynpc3, Kcnh2 And Mitochondrial Trna Cysteine, M. J. Guillen Sacoto, Kimberly A. Chapman, D. Heath, M. B. Seprish, Dina Zand

Pediatrics Faculty Publications

We describe a young girl with dilated cardiomyopathy, long QT syndrome, and possible energy deficiency. Two major sequence changes were identified by whole exome sequencing (WES) and mitochondrial DNA analysis that were interpreted as potentially causative. Changes were identified in the KCNH2 gene and mitochondrial tRNA for cysteine. A variation was also seen in MYPBC3. Since the launch of WES as a clinically available technology in 2010, there has been concern regarding the identification of variants unrelated to the patient's phenotype. However, in cases where targeted sequencing fails to explain the clinical presentation, the underlying etiology could be more complex …

Predictive Performance Of A Vancomycin Population Pharmacokinetic Model In Neonates, Chris Stockmann, Adam L. Hersh, Jessica K. Roberts, Jiraganya Bhongsatiern, E. Kent Korgenski, Michael G. Spigarelli, Catherine M.T. Sherwin, Adam Frymoyer

Pediatrics Faculty Publications

Introduction

The pharmacokinetics of vancomycin are highly variable among neonates, which makes dosing challenging in this population. However, adequate drug exposure is critical, especially when treating methicillin-resistant Staphylococcus aureus (MRSA) infections. Utilization of population pharmacokinetic models and Bayesian methods offers the potential for developing individualized therapeutic approaches. To meet this need, a neonatal vancomycin population pharmacokinetic model was recently published. The current study sought to externally evaluate the predictive performance and generalizability of this model.

Methods

A retrospective chart review of neonates who received vancomycin and had ≥1 peak and ≥1 trough concentrations at five Intermountain Healthcare neonatal intensive care …

The Significance Of Macrocephaly Or Enlarging Head Circumference In Infants With The Triad: Further Evidence Of Mimics Of Shaken Baby Syndrome, David Miller, Patrick Barnes, Marvin E. Miller

Pediatrics Faculty Publications

Infants with the triad (neurologic dysfunction, subdural hematoma [SDH], and retinal hemorrhage) are often diagnosed as victims of shaken baby syndrome. Medical conditions/predisposing factors to developing the triad are often dismissed: short falls, birth-related SDH that enlarges, macrocephaly, sinus/cortical vein thrombosis, and others. Six infants with the triad are described in which child abuse was diagnosed, but parents denied wrongdoing. All 6 had either macrocephaly or enlarging head circumference, which suggested medical explanations. Three infants incurred short falls, 1 had a difficult delivery in which there was likely a rebleed of a birth-related SDH, 1 had a spontaneous SDH associated …

Prophylactic Methylprednisolone To Reduce Inflammation And Improve Outcomes From One Lung Ventilation In Children: A Randomized Clinical Trial., Mary C. Theroux, Alicia Olivant Fisher, Maria E. Rodriguez, Robert P. Brislin, Kirk W. Reichard, Suken A. Shah, Matt Mccoy, Melinda Brown, Kirk W. Dabney, William G. Mackenzie, Douglas A. Katz, Thomas H. Shaffer

Department of Anesthesiology Faculty Papers

BACKGROUND: One lung ventilation (OLV) results in inflammatory and mechanical injury, leading to intraoperative and postoperative complications in children. No interventions have been studied in children to minimize such injury.

OBJECTIVE: We hypothesized that a single 2-mg·kg(-1) dose of methylprednisolone given 45-60 min prior to lung collapse would minimize injury from OLV and improve physiological stability.

METHODS: Twenty-eight children scheduled to undergo OLV were randomly assigned to receive 2 mg·kg(-1) methylprednisolone (MP) or normal saline (placebo group) prior to OLV. Anesthetic management was standardized, and data were collected for physiological stability (bronchospasm, respiratory resistance, and compliance). Plasma was assayed for …

Emergence Of Multidrug Resistant Influenza A(H1n1)Pdm09 Virus Variants In An Immunocompromised Child Treated With Oseltamivir And Zanamivir., Daisuke Tamura, Roberta L. Debiasi, Margaret Okomo-Adhiambo, Vasiliy P. Mishin, Angela P. Campbell, Brett Loechelt, Bernhard L. Wiedermann, Alicia M. Fry, Larisa V. Gubareva

Pediatrics Faculty Publications

Prolonged treatment of an immunocompromised child with oseltamivir and zanamivir for A(H1N1)pdm09 virus infection led to the emergence of viruses carrying H275Y and/or E119G in the neuraminidase. When phenotypically evaluated by neuraminidase inhibition, the dual H275Y-E119G substitution caused highly reduced inhibition by four neuraminidase inhibitors including oseltamivir, zanamivir, peramivir and laninamivir.

