Pediatrics Commons^™

A Case Of Worsening Stridor In A Neonate, Richard J. Mazzaccaro Md

Department of Pediatrics

No abstract provided.

Mothers’S Education And Working Status; Do They Contribute To Corrosive Poisoning Among Paediatric Patients Of Karachi, Pakistan?, Emaduddin Siddiqui, Kiran Ejaz, Sayyeda Ghazala Irfan Kazi, Saif Siddiqui, Syed Jamal Raza

Department of Emergency Medicine

Objectives: To determine the causative factors behind corrosive poisoning in children like effect of working mothers, their educational status, financial background, family size and number of siblings.

Methods: The multi-centre, prospective, case series of all paediatric patients presenting to the Emergency Department of the National Institute of Child Health and the Aga Khan University Hospital, Karachi from August 2008 to July 2009 is presented. It comprised all paediatric patients with a history of corrosive poisoning at the two hospitals. SPSS 20 was used for statistical analysis.

Results: Out of 105 cases, 56(53%) related to the private-sector Aga Khan University Hospital, …

Pediatric Palliative Care For Youth With Hiv/Aids: Systematic Review Of The Literature, Megan L. Wilkins, Ronald H. Dallas, Kathleen E. Fanone, Maureen E. Lyon

Pediatrics Faculty Publications

Improvement in treatment has led to decreased death in youth with human immunodeficiency virus (HIV) in developed countries. Despite this, youth with HIV are still at risk for increased mortality and morbidity compared with their uninfected counterparts. In developing countries, high numbers of youth die from acquired immune deficiency syndrome (AIDS)-related illnesses due to lack of access to consistent antiretroviral treatment. As a result, pediatric palliative care is a relevant topic for those providing care to youth with HIV. A systematic review was conducted to gather information regarding the status of the literature related to pediatric palliative care and medical …

Crystal Structure Of The N-Acetyltransferase Domain Of Human N-Acetyl-L-Glutamate Synthase In Complex With N-Acetyl-L-Glutamate Provides Insights Into Its Catalytic And Regulatory Mechanisms, Gengxiang Zhao, Zhongmin Jin, Norma M. Allewell, Mendel Tuchman, Dashuang Shi

Pediatrics Faculty Publications

N-acetylglutamate synthase (NAGS) catalyzes the conversion of AcCoA and L-glutamate to CoA and N-acetyl-L-glutamate (NAG), an obligate cofactor for carbamyl phosphate synthetase I (CPSI) in the urea cycle. NAGS deficiency results in elevated levels of plasma ammonia which is neurotoxic. We report herein the first crystal structure of human NAGS, that of the catalyticN-acetyltransferase (hNAT) domain with N-acetyl-L-glutamate bound at 2.1 Å resolution. Functional studies indicate that the hNAT domain retains catalytic activity in the absence of the amino acid kinase (AAK) domain. Instead, the major functions of the AAK domain appear to be providing …

A Synthetic Model Of Human Beta-Thalassemia Erythropoiesis Using Cd34+ Cells From Healthy Adult Donors, Y. Terry Lee, Ki-Soon Kim, Colleen Byrnes, Jaira F. De Vasconcellos, Seung-Jae Noh, Antoinette Rabel, Emily Riehm Meier, Jeffrey L. Meier

Pediatrics Faculty Publications

Based upon the lack of clinical samples available for research in many laboratories worldwide, a significant gap exists between basic and clinical studies of beta-thalassemia major. To bridge this gap, we developed an artificially engineered model for human beta thalassemia by knocking down beta-globin gene and protein expression in cultured CD34+ cells obtained from healthy adults. Lentiviral-mediated transduction of beta-globin shRNA (beta-KD) caused imbalanced globin chain production. Beta-globin mRNA was reduced by 90% compared to controls, while alpha-globin mRNA levels were maintained. HPLC analyses revealed a 96% reduction in HbA with only a minor increase in HbF. During the terminal …

Is Adrenalectomy Necessary During Unilateral Nephrectomy For Wilms Tumor? A Report From The Children's Oncology Group., Kathleen Kieran, James R Anderson, Jeffrey S Dome, Peter F Ehrlich, Michael L Ritchey, Robert C Shamberger, Elizabeth J Perlman, Daniel M Green, Andrew M Davidoff

Pediatrics Faculty Publications

PURPOSE: To determine whether performing adrenalectomy at the time of nephrectomy for unilateral Wilms tumor impacts clinical outcome.

