Pediatrics Commons^™

Lower Red Cell Transfusion Rates With Use Of Tranexamic Acid In Single-Stage Bilateral Total Knee Arthroplasty: A Retrospective Audit, Muhammad Aziz, Salman Bin Mahmood,, Riaz Hussain Lakdawala, Tashfeen Ahmad

Department of Surgery Docs

Abstract

Bilateral total knee arthroplasty (BTKA) patients may require blood transfusion which has its risks. Anti-fibrinolytic drugs such as aprotinin, aminocaproic acid and tranexamic acid (TXA) have reduced transfusion requirements in major surgery. This retrospective audit was performed to assess effectiveness of TXA in reducing blood transfusion rate in single-stage sequential BTKA cases operated by a single surgeon. Records of 91 patients given TXA and 80 controls who were operated before 2012 and not given TXA were reviewed. TXA was given 15mg/kg intravenously (IV) before tourniquet deflation and 3 hours postoperatively.Blood transfusion was done in 9(10%) patients in the TXA …

Recent Strategies To Improve Community Case Management Of Diarrhea Among Children Under Five In Developing Countries, Fauziah Rabbani, Aysha Zahidie

Community Health Sciences

No abstract provided.

Health Care Provider Knowledge And Routine Management Of Pre-Eclampsia In Pakistan, Sana Sheikh, Rahat Najam Qureshi, Asif Raza Khowaja, Rehana Salam, Marianne Vidler, Diane Sawchuck, Peter Von Dadelszen, Shujat Zaidi, Zulfiqar Bhutta

Department of Paediatrics and Child Health

Background: Maternal mortality ratio is 276 per 100,000 live births in Pakistan. Eclampsia is responsible for one in every ten maternal deaths despite the fact that management of this disease is inexpensive and has been available for decades. Many studies have shown that health care providers in low and middle-income countries have limited training to manage patients with eclampsia. Hence, we aimed to explore the knowledge of different cadres of health care providers regarding aetiology, diagnosis and treatment of pre-eclampsia and eclampsia and current management practices.
Methods: We conducted a mixed method study in the districts of Hyderabad and Matiari …

Potential For Task-Sharing To Lady Health Workers For Identification And Emergency Management Of Pre-Eclampsia At Community Level In Pakistan, Rehana A. Salam, Rahat Najam Qureshi, Sana Sheikh, Asif Raza Khowaja, Diane Sawchuck, Marianne Vidler, Peter Von Dadelszen, Shujaat Zaidi, Zulfiqar Bhutta

Department of Paediatrics and Child Health

Background: An estimated 276 Pakistani women die for every 100,000 live births; with eclampsia accounting for about 10% of these deaths. Community health workers contribute to the existing health system in Pakistan under the banner of the Lady Health Worker (LHW) Programme and are responsible to provide a comprehensive package of antenatal services. However, there is a need to increase focus on early identification and prompt diagnosis of pre-eclampsia in community settings, since women with mild pre-eclampsia often present without symptoms. This study aims to explore the potential for task-sharing to LHWs for the community-level management of pre-eclampsia and eclampsia …

Glycemic Variability Predicts Inflammation In Adolescents With Type 1 Diabetes, Robert P. Hoffman, Amanda S. Dye, Hong Huang, John A. Bauer

Pediatrics Faculty Publications

Background: Adolescents with type 1 diabetes (T1D) have increased risk of cardiovascular disease as well as elevations in biomarkers of systemic inflammation, plasma protein oxidation and vascular endothelial injury. It is unclear whether hyperglycemia itself, or variations in blood glucose are predictors of these abnormalities.

Methods: This study was designed to determine the relationship of inflammatory (C-reactive protein, CRP), oxidative (total anti-oxidative capacity, TAOC) and endothelial injury (soluble intracellular adhesion molecule 1, sICAM1) markers to glycemic control measures from 3 days of continuous glucose monitoring (CGM) and to hemoglobin A_1c (HbA_1c), and HbA_1c×duration area under …

A Novel Adaptation Of A Parent-Child Observational Assessment Tool For Appraisals And Coping In Children Exposed To Acute Trauma, Meghan L. Marsac, Nancy Kassam-Adams

Pediatrics Faculty Publications

Background: Millions of children worldwide are exposed to acute potentially traumatic events (PTEs) annually. Many children and their families experience significant emotional distress and/or functional impairment following PTEs. While current research has begun to highlight a role for early appraisals and coping in promoting or preventing full recovery from PTEs, the exact nature of the relationships among appraisals, coping, and traumatic stress reactions as well as how appraisals and coping behaviors are influenced by the child's environment (e.g., parents) remains unclear; assessment tools that reach beyond self-report are needed to improve this understanding.