Pthrp-Related Hypercalcaemia In Infancy And Congenital Anomalies Of The Kidney And Urinary Tract (Cakut), Nardeen Kodous, Guido Filler, Ajay Parkash Sharma, Tamara Angela Van Hooren

Paediatrics Publications

BACKGROUND: Severe hypercalcaemia is a rare but clinically significant condition in infancy and childhood. Parathyroid hormone-related peptide (PTHrP)-mediated hypercalcaemia resulting from a malignancy is rare and only a handful of case reports have outlined its incidence alongside a benign condition.

OBJECTIVES: To describe the diagnostic workup and management of an infant with hypercalcaemia, renal dysplasia, and elevated PTHrP levels.

DESIGN: Case report.

SETTING: The Victoria Hospital campus of the London Health Sciences Centre in London, Ontario, Canada.

PATIENTS: A child with congenital anomalies of the kidneys and urinary tract (CAKUT), stage 2 chronic kidney disease (CKD), and renal dysplasia who …

Discharge Education Curriculum For Residents Rotating On An Inpatient Pediatric Ward, Kaitlin Widmer, Priti Bhansali, Julie Noffsinger

E-Learning Modules

Adult literature has demonstrated that patient preferences and understanding of discharge instructions can impact success of discharge and even predict return ER visits. There is very little data in pediatric literature describing what information should be discussed with families when providing discharge instructions, and most pediatric residents do not receive formal education on the topic. This curriculum is designed as a brief educational intervention that can improve residents’ comfort and skills in providing discharge education to families. The materials include both how to present the curriculum and tools to assess resident knowledge, behaviors and attitudes regarding discharge education as well …

Executive Function And Attention-Deficit/Hyperactivity Disorder In Ugandan Children With Perinatal Hiv Exposure, Matthew Burkey, Robert Opoka

Paediatrics and Child Health, East Africa

Background: Attention-deficit/hyperactivity disorder (ADHD) is among the most commonly diagnosed mental disorders in childhood and is associated with substantial deficits in executive functioning and lost academic and occupational attainment. This study evaluates symptoms of ADHD and their association with neurocognitive deficits in a cohort of rural Ugandan children who were born to HIV-infected mothers.

Methods: We assessed ADHD symptoms and executive function (including memory and attention) in a non-clinical sample of children born to HIV-infected mothers in rural eastern Uganda. Analyses included assessments of the psychometric properties, factor structure, and convergent and discriminant validity of the ADHD measure (ADHD-Rating Scale-IV); …

Pediapp Finder: Creating A Pediatric Application Database, Sara Pro, Adrianne Leblanc, April Wasjutin, Robert Williams

Dissertations, Masters Theses, Capstones, and Culminating Projects

It is estimated that of the 85% of Americans that own cell phones, over half of these cell phone owners use apps on their phone (Fox & Duggan, 2012; Purcell, 2011). According to the U.S. Food and Drug Administration (2013), by 2015, 500 million mobile device users will be using mobile health, or “m-Health” apps. Healthcare professionals are increasingly adopting mobile technology as an innovative, cost-efficient, and timesaving tool that may promote patient wellness and disease prevention (Kumar, 2013; mHealth Bible, 2013). Although mobile technology is a natural fit to the field of occupational therapy, research demonstrates that few resources …

Compensatory Fetal Membrane Mechanisms Between Biglycan And Decorin In Inflammation., Luciana Batalha De Miranda De Araujo, Casie E Horgan, Abraham Aron, Renato V. Iozzo, Beatrice E Lechner

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Preterm premature rupture of fetal membranes (PPROM) is associated with infection, and is one of the most common causes of preterm birth. Abnormal expression of biglycan and decorin, two extracellular matrix proteoglycans, leads to preterm birth and aberrant fetal membrane morphology and signaling in the mouse. In humans and mice, decorin dysregulation is associated with inflammation in PPROM. We therefore investigated the link between biglycan and decorin and inflammation in fetal membranes using mouse models of intraperitoneal Escherichia coli injections superimposed on genetic biglycan and decorin deficiencies. We assessed outcomes in vivo as well as in vitro using quantitative PCR, …

