Pediatrics Commons^™

Primary Health Care: Potential Home For Family-Focused Preventive Interventions, Laurel K Leslie, Christopher J Mehus, J David Hawkins, Thomas Boat, Mary Ann Mccabe, Shari Barkin, Ellen C Perrin, Carol W Metzler, And 4 Others

Pediatrics Faculty Publications

Family-focused prevention programs have been shown to effectively reduce a range of negative behavioral health outcomes but have had limited reach. Three key barriers must be overcome to expand the reach of family-focused prevention programs and thereby achieve a significant public health impact. These barriers are (1) current social norms and perceptions of parenting programs; (2) concerns about the expertise and legitimacy of sponsoring organizations to offer parenting advice; and (3) a paucity of stable, sustainable funding mechanisms. Primary healthcare settings are well positioned to overcome these barriers. Recent changes within health care make primary care settings an increasingly favorable …

Expanding The Phenotype Associated With Naa10-Related N-Terminal Acetylation Deficiency., Chloé Saunier, Svein Isungset Støve, Bernt Popp, Bénédicte Gérard, Marina Blenski, Nicholas Ahmew, +Several Additional Authors

Pediatrics Faculty Publications

N-terminal acetylation is a common protein modification in eukaryotes associated with numerous cellular processes. Inherited mutations in NAA10, encoding the catalytic subunit of the major N-terminal acetylation complex NatA have been associated with diverse, syndromic X-linked recessive disorders, whereas de novo missense mutations have been reported in one male and one female individual with severe intellectual disability but otherwise unspecific phenotypes. Thus, the full genetic and clinical spectrum of NAA10 deficiency is yet to be delineated. We identified three different novel and one known missense mutation in NAA10, de novo in 11 females, and due to maternal germ …

Type 1 Diabetes Mellitus Presenting With Diabetic Ketoacidosis (Dka) In A Neonate, Fareed Ahmed, Sayyeda Ghazala Kazi, Waqas Khan

Department of Emergency Medicine

Neonatal diabetes mellitus (NDM) is a rare manifestation with an incidence of one affected individual among 400000 live births. NDM can be divided into Transient (TNDM) and Permanent (PNDM) types. A significant overlap occurs between both groups, to an extent that TNDM cannot be distinguished from PNDM based solely on clinical features. Diabetic ketoacidosis (DKA) is the leading cause of morbidity and mortality in children with type 1 diabetes mellitus (TIDM). DKA at diagnosis is more common in young children near the age of five years. Neonatal DKA is a rare occurrence causing it to be missed in the differential …

Failure To Thrive Hospitalizations And Risk Factors For Readmission To Children's Hospitals., Henry T. Puls, Matt Hall, Jessica L. Bettenhausen, Matthew B. Johnson, Christina Peacock, Jean L. Raphael, Jason G. Newland, Jeffrey D. Colvin

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Risk factors for failure to thrive (FTT) readmissions, including medical complexity, have not been described. We sought to characterize children hospitalized for FTT and identify risk factors associated with FTT-specific readmissions during the current era of increasing medical complexity among hospitalized children.

METHODS: This retrospective cohort study used the Pediatric Health Information System database of 43 freestanding children's hospitals across the United States. The cohort included childrenmodels, we assessed the association of demographic, clinical, diagnostic, and treatment characteristics with FTT-specific readmission.

RESULTS: There were 10 499 FTT hospitalizations, with 14.1% being readmitted for FTT within 3 years and 4.8% …

Pattern Of Relapse In Paediatric Acute Lymphoblastic Leukaemia In A Tertiary Care Unit, Emaduddin Siddiqui, Sayyeda Ghazala Kazi, Muhammad Irfan Habib, Khalid Mehmood Ahmed Khan, Nukhba Zia

Department of Emergency Medicine

OBJECTIVE:

To determine the frequency, site and time to relapse from diagnosis, and to see the relationship of relapse with important prognostic factors.

METHODS:

The prospective descriptive observational study was conducted at the National Institute of Child Health, Karachi, June 2005 to May 2007, and comprised newly-diagnosed cases of acute lymphoblastic leukaemia. Bone marrow aspiration was done on reappearance of blast cells in peripheral smear and cerebrospinal fluid. Detailed report was done each time when intra-thecal chemotherapy was given or there were signs and symptoms suggestive of central nervous system relapse. SPSS 12 was used for data analysis.