Objective: The objective of the current study …

Decreasing Patient Cost And Travel Time Through Pediatric Rheumatology Telemedicine Visits., Elizabeth A. Kessler, Ashley K. Sherman, Mara L. Becker

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: There is a critical shortage of pediatric rheumatologists in the US. Substantial travel to clinics can impose time and monetary burdens on families. The aim of this study was to evaluate the cost of in-person pediatric rheumatology visits for families and determine if telemedicine clinics resulted in time and cost savings. Factors associated with interest in telemedicine were also explored.

METHODS: Surveys were offered to parents and guardians of patients in Pediatric Rheumatology follow-up clinics in Kansas City, Missouri, the primary site of in-person care, and at a telemedicine outreach site 160 miles away, in Joplin, Missouri. Survey questions …

Airway Secretory Micrornaome Changes During Rhinovirus Infection In Early Childhood, Maria J. Gutierrez, Jose L. Gomez, Geovanny F. Perez, Krishna Pancham, Stephanie Val, Dinesh K. Pillai, Mamta Giri, Sarah Ferrante, Robert Freishtat, Mary C. Rose, Diego Preciado, Gustavo Nino

Pediatrics Faculty Publications

Background Innate immune responses are fine-tuned by small noncoding RNA molecules termed microRNAs (miRs) that modify gene expression in response to the environment. During acute infections, miRs can be secreted in extracellular vesicles (EV) to facilitate cell-to-cell genetic communication. The purpose of this study was to characterize the baseline population of miRs secreted in EVs in the airways of young children (airway secretory microRNAome) and examine the changes during rhinovirus (RV) infection, the most common cause of asthma exacerbations and the most important early risk factor for the development of asthma beyond childhood.

Methods Nasal airway secretions were obtained from …

Prevalence Of Stroke In Children Admitted With Sickle Cell Anaemia To Mulago Hospital, Samden Lhatoo, Martha Sajatovic

Faculty Scholarship

Background: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. Methods: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in …

Biallelic Hypomorphic Mutations In A Linear Deubiquitinase Define Otulipenia, An Early-Onset Autoinflammatory Disease, Qing Zhou, Xiaomin Yu, Erkan Demirkaya, Natalie Deuitch, Deborah Stone, Wanxia Li Tsai, Hye Sun Kuehn, Hongying Wang, Dan Yang, Yong Hwan Park, Amanda K. Ombrello, Mary Blake, Tina Romeo, Elaine F. Remmers, Jae Jin Chae, James C. Mullikin, Ferhat Güzel, Joshua D. Milner, Manfred Boehm, Sergio D. Rosenzweig, Massimo Gadina, Steven B. Welch, Seza Özen, Rezan Topaloglu, Mario Abinun, Daniel L. Kastner, Ivona Aksentijevich

Paediatrics Publications

Systemic autoinflammatory diseases are caused by mutations in genes that function in innate immunity. Here, we report an autoinflammatory disease caused by loss-of-function mutations in OTULIN (FAM105B), encoding a deubiquitinase with linear linkage specificity. We identified two missense and one frameshift mutations in one Pakistani and two Turkish families with four affected patients. Patients presented with neonatal-onset fever, neutrophilic dermatitis/panniculitis, and failure to thrive, but without obvious primary immunodeficiency. HEK293 cells transfected with mutated OTULIN had decreased enzyme activity relative to cells transfected with WT OTULIN, and showed a substantial defect in the linear deubiquitination of target molecules. Stimulated patients' …

An Interactive Videogame Designed To Improve Respiratory Navigator Efficiency In Children Undergoing Cardiovascular Magnetic Resonance, Sean M. Hamlet, Christopher M. Haggerty, Jonathan D. Suever, Gregory J. Wehner, Jonathan D. Grabau, Kristin N. Andres, Moriel H. Vandsburger, David K. Powell, Vincent L. Sorrell, Brandon K. Fornwalt

Saha Cardiovascular Research Center Faculty Publications

Background: Advanced cardiovascular magnetic resonance (CMR) acquisitions often require long scan durations that necessitate respiratory navigator gating. The tradeoff of navigator gating is reduced scan efficiency, particularly when the patient’s breathing patterns are inconsistent, as is commonly seen in children. We hypothesized that engaging pediatric participants with a navigator-controlled videogame to help control breathing patterns would improve navigator efficiency and maintain image quality.