METHODS: We reviewed information on all patients enrolled on National Wilms Tumor Study-4 and -5. Data were abstracted on patient demographics, tumor characteristics, surgical and pathologic status of the adrenal gland, and patient outcomes. The primary endpoints were intraoperative spill and five-year event-free survival (EFS) in patients who did or did not undergo adrenalectomy.

RESULTS: Of 3825 patients with complete evaluable data, the adrenal was left in situ in 2264 (57.9%) patients, and was removed completely in 1367 patients (36.7%) or partially …

Effect Of A Voice Recognition System On Paediatric Outpatient Medication Errors, Angela Nyangore Migowa

Master of Medicine in Paediatrics and Child Health

Background: Medication errors have potential to cause harm and death; especially children who are three times more vulnerable than adults. Risk of medication errors is higher in out- patient settings due to a stressful work environment with less familiarity of individual patients. This problem in sub-Saharan Africa is however largely undetermined. A Voice Recognition System that converts verbal messages into text and stores it in a database in a retrievable format could impact on reduction of medication errors. Objectives: The primary objective was to compare medication prescription and dispensing errors in written prescriptions with those from a Voice Recognition System. …

Effectiveness Of Pentavalent And Monovalent Rotavirus Vaccines In Concurrent Use Among Us Children <5 Years Of Age, 2009-2011, Daniel C. Payne, Julie A. Boom, Mary Allen Staat, Kathryn M. Edwards, Peter G. Szilagyi, Eileen J. Klein, Rangaraj Selvarangan, Parvin H. Azimi, Christopher J. Harrison, Mary Moffatt, Samantha H. Johnston, Leila C. Sahni, Carol J, Baker, Marcia A. Rench, Stephanie Donauer, Monica Mcneal, James Chappell, Geoffrey A. Weinberg, Azadeh Tasslimi, Jacqueline E. Tate, Mary Wikswo, Aaron T. Curns, Iddrisu Sulemana, Slavica Mijatovic-Rustempasic, Mathew D. Esona, Michael D. Bowen, Jon R. Gentsch, Umesh D. Parashar

Manuscripts, Articles, Book Chapters and Other Papers

Background We assessed vaccine effectiveness (VE) for RotaTeq (RV5; 3 doses) and Rotarix (RV1; 2 doses) at reducing rotavirus acute gastroenteritis (AGE) inpatient and emergency department (ED) visits in US children.

Methods We enrolled children vaccine, age, ethnicity, genotype, and clinical setting.

Results RV5-specific analyses included 359 rotavirus cases and 1811 rotavirus-negative AGE controls. RV1-specific analyses included 60 rotavirus cases and 155 rotavirus-negative AGE controls. RV5 and RV1 were 84% (95% confidence interval [CI], 78%-88%) and 70% (95% CI, 39%-86%) effective, respectively, against rotavirus-associated ED visits and hospitalizations combined. By clinical setting, RV5 VE against ED and inpatient rotavirus-associated visits …

Β-Estradiol-Dependent Activation Of The Jak/Stat Pathway Requires P/Cip And Carm1, N. Coughlan, G. Thillainadesan, J. Andrews, M. Isovic, J. Torchia

Paediatrics Publications

The steroid receptor coactivator p/CIP, also known as SRC-3, is an oncogene commonly amplified in breast and ovarian cancers. p/CIP is known to associate with coactivator arginine methyltransferase 1 (CARM1) on select estrogen responsive genes. We have shown, using a ChIP-on-chip approach, that in response to stimulation with 17β-estradiol (E2), the p/CIP/CARM1 complex is recruited to 204 proximal promoters in MCF-7 cells. Many of the complex target genes have been previously implicated in signaling pathways related to oncogenesis. Jak2, a member of the Jak/Stat signaling cascade, is one of the direct E2-dependent targets of the p/CIP/CARM1 complex. Following E2-treatment, histone …

Maternal Taurine Supplementation In Rats Partially Prevents The Adverse Effects Of Early-Life Protein Deprivation On Β-Cell Function And Insulin Sensitivity, Christine Tang, Kelly Marchand, Loretta Lam, Victoria Lux-Lantos, Sandra M. Thyssen, June Guo, Adria Giacca, Edith Arany