RESULTS:

Of …

Frequency And Outcome Of Meningitis In Pediatric Intensive Care Unit Of Pakistan, Amna Jawaid, Surriya Bano, Anwarul Haque, Khubaib Arif

Department of Emergency Medicine

Meningitis is a leading cause of morbidity and mortality worldwide in intensive care settings. The aim of this study was to assess the frequency and outcome in children with meningitis through a retrospective chart review done in pediatric intensive care unit of a tertiary care hospital from January 2000 to December 2014. During these 14 years, 64 patients were admitted with meningitis in pediatric intensive care unit. Out of 64, 36 were diagnosed with pyogenic meningitis, 18 patients with viral meningitis, and 10 with tuberculous meningitis. Most complications were observed in the initial 48 hours. Most common presentation was altered …

Effects Of Responsive Stimulation And Nutrition Interventions On Children's Development And Growth At Age 4 Years In A Disadvantaged Population In Pakistan: A Longitudinal Follow-Up Of A Cluster-Randomised Factorial Effectiveness Trial, Aisha K. Yousafzai, Jelena Obradovic, Muneera A. Rasheed, Arjumand Rizvi, Ximena A. Portilla, Nicole Tirado-Strayer, Saima Siyal, Uzma Memon

Department of Paediatrics and Child Health

Background: A previous study in Pakistan assessed the effectiveness of delivering responsive stimulation and enhanced nutrition interventions to young children. Responsive stimulation significantly improved children's cognitive, language, and motor development at 2 years of age. Both interventions significantly improved parenting skills, with responsive stimulation showing larger effects. In this follow-up study, we investigated whether interventions had benefits on children's healthy development and care at 4 years of age.
Methods: We implemented a follow-up study of the initial, community-based cluster-randomised effectiveness trial, which was conducted through the Lady Health Worker programme in Sindh, Pakistan. We re-enrolled 1302 mother-child dyads (87% of …

Pediatric Residents' Attitudes, Knowledge And Skills In Primary Care Behavioral Health Service Delivery, Jeffrey D. Shahidullah Phd, Paul W. Kettlewell Phd, Kathryn A. Dehart Md, Ilene Ladd Ma, Tyler Bogaczyk Bs, Kris Rooney Md, Amy Signore Phd, Sharon Larson Phd

Department of Pediatrics

No abstract provided.

Comparison Of A Powdered, Acidified Liquid, And Non-Acidified Liquid Human Milk Fortifier On Clinical Outcomes In Premature Infants., Melissa Thoene, Elizabeth R. Lyden, Kara Weishaar, Elizabeth Elliott, Ruomei Wu, Katelyn White, Hayley Timm, Ann Anderson-Berry

Journal Articles: Pediatrics

We previously compared infant outcomes between a powdered human milk fortifier (P-HMF) vs. acidified liquid HMF (AL-HMF). A non-acidified liquid HMF (NAL-HMF) is now commercially available. The purpose of this study is to compare growth and outcomes of premature infants receiving P-HMF, AL-HMF or NAL-HMF. An Institutional Review Board (IRB) approved retrospective chart review compared infant outcomes (born < 2000 g) who received one of three HMF. Growth, enteral nutrition, laboratory and demographic data were compared. 120 infants were included (P-HMF = 46, AL-HMF = 23, NAL-HMF = 51). AL-HMF infants grew slower in g/day (median 23.66 vs. P-HMF 31.27, NAL-HMF 31.74 (p < 0.05)) and in g/kg/day, median 10.59 vs. 15.37, 14.03 (p < 0.0001). AL-HMF vs. NAL-HMF infants were smaller at 36 weeks gestational age (median 2046 vs. 2404 g, p < 0.05). However AL-HMF infants received more daily calories (p = 0.21) and protein (p < 0.0001), mean 129 cal/kg, 4.2 g protein/kg vs. P-HMF 117 cal/kg, 3.7 g protein/kg , NAL-HMF 120 cal/kg, 4.0 g protein/kg. AL-HMF infants exhibited lower carbon dioxide levels after day of life 14 and 30 (p < 0.0001, p = 0.0038). Three AL-HMF infants (13%) developed necrotizing enterocolitis (NEC) vs. no infants in the remaining groups (p = 0.0056). A NAL-HMF is the most optimal choice for premature human milk-fed infants in a high acuity neonatal intensive care unit (NICU).

Cardiac Arrest In An Adolescent With Uhl’S Anomaly: Two Unusual Cardiac Arrhythmia Mechanisms, Jeffrey P. Moak, Gail D. Pearson, Bradley Clark, Charles I. Berul, Russell R. Cross, Dilip S. Nath

Pediatrics Faculty Publications

No abstract provided.

Improving Sickle Cell Transitions Of Care Through Health Information Technology., Jennifer R Frost, Rebecca K Cherry, Suzette O Oyeku, Elissa Z Faro, Lori E Crosby, Maria Britto, Lisa K. Tuchman, Ivor B. Horn, Charles J Homer, Anjali Jain

Pediatrics Faculty Publications

Introduction

Transitions between inpatient and outpatient care and pediatric to adult care are associated with increased mortality for sickle cell disease (SCD) patients. As accurate and timely sharing of health information is essential during transitions, a health information technology (HIT)-enabled tool holds promise to improve care transitions.