Methods: We developed custom software that processed the Siemens respiratory navigator image in real-time during CMR and represented diaphragm position using a cartoon avatar, which was projected to the participant in the scanner as visual feedback. The …

Airway Secretory Micrornaome Changes During Rhinovirus Infection In Early Childhood., M. Gutierrez, J. Gomez, Geovanny Perez, Krishna Pancham, Dinesh K. Pillai, M. Giri, S. Ferrante, Robert J. Freishtat, Mary C. Rose, Diego A. Preciado, Gustavo Nino

Pediatrics Faculty Publications

BACKGROUND:

Innate immune responses are fine-tuned by small noncoding RNA molecules termed microRNAs (miRs) that modify gene expression in response to the environment. During acute infections, miRs can be secreted in extracellular vesicles (EV) to facilitate cell-to-cell genetic communication. The purpose of this study was to characterize the baseline population of miRs secreted in EVs in the airways of young children (airway secretory microRNAome) and examine the changes during rhinovirus (RV) infection, the most common cause of asthma exacerbations and the most important early risk factor for the development of asthma beyond childhood.

METHODS:

Nasal airway secretions were obtained from …

221 Newborn-Screened Neonates With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency: Findings From The Inborn Errors Of Metabolism Collaborative, K Bentler, S Zhai, S Elsbecker, G Arnold, B Burton, J Vockley, C Cameron, S Hiner, M Edick, S Berry, J Thomas, M Dodge, R Singh, S Lakshman, David Kronn, Inborn Errors Of Metabolism Collaborative

NYMC Faculty Publications

Introduction: There is limited understanding of relationships between genotype, phenotype and other conditions contributing to health in neonates with medium-chain acyl-coenzyme A dehydrogenase deficiency (MCADD) identified through newborn screening. Methods: Retrospective analysis of comprehensive data from a cohort of 221 newborn-screened subjects identified as affected with MCADD in the Inborn Errors of Metabolism-Information System (IBEM-IS), a long term follow-up database of the Inborn Errors of Metabolism Collaborative, was performed. Results: The average age at notification of first newborn screen results to primary care or metabolic providers was 7.45 days. The average octanoylcamitine (C8) value on first newborn screen was 11.2 …

Survival Of High-Risk Pediatric Neuroblastoma Patients In A Developing Country, Joseph C. Easton, Sergio Gomez, Peter H. Asdahl, J. Michael Conner, Alcira B. Flynn, Claudia Ruiz, Rohit P. Ojha

Faculty Publications

Little information is available about survival of high‐risk pediatric neuroblastoma patients in developing countries. We aimed to assess survival among high‐risk pediatric neuroblastoma patients in La Plata, Argentina. Individuals eligible for our cohort were aged4 yr at diagnosis, 54% were male, and 62% had adrenal neuroblastoma. We observed 18 deaths, and the median survival time of our study population was 1.7 yr. The five‐yr overall survival probability was 24% (95% CL: 10%, 41%). In contrast, five‐yr survival of high‐risk neuroblastoma patients ranges between 23% and 76% in developed countries. Survival among high‐risk neuroblastoma patients is generally poor regardless of geographic …

Countdown To 2015 Country Case Studies: What Can Analysis Of National Health Financing Contribute To Understanding Mdg 4 And 5 Progress?, Carlyn Mann, Courtney Ng, Nadia Akseer, Zulfiqar Ahmed Bhutta, Josephine Borghi, Tim Colbourn, Patricia Hernández-Peña, Luis Huicho, Muhammad Ashar Malik, Melisa Ashar Martinez-Alvarez, Spy Munthali, Ahmad Shah Salehi, Mekonnen Tadesse, Mohammed Yassin, Peter Berman

Community Health Sciences

Background

Countdown to 2015 (Countdown) supported countries to produce case studies that examine how and why progress was made toward the Millennium Development Goals (MDGs) 4 and 5. Analysing how health-financing data explains improvements in RMNCH outcomes was one of the components to the case studies.