Paediatrics Publications

Dietary protein restriction during pregnancy and lactation in rats impairs β-cell function and mass in neonates and leads to glucose intolerance in adult offspring. Maternal taurine (Tau) supplementation during pregnancy in rats restores β-cell function and mass in neonates, but its long-term effects are unclear. The prevention of postnatal catch-up growth has been suggested to improve glucose tolerance in adult offspring of low-protein (LP)-fed mothers. The objective of this study was to examine the relative contribution of β-cell dysfunction and insulin resistance to impaired glucose tolerance in 130-day-old rat offspring of LP-fed mothers and the effects of maternal Tau supplementation …

Narrative Medicine As A Means Of Training Medical Students Toward Residency Competencies, Shannon L. Arntfield, Kristen Slesar, Jennifer Dickson, Rita Charon

Paediatrics Publications

Objective: This study sought to explore the perceived influence of narrative medicine training on clinical skill development of fourth-year medical students, focusing on competencies mandated by ACGME and the RCPSC in areas of communication, collaboration, and professionalism. Methods: Using grounded-theory, three methods of data collection were used to query twelve medical students participating in a one-month narrative medicine elective regarding the process of training and the influence on clinical skills. Iterative thematic analysis and data triangulation occurred. Results: Response rate was 91% (survey), 50% (focus group) and 25% (follow-up). Five major findings emerged. Students perceive that they: develop and improve …

Expression Of Ski Can Act As A Negative Feedback Mechanism On Retinoic Acid Signaling, Meaghan A. Melling, Charlotte R.C. Friendship, Trevor G. Shepherd, Thomas A. Drysdale

Paediatrics Publications

Background: Retinoic acid signaling is essential for many aspects of early development in vertebrates. To control the levels of signaling, several retinoic acid target genes have been identified that act to suppress retinoic acid signaling in a negative feedback loop. The nuclear protein Ski has been extensively studied for its ability to suppress transforming growth factor-beta (TGF-β) signaling but has also been implicated in the repression of retinoic acid signaling. Results: We demonstrate that ski expression is up-regulated in response to retinoic acid in both early Xenopus embryos and in human cell lines. Blocking retinoic acid signaling using a retinoic …

How Do Symbolic And Non-Symbolic Numerical Magnitude Processing Skills Relate To Individual Differences In Children's Mathematical Skills? A Review Of Evidence From Brain And Behavior, Bert De Smedt, Marie-Pascale Noël, Camilla Gilmore, Daniel Ansari

Paediatrics Publications

Many studies have tested the association between number magnitude processing and mathematics achievement. However, results appear to be quite different depending on the number format used. When using symbolic numbers (digits), data consistent and robust across studies and populations have been found, with weaker performance associated with weak math achievement and dyscalculia. However, when using non-symbolic format (dots), many conflicting findings are reported. These inconsistencies might be explained by methodological issues. Alternatively, it might be that the processes measured by non-symbolic tasks are not particularly critical for school-relevant mathematics. A few neuroimaging studies have also shown the brain signature of …

Standardized Pediatric Vancomycin Dosing And Influence On Target Concentrations, Tibisay Villalobos Md, Jenny Boucher Pharmd, Jd, Bcps

Department of Pediatrics

No abstract provided.

Botulism In Children: A Diagnostic Dilemma In Developing Countries, Muhammad Rehan Khan, Prem Kumar, Shahnaz Shahid, Anwarul Haque

Department of Paediatrics and Child Health

Botulism is a well-known disease of the neuromuscular junction. It is a rare but curable cause of paralysis in paediatric population. In addition to classical clinical signs and symptoms, the diagnosis of botulism requires laboratory confirmation of intoxication by various biological tests. These include demonstration of botulinum toxin in serum or isolation of the Clostridium botulinum from stool/gastric aspirates. However, it is not always possible to confirm intoxication due to unavailability of technical facilities, especially in resource limited countries like Pakistan. Under these circumstances, electrophysiological studies serve as an excellent diagnostic tool. These studies can provide quick diagnosis of botulism …

Uv Radiation And The Skin, John A. D'Orazio, Stuart G. Jarrett, Alexandra Amaro-Ortiz, Timothy Scott

Toxicology and Cancer Biology Faculty Publications

UV radiation (UV) is classified as a "complete carcinogen" because it is both a mutagen and a non-specific damaging agent and has properties of both a tumor initiator and a tumor promoter. In environmental abundance, UV is the most important modifiable risk factor for skin cancer and many other environmentally-influenced skin disorders. However, UV also benefits human health by mediating natural synthesis of vitamin D and endorphins in the skin, therefore UV has complex and mixed effects on human health. Nonetheless, excessive exposure to UV carries profound health risks, including atrophy, pigmentary changes, wrinkling and malignancy. UV is epidemiologically and …

"Mercy Tape" Unveiled: Provides Better Weight Estimates For Children, Bill Van Kirk

Our Story Continues

Susan Abdel-Rahman, Pharm.D, Clinical Pharmacology and Medical Toxicology, led the Children's Mercy research team that developed the Mercy TAPE, a new device to estimate the weight of pediatric patients ages two months to 16 years.