Methods

From 2012 through 2014, the team conducted and analyzed data from an environmental scan, key informant interviews, and focus groups to inform the development of an HIT-enabled tool for SCD patients’ use during care transitions. The scan included searches of peer-reviewed and gray literature to understand SCD patient needs, transition concerns, and …

Intracardiac Tumor Causing Left-Ventricular Outflow-Tract Obstruction In A Newborn, Christopher P. Jordan, John P. Costello, Kendal Endicott, Christine Reyes, Thomas Hougen, Susan Cummings, Dilip S. Nath

Pediatrics Faculty Publications

The following report describes the case of newborn girl with an asymptomatic systolic murmur, which on imaging revealed a nearly obstructive mass in the left-ventricular outflow tract. The mass was resected and found to be consistent with a rhabdomyoma. Here, we describe the pathologic and clinical characteristics of this tumor.

Neonatal Pain Management: Still In Search For The Holy Grail., Karel Allegaert, John N. Van Den Anker

Pediatrics Faculty Publications

Inadequate pain management but also inappropriate use of analgesics in early infancy has negative effects on neurodevelopmental outcome. As a consequence, neonatal pain management is still in search for the Holy Grail. At best, effective pain management is based on prevention, assessment, and treatment followed by a re-assessment of the pain to determine if additional treatment is still necessary. Unfortunately, epidemiological observations suggest that neonates are undergoing painful procedures very frequently, unveiling the need for effective preventive, non-pharmacological strategies. In addition, assessment is still based on validated, multimodal, but subjective pain assessment tools. Finally, in neonatal intensive care units, there …

Calls To A Teratogen Information Service Regarding Potential Exposures In Pregnancy And Breastfeeding, Sarah C. Campbell, Tyler T. Kast, Julia Robertson, Catherine M.T. Sherwin

Pediatrics Faculty Publications

Background

MotherToBaby Utah is a teratogen information service that provides support for pregnant and breastfeeding women and healthcare providers regarding risks of exposures to medications, infections, herbals, homeopathic and dietary medications, chemicals and other substances. Calls are anonymous and free of charge. This study was undertaken to examine the volume and classification of calls regarding exposures during pregnancy and breastfeeding.

Methods

Data were extracted from calls requesting information about medication use and other exposures to pregnant and breastfeeding women, between January 1 2009 and December 31 2012. Descriptive statistics were calculated.

Results

A total of 27,299 calls regarding 46,031 exposures …

Decreased Pregnane X Receptor Expression In Children With Active Crohn's Disease, Valentina Shakhnovich, Carrie A. Vyhlidal, Craig A. Friesen, Amber Hildreth, Vivekanand Singh, James Daniel, Gregory L. Kearns, J Steven Leeder

Manuscripts, Articles, Book Chapters and Other Papers

Copyright © 2016 The Author(s). Expression of the pregnane X receptor (PXR) has been reported to be decreased in animal models of inflammatory bowel disease (IBD). To investigate the differential expression of PXR in children with Crohn's disease, a type of IBD, RNA was extracted from archived intestinal biopsies from 18 children with Crohn's disease (CD) and 12 age- and sex-matched controls (aged 7-17yrs). The aim of this investigation was to compare the relative mRNA expression of PXR, cytochrome p450 3A4 (CYP3A4), and villin 1 (VIL1) (a marker of epithelial cell integrity) in the inflamed terminal ileum (TI) versus noninflamed …

Validation Of Noninvasive Measures Of Left Ventricular Mechanics In Children: A Simultaneous Echocardiographic And Conductance Catheterization Study., Shahryar M. Chowdhury, Ryan J. Butts, Carolyn L. Taylor, Varsha M. Bandisode, Karen S. Chessa, Anthony M. Hlavacek, Girish S. Shirali, G Hamilton Baker

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The accuracy of echocardiography in evaluating left ventricular contractility has not been validated in children. The objective of this study was to compare echocardiographic measures of contractility with those derived from pressure-volume loop (PVL) analysis in children.