Methods

This paper presents a descriptive analysis on health financing from six Countdown case studies (Afghanistan, Ethiopia, Malawi, Pakistan, Peru, and Tanzania), supplemented by additional data from global databases and country reports on macroeconomic, health financing, demographic, and RMNCH outcome data as needed. It also examines the effect of other contextual factors presented in …

Autism Screening In Primary Care: Community Providers Incorrectly Report Adherence To Aap Autism Screening Guidelines, Jose Lopez-Lizarranga Md, Kimberly J. Reid, Sarah Nyp, Cy Nadler

Posters

Purpose: To compare community provider reports of adherence to current American Academy of Pediatrics (AAP) autism screening guidelines with their self-reported practices, including implementation of the Modified Checklist for Autism in Toddlers (M-CHAT).

Methods: An online survey was distributed to primary care providers from Kansas, Oklahoma, and North Dakota. The survey collected demographics and self-reported ASD screening practices (including M-CHAT implementation procedures) and knowledge questions.

Results: 133 providers completed the survey (50% pediatricians, 32% int. med/fam. physicians, 18% physician assistants). Of the 96 who reported routinely screening for ASD; 78% reported adhering to AAP guidelines. However, no providers correctly answered …

Improving Human Papillomavirus Immunization Rates In Kentucky, Nneka S. Udechukwu, Paul Duffin, Alissa Briggs, Hatim A. Omar

Pediatrics Faculty Publications

No abstract provided.

Pediatric Statin Administration: Navigating A Frontier With Limited Data., Jonathan B. Wagner, Susan M. Abdel-Rahman

Manuscripts, Articles, Book Chapters and Other Papers

Increasingly, children and adolescents with dyslipidemia qualify for pharmacologic intervention. As they are for adults, 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase inhibitors (statins) are the mainstay of pediatric dyslipidemia treatment when lifestyle modifications have failed. Despite the overall success of these drugs, the magnitude of variability in dose-exposure-response profiles contributes to adverse events and treatment failure. In children, the cause of treatment failures remains unclear. This review describes the updated guidelines for screening and management of pediatric dyslipidemia and statin disposition pathway to assist the provider in recognizing scenarios where alterations in dosage may be warranted to meet patients' specific needs.

A Hypothesis For Using Pathway Genetic Load Analysis For Understanding Complex Outcomes In Bilirubin Encephalopathy, Sean M. Riordan, Douglas C. Bittel, Jean-Baptist Lepichon, Silvia Gazzin, Claudio Tiribelli, Jon F. Watchko, Richard P. Wennberg, Steven Shapiro

Manuscripts, Articles, Book Chapters and Other Papers

© 2016 Riordan, Bittel, Le Pichon, Gazzin, Tiribelli, Watchko, Wennberg and Shapiro.

Genetic-based susceptibility to bilirubin neurotoxicity and chronic bilirubin encephalopathy (kernicterus) is still poorly understood. Neonatal jaundice affects 60-80% of newborns, and considerable effort goes into preventing this relatively benign condition from escalating into the development of kernicterus making the incidence of this potentially devastating condition very rare in more developed countries. The current understanding of the genetic background of kernicterus is largely comprised of mutations related to alterations of bilirubin production, elimination, or both. Less is known about mutations that may predispose or protect against CNS bilirubin neurotoxicity. …

The Airway Microbiome At Birth., Charitharth Vivek Lal, Colm Travers, Zubair H. Aghai, Peter Eipers, Tamas Jilling, Brian Halloran, Waldemar A. Carlo, Jordan Keeley, Gabriel Rezonzew, Ranjit Kumar, Casey Morrow, Vineet Bhandari, Namasivayam Ambalavanan

Department of Pediatrics Faculty Papers

Alterations of pulmonary microbiome have been recognized in multiple respiratory disorders. It is critically important to ascertain if an airway microbiome exists at birth and if so, whether it is associated with subsequent lung disease. We found an established diverse and similar airway microbiome at birth in both preterm and term infants, which was more diverse and different from that of older preterm infants with established chronic lung disease (bronchopulmonary dysplasia). Consistent temporal dysbiotic changes in the airway microbiome were seen from birth to the development of bronchopulmonary dysplasia in extremely preterm infants. Genus Lactobacillus was decreased at birth in …