Accurate weight estimates in resource-restricted settings (lacking the means to weigh a child) are important because they are used to determine appropriate medication dosage, fluid volumes for resuscitation, breathing tube sizes, and more.

National Estimates Of Emergency Department Visits For Pediatric Severe Sepsis In The United States, Sara Singhal, Mathias W. Allen, John-Ryan Mcannally, Kenneth S. Smith, John P. Donnelly, Henry E. Wang

Emergency Medicine Faculty Publications

Objective. We sought to determine the characteristics of children presenting to United States (US) Emergency Departments (ED) with severe sepsis.

Study design. Cross-sectional analysis using data from the National Hospital Ambulatory Medical Care Survey (NHAMCS). Using triage vital signs and ED diagnoses (defined by the International Classification of Diseases, Ninth Revision codes), we identified children(triage fever or ICD-9 infection) and organ dysfunction (triage hypotension or ICD-9 organ dysfunction).

Results. Of 28.2 million pediatric patients presenting to US EDs each year, severe sepsis was present in 95,055 (0.34%; 95% CI: 0.29-0.39%). Fever and respiratory infection were the most common indicators of …

A Quality Improvement Investigation Of Pediatric Asthma Services For Patients And Their Families, Tina Dimartino

Graduate Student Independent Studies

This quality improvement project sought to identify attributes of pediatric patients with asthma and their caregivers at a hospital serving pediatric patients and families in Bronx, NY. Anonymous, self-report surveys focused on rates of asthma inpatient admission and emergency department visits, general understanding of asthma, current services utilized in the hospital and interest in the potential expansion of existing services. Additionally, the project explored the prospective development of the current educational healthcare video system within the hospital.

Late Effects Of Childhood Cancer : Implications For Child Life Specialists, Meghan Brewer

Graduate Student Independent Studies

No abstract provided.

Providing Family-Centered Care For Families And Patients With Autism Spectrum Disorders : A Guide For Healthcare Professionals, Kacie Wilson

Graduate Student Independent Studies

The purpose of this thesis is to provide healthcare professionals in the pediatric healthcare setting with the necessary tools to provide high quality family-centered care to children with ASD and to their families.

Creating An Optimal Healing Environment For Pediatric Patients And Families : A Medical School Course On Child Life Theory And Practice, Erin Halman

Graduate Student Independent Studies

This project outlines an introductory child life course intended for medical students. The course covers salient topics from a graduate level child life curriculum, as well as skills identified as the most useful for physicians in a survey of certified child life specialists.

A Trip To The Epilepsy Monitoring Unit : Using Video Modeling To Prepare Children And Caregivers For Inpatient Eeg Monitoring, Peyton Katz

Graduate Student Independent Studies

The goal of this project was to outline the benefits of utilizing video modeling in the preparation of children and families within a child life department's preparation program.

From Qualitative Work To Intervention Development In Pediatric Oncology Palliative Care Research, Terrah Foster Akard, Mary Jo Gilmer, Debra L. Friedman, Barbara Given, Verna L. Hendricks-Ferguson, Pamela S. Hinds

Pediatrics Faculty Publications

Qualitative methods can be particularly useful approaches to use with individuals who are experiencing a rare disease and thus who comprise a small sample (such as children with cancer) and are at points in care that few experience (such as end of life). This data-based methods article describes how findings from a qualitative study were used to guide and shape a pediatric oncology palliative care intervention. Qualitative data can lay a strong foundation for subsequent pilot intervention work by facilitating the development of an underlying study conceptualization, providing recruitment feasibility estimates, helping establish clinically meaningful inclusion criteria, establishing staff acceptability …

Characteristics Of Children With The Best And Poorest First- And Second-Year Growth During Rhgh Therapy: Data From 25 Years Of The Genentech National Cooperative Growth Study (Ncgs), Paul B. Kaplowitz, Dorothy I. Shulman, James W. Frane, Joan Jacobs, Barbara Lippe

Pediatrics Faculty Publications

Background

Models assessing characteristics contributing to response to recombinant human growth hormone (rhGH) response rarely address growth extremes in both years 1 and 2 or examine how children track from year to year. Using National Cooperative Growth Study (NCGS) data, we determined characteristics contributing to responsiveness to rhGH and the pattern of change from years 1 to 2.