METHODS: Patients with relatively normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. The gold-standard measure of contractility, end-systolic elastance (Ees), was obtained via balloon occlusion of one or both vena cavae. Echocardiograms were performed immediately after PVL analysis under the same anesthetic conditions. Single-beat estimations of echocardiographic Ees were calculated using four different …

Maternal Healthcare In Eastern Uganda: The Three Delays, Mothers Making Empowered Choices, And Combatting Maternal Mortality, Emma Gier

Independent Study Project (ISP) Collection

Maternal and child health has been noted as an international concern and issue. While access to maternal healthcare has improved in Uganda since 2000, maternal mortality rates have remained high in the country as of 2015. However, maternal and child healthcare are inherently important in promoting sustainable and positive development. For that reason, I have explored maternal healthcare in Uganda’s Eastern region, specifically in Mbale Town and surrounding rural areas including Kween and Manafwa districts. Specifically I have looked at what factors influence women’s decision to give birth in a health centre or hospital, versus at home. I have also …

Outcomes After Pediatric Fundoplication: Defining The Redo Population, J E. Baerg, E E. Perrone, R A. Vannix, D L. Thorpe, A Gasior, Shawn D. St Peter

Manuscripts, Articles, Book Chapters and Other Papers

Objective: The aims were to compare outcome variables in children with gastroesophageal reflux disease (GERD) and one Nissen fundoplication to children with redo fundoplications and define the pediatric redo population. Methods: After IRB approval (#5100277), a case control study was conducted of children younger than 18 years, from two children’s hospitals, with one Nissen fundoplication (control group) or a redo performed between January 1995 and March 2011. Complete data were collected by phone calls to caregivers in December 2012. Only redo operations performed after recurrence of GERD symptoms and wrap herniation into the mediastinum confirmed by contrast radiograph were included. …

Pik3ca-Associated Developmental Disorders Exhibit Distinct Classes Of Mutations With Variable Expression And Tissue Distribution, Ghayda Mirzaa, Andrew E Timms, Valerio Conti, Evan August Boyle, Katta M Girisha, Beth Martin, Martin Kircher, Carissa Olds, Jane Juusola, Sarah Collins, Kaylee Park, Melissa Carter, Ian Glass, Inge Krägeloh-Mann, David Chitayat, Aditi Shah Parikh, Rachael Bradshaw, Erin Torti, Stephen Braddock, Leah Burke, Sondhya Ghedia, Mark Stephan, Fiona Stewart, Chitra Prasad, Melanie Napier, Sulagna Saitta, Rachel Straussberg, Michael Gabbett, Bridget C O'Connor, Catherine E Keegan, Lim Jiin Yin, Angeline Hwei Meeng Lai, Nicole Martin, Margaret Mckinnon, Marie-Claude Addor, Luigi Boccuto, Charles E Schwartz, Agustina Lanoel, Robert L Conway, Koenraad Devriendt, Katrina Tatton-Brown, Mary Ella Pierpont, Michael Painter, Lisa Worgan, James Reggin, Raoul Hennekam, Karen Tsuchiya, Colin C Pritchard, Mariana Aracena, Karen W Gripp, Maria Cordisco, Hilde Van Esch, Livia Garavelli, Cynthia Curry, Anne Goriely, Hulya Kayserilli, Jay Shendure, John Graham, Renzo Guerrini, William B Dobyns

Articles, Abstracts, and Reports

Mosaicism is increasingly recognized as a cause of developmental disorders with the advent of next-generation sequencing (NGS). Mosaic mutations of PIK3CA have been associated with the widest spectrum of phenotypes associated with overgrowth and vascular malformations. We performed targeted NGS using 2 independent deep-coverage methods that utilize molecular inversion probes and amplicon sequencing in a cohort of 241 samples from 181 individuals with brain and/or body overgrowth. We identified PIK3CA mutations in 60 individuals. Several other individuals (n = 12) were identified separately to have mutations in PIK3CA by clinical targeted-panel testing (n = 6), whole-exome sequencing ( …

The Feasibility Of Community Level Interventions For Pre-Eclampsia In South Asia And Sub-Saharan Africa: A Mixed-Methods Design, Asif Raza Khowaja, Rahat Najam Qureshi, Diane Sawchuck, Olufemi T. Oladapo, Olalekan O. Adetoro, Elizabeth A. Orenuga, Mrutyunjaya Bellad, Ashalata Mallapur, Umesh Charantimath, Esperanca Sevene

Department of Paediatrics and Child Health

Background: Globally, pre-eclampsia and eclampsia are major contributors to maternal and perinatal mortality; of which the vast majority of deaths occur in less developed countries. In addition, a disproportionate number of morbidities and mortalities occur due to delayed access to health services. The Community Level Interventions for Pre-eclampsia (CLIP) Trial aims to task-shift to community health workers the identification and emergency management of pre-eclampsia and eclampsia to improve access and timely care. Literature revealed paucity of published feasibility assessments prior to initiating large-scale community-based interventions. Arguably, well-conducted feasibility studies can provide valuable information about the potential success of clinical trials …