Management Of Pediatric Headaches In The Emergency Department At Connecticut Children’S Medical Center, Chevaughn Wellington

Honors Scholar Theses

Headaches are a common complaint for children presenting to the Emergency Department (ED) at Connecticut Children’s Medical Center (CCMC). Currently, there is little published regarding a standard treatment plan for pediatric headaches. The purpose of this study is to determine which medications are most commonly utilized and which are most effective in managing pediatric headaches. Differences in management of acute and chronic headaches in the ED at CCMC were examined. A retrospective chart review was conducted of all children who presented to the ED from January 1 to March 31, 2013 with a chief complaint or discharge diagnosis of “headache” …

Central Line-Associated Blood Stream Infections In Pediatric Intensive Care Units: Longitudinal Trends And Compliance With Bundle Strategies., Jeffrey D. Edwards, Carolyn T. Herzig, Hangsheng Liu, Monika Pogorzelska-Maziarz, Philip Zachariah, Andrew W. Dick, Lisa Saiman, Patricia W. Stone, E. Yoko Furuya

College of Nursing Faculty Papers & Presentations

BACKGROUND: Knowing the temporal trend central line-associated bloodstream infection (CLABSI) rates among U.S. pediatric intensive care units (PICUs), the current extent of central line bundle compliance, and the impact of compliance on rates is necessary to understand what has been accomplished and can be improved in CLABSI prevention.

METHODS: This is a longitudinal study of PICUs in National Healthcare Safety Network hospitals and a cross-sectional survey of directors and managers of infection prevention and control departments regarding PICU CLABSI prevention practices, including self-reported compliance with elements of central line bundles. Associations between 2011-2012 PICU CLABSI rates and infection prevention practices …

Oseltamivir-Warfarin Interaction In Hypoplastic Left Heart Syndrome: Case Report And Review., Jonathan B. Wagner, Susan M. Abdel-Rahman

Manuscripts, Articles, Book Chapters and Other Papers

An 8-year-old boy with hypoplastic left heart syndrome with a previous history of thrombosis within the inferior vena cava receiving stable warfarin dosing for anticoagulation was diagnosed with influenza B. He was subsequently placed on oseltamivir therapy according to the Centers for Disease Control and Prevention clinical practice guidelines. During the hospitalization, his international normalized ratio steadily increased to supratherapeutic levels and returned to baseline after discontinuation of oseltamivir therapy. This case represents a drug-drug interaction that has not been previously reported in children or adolescents. An extensive review of the pharmacokinetic and pharmacodynamic literature did not uncover a definitive …

The Reproducibility And Absolute Values Of Echocardiographic Measurements Of Left Ventricular Size And Function In Children Are Algorithm Dependent., Renee Margossian, Shan Chen, Lynn A. Sleeper, Lloyd Y. Tani, Girish S. Shirali, Fraser Golding, Elif Seda Selamet Tierney, Karen Altmann, Michael J. Campbell, Anita Szwast, Angela Sharkey, Elizabeth Radojewski, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Several quantification algorithms for measuring left ventricular (LV) size and function are used in clinical and research settings. The aims of this study were to investigate the effects of measurement algorithm and beat averaging on the reproducibility of measurements of the left ventricle and to assess the magnitude of agreement among the algorithms in children with dilated cardiomyopathy.

METHODS: Echocardiograms were obtained in 169 children from eight clinical centers. Inter- and intrareader reproducibility was assessed on measurements of LV volumes using the biplane Simpson, modified Simpson, and 5/6 × area × length (5/6AL) algorithms. Percentage error was calculated as …

Effect Of Clopidogrel On Platelet Cd Markers In Normal Individuals And In Patients With Untreated Type 2 Diabetes Mellitus, Muhammad Saboor, Moinuddin, Samina Ilyas

Department of Paediatrics and Child Health

The objective of this study was to evaluate the effect of clopidogrel response in patients with untreated type 2 diabetes mellitus as compared with normal individuals. One hundred and seven subjects i.e. 32 normal and 75 patients with untreated type 2 diabetes mellitus were enrolled in this study. In the first step, normal subjects as well as diabetic patients were selected and tested for various laboratory parameters and platelets flow cytometry. In the second step, an antiplatelet drug (clopidogrel) was administered for 10 days to each individual enrolled in the study. After 10 days blood samples were collected for platelets …