Patients and methods

Height velocity standard deviation score (HV SDS) for 2 years for prepubertal children with idiopathic GH deficiency (IGHD) (n = 1899) and idiopathic short stature (ISS) (n = 1186) treated with similar doses for two years were computed. Group …

The Function Of The Left Angular Gyrus In Mental Arithmetic: Evidence From The Associative Confusion Effect, Roland H. Grabner, Daniel Ansari, Karl Koschutnig, Gernot Reishofer, Franz Ebner

Paediatrics Publications

While the left angular gyrus (lAG) has been repeatedly implicated in mental arithmetic, its precise functional role has not been established. On the one hand, it has been speculated that the lAG is involved in task-specific processes. On the other hand, the observation of relative deactivation during arithmetic has led to the contention that differential lAG activation reflects task-unrelated difficulty effects associated with the default mode network (DMN). Using functional magnetic resonance imaging, we investigated the neural correlates of the associative confusion effect that allowed us to dissociate effects of task difficulty and task-related arithmetic processes on lAG activation. The …

Umbilical Uptakes And Transplacental Concentration Ratios Of Amino Acids In Severe Fetal Growth Restriction, Timothy R.H. Regnault, Barbra De Vrijer, Henry L. Galan, Randall B. Wilkening, Frederick C. Battaglia, Giacomo Meschia

Paediatrics Publications

Background: This study examines the relationship between placental amino acid (AA) transport and fetal AA demand in an ovine fetal growth restriction (FGR) model in which placental underdevelopment induces fetal hypoxemia and hypoglycemia. Methods: Umbilical uptakes of AA, oxygen, glucose, and lactate were measured near term in eight experimental ewes (FGR group) and in eight controls (C group). Results: The FGR group demonstrated significantly reduced umbilical uptakes of oxygen, glucose, lactate, and 11 AAs per kg fetus. The combined uptake of glucose, lactate, and AAs, expressed as nutrient/oxygen quotients, was reduced almost to 1.00 (FGR: 1.05 vs. C: 1.32, P …

Molecular Mechanisms Underlying Rb Protein Function, Frederick A. Dick, Seth M. Rubin

Paediatrics Publications

Inactivation of the RB protein is one of the most fundamental events in cancer. Coming to a molecular understanding of its function in normal cells and how it impedes cancer development has been challenging. Historically, the ability of RB to regulate the cell cycle placed it in a central role in proliferative control, and research focused on RB regulation of the E2F family of transcription factors. Remarkably, several recent studies have found additional tumour-suppressor functions of RB, including alternative roles in the cell cycle, maintenance of genome stability and apoptosis. These advances and new structural studies are combining to define …

Comparison Of The Pervasive Developmental Disorders Screening Test And Modified Checklist For Autism In Toddlers: Which Is The Better Predictor Of Autism In Toddlers?, Vanessa Marie Fessenden

UNLV Theses, Dissertations, Professional Papers, and Capstones

Early intervention for children with autism spectrum disorders (ASD) has significant impact on children and families. Early intervention optimizes long-term diagnosis for children with ASD. Unfortunately, many children with ASD are not diagnosed until after age three and often receive services from a local school district rather than through early intervention services. However, many of the symptoms of ASD can be hard to identify because symptoms during infancy may be more difficult to detect or may present differently than manifestations of the symptoms at older ages. Despite the difficulty in identifying symptoms of ASD in young children, there are certain …

Cinacalcet As Adjunctive Therapy For Hereditary 1,25-Dihydroxyvitamin D-Resistant Rickets., Tarak Srivastava, Uri S. Alon

Manuscripts, Articles, Book Chapters and Other Papers

Secondary hyperparathyroidism from inadequate calcium absorption in the gut, is the underlying pathophysiology for rachitic changes in hereditary vitamin D-resistant rickets (HVDRR). We describe a novel use of Cinacalcet to treat a child with HVDRR in whom conventional modes of therapy had to be discontinued. Cinacalcet therapy with high-dose oral calcium effectively normalized the metabolic abnormalities and bone condition. The relative ease of administration of the calcimimetic as a once- or twice-daily oral preparation, compared with traditional intravenous calcium administration, should encourage its move to the frontline of treatment of the disorder.