Comparing Functional Motor Control And Gait Parameters In Children With Autism To Those Of Age-Matched Peers Who Are Typically Developing, Patricia Stevenson, Samantha Novotny, Jillian May, Christopher Ancell

UNLV Theses, Dissertations, Professional Papers, and Capstones

The purpose of this study was to compare motor performance of children with Autism Spectrum Disorder (ASD) to that of age-matched peers who are typically developing (TD) on motor control tasks plus symmetry and variability of gait parameters across four walking conditions. A sample of convenience of children with ASD (n=6) and peers who are TD (n=6) were recruited. Motor control was assessed using initiation and completion times on the Timed Up and Go (TUG) test. Gait parameters were collected using a computerized walkway under four trial conditions: 1) walking at self-selected velocity (SSV); 2) walking during a tray-carrying task …

Use Of N-Acetylcysteine In Children With Fulminant Hepatic Failure Caused By Acute Viral Hepatitis, Ali Faisal Saleem, Qalab Abbas, Anwarul Haque

Department of Paediatrics and Child Health

Objective: To determine the efficacy of N-acetylcysteine (NAC) in children aged > 1 month to 16 years admitted with Fulminant Hepatic Failure (FHF) secondary to Acute Viral Hepatitis (AVH) in a tertiary care center of a developing country.

Study Design: Analytical study.

Place and Duration of Study: Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2007 to December 2011.

Methodology: Medical records of children (> 1 month - 16 years) with FHF admitted with AVH of known etiology who received NAC were reviewed retrospectively. Liver function tests (mean ± SD) at baseline, 24 hours after NAC and …

The Effects Of Adderall On Maternal And Fetal Health, Alyson Brady

Honors Theses

Information available for woman prescribed Adderall to treat ADHD and the effects that it may have on her fetus is limited. However, Adderall and methamphetamine have similar pharmacological and addictive qualities. This paper is concerned with whether there is an adequate approach to treatment that balances the needs of the mother and the fetus. With Adderall use becoming more prevalent, it is essential that society is aware of the physiology and pharmacology of this drug, as well as, the need for more research on this subject to be produced.

Evidence-Based Recommendations For Genetic Diagnosis Of Familial Mediterranean Fever, Gabriella Giancane, Nienke M.Ter Haar, Nico Wulffraat, Sebastiaan J. Vastert, Karyl Barron, Veronique Hentgen, Tilmann Kallinich, Huri Ozdogan, Jordi Anton, Paul Brogan, Luca Cantarini, Joost Frenkel, Caroline Galeotti, Marco Gattorno, Gilles Grateau, Michael Hofer, Isabelle Kone-Paut, Jasmin Kuemmerle-Deschner, Helen J. Lachmann, Anna Simon, Erkan Demirkaya, Brian Feldman, Yosef Uziel, Seza Ozen

Paediatrics Publications

Familial Mediterranean fever (FMF) is a disease of early onset which can lead to significant morbidity. In 2012, Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched with the aim of optimising and disseminating diagnostic and management regimens for children and young adults with rheumatic diseases. The objective was to establish recommendations for FMF focusing on provision of diagnostic tools for inexperienced clinicians particularly regarding interpretation of MEFV mutations. Evidencebased recommendations were developed using the European League against Rheumatism standard operating procedure. An expert committee of paediatric rheumatologists defined search terms for the systematic literature review. …

Certain General Anesthetics Used In Pediatrics Hinder Neurological Development In Infants, Michael R. Hirschi

Selected Honors Theses

General anesthetics act by either blocking N-methyl-d-aspartate (NMDA) receptors or over stimulating γ-aminobutyric acid (GABA) receptors.^1–3 The actions of these receptors are responsible for the anesthetized state and are also crucial in the neurological development of infants.^2,4–8 Animal studies, although limited, provide vital information about general anesthesia’s neurotoxicity its hindrance of neurological development. Exposure to general anesthesia can severely hinder proper neuronal migration, synaptogenesis, and can drastically increase neuronal apoptosis in infant animals.^9–16 General anesthetics are more neurotoxic to infant animals in combination compared to individually.^10,16,17 Additionally, multiple exposures to general anesthesia tend to have compounding …