Pediatrics Commons^™

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 …

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 …

Point-Of-Care Ultrasonography By Pediatric Emergency Medicine Physicians., Jennifer R. Marin, Resa E. Lewiss, American Academy Of Pediatrics, Committee On Pediatric Emergency Medicine, Society For Academic Emergency Medicine, Academy Of Emergency Ultrasound, American College Of Emergency Physicians, Pediatric Emergency Medicine Committee, World Interactive Network Focused On Critical Ultrasound, Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

Emergency physicians have used point-of-care ultrasonography since the 1990 s. Pediatric emergency medicine physicians have more recently adopted this technology. Point-of-care ultrasonography is used for various scenarios, particularly the evaluation of soft tissue infections or blunt abdominal trauma and procedural guidance. To date, there are no published statements from national organizations specifically for pediatric emergency physicians describing the incorporation of point-of-care ultrasonography into their practice. This document outlines how pediatric emergency departments may establish a formal point-of-care ultrasonography program. This task includes appointing leaders with expertise in point-of-care ultrasonography, effectively training and credentialing physicians in the department, and providing ongoing …

Point-Of-Care Ultrasonography By Pediatric Emergency Medicine Physicians, American Academy Of Pediatrics, Committee On Pediatric Emergency Medicine, Society For Academic Emergency Medicine,Academy Of Emergency Ultrasound, American College Of Emergency Physicians, Pediatric Emergency Medicine Committee, World Interactive Network Focused On Critical Ultrasound, Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

Point-of-care ultrasonography is increasingly being used to facilitate accurate abstract and timely diagnoses and to guide procedures. It is important for pediatric emergency medicine (PEM) physicians caring for patients in the emergency department to receive adequate and continued point-of-care ultrasonography training for those indications used in their practice setting. Emergency departments should have credentialing and quality assurance programs. PEM fellowships should provide appropriate training to physician trainees. Hospitals should provide privileges to physicians who demonstrate competency in point-of-care ultrasonography. Ongoing research will provide the necessary measures to define the optimal training and competency assessment standards. Requirements for credentialing and hospital …

Clinical Assessment Of The Infant And Child Following Perinatal Brachial Plexus Injury, Susan V. Duff, Carol Dematteo

Physical Therapy Faculty Articles and Research

Introduction

After perinatal brachial plexus injury (PBPI), clinicians play an important role in injury classification as well as the assessment of recovery and secondary conditions. Early assessment guides the initial plan of care and influences follow-up and long-term outcome.

Purpose

To review methods used to assess, classify and monitor the extent and influence of PBPI with an emphasis on guidelines for clinicians.

Methods

We use The International Classification of Functioning, Disability, and Health (ICF) model to provide a guide to assessment after PBPI for rehabilitation clinicians.

Discussion

With information gained from targeted assessments, clinicians can design interventions to increase the …

Patient- And Family-Centered Care Of Children In The Emergency Department., Nanette Dudley, Alice Ackerman, Kathleen M. Brown, Sally K. Snow, American Academy Of Pediatrics Committee On Pediatric Emergency Medicine, American College Of Emergency Physicians Pediatric Emergency Medicine Committee, Emergency Nurses Association Pediatric Committee., Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

Patient- and family-centered care is an approach to the planning, delivery, and evaluation of health care that is grounded in a mutually beneficial partnership among patients, families, and health care professionals. Providing patient- and family-centered care to children in the emergency department setting presents many opportunities and challenges. This revised technical report draws on previously published policy statements and reports, reviews the current literature, and describes the present state of practice and research regarding patient- and family-centered care for children in the emergency department setting as well as some of the complexities of providing such care.

Best Practices For Improving Flow And Care Of Pediatric Patients In The Emergency Department., Isabel Barata, Kathleen M. Brown, Laura Fitzmaurice, Elizabeth Stone Griffin, Sally K. Snow, American College Of Emergency Physicians Pediatric Emergency Medicine Committee, Emergency Nurses Association Pediatric Committee, Gregory P. Conners, American Academy Of Pediatrics Committee On Pediatric Emergency Medicine

Manuscripts, Articles, Book Chapters and Other Papers

This report provides a summary of best practices for improving flow, reducing waiting times, and improving the quality of care of pediatric patients in the emergency department.

Pupillometry: A Non-Invasive Technique For Pain Assessment In Paediatric Patients., Mark A. Connelly, Jacob T. Brown, Gregory L. Kearns, Rawni A. Anderson, Shawn D. St Peter, Kathleen A. Neville

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Pupillometry has been used to assess pain intensity and response to analgesic medications in adults. The aim of this observational study was to explore proof of concept for the use of this technique in paediatric patients. Changes in pupil parameters before and after opioid exposure also were evaluated.

DESIGN AND SETTING: This was a single-centre, prospective study conducted at an academic paediatric medical centre.

PATIENTS: Children 9-17 years of age undergoing elective surgical correction of pectus excavatum were enrolled into a protocol approved by the human ethical committee (institutional review board).

INTERVENTIONS: Pupil size and reactivity were measured using …

Subspecialty Surveillance Of Long-Term Course Of Small And Moderate Muscular Ventricular Septal Defect: Heterogenous Practices, Low Yield., Erik L. Frandsen, Aswathy V. House, Yunbin Xiao, David A. Danford, Shelby Kutty

Journal Articles: Pediatrics

BACKGROUND: No expert consensus guides practice for intensity of ongoing pediatric cardiology surveillance of hemodynamically insignificant small and moderate muscular ventricular septal defect (mVSD). Therefore, despite the well-established benign natural history of mVSD, there is potential for widely divergent follow up practices. The purpose of this investigation was to evaluate (1) variations in follow up of mVSD within an academic children's hospital based pediatric cardiology practice, and (2) the frequency of active medical or surgical management resulting from follow up of mVSD.

METHODS: We retrospectively reviewed records of 600 patients with isolated mVSD echocardiographically diagnosed between 2006 and 2012. Large …

Death Of A Child In The Emergency Department., American Academy Of Pediatrics Committee On Pediatric Emergency Medicine, American College Of Emergency Physicians Pediatric Emergency Medicine Committee, Emergency Nurses Association Pediatric Committee, Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

The American Academy of Pediatrics, American College of Emergency Physicians, and Emergency Nurses Association have collaborated to identify practices and principles to guide the care of children, families, and staff in the challenging and uncommon event of the death of a child in the emergency department in this policy statement and in an accompanying technical report.

Death Of A Child In The Emergency Department., Patricia O'Malley, Isabel Barata, Sally Snow, American Academy Of Pediatrics Committee On Pediatric Emergency Medicine, American College Of Emergency Physicians Pediatric Emergency Medicine Committee, Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

The death of a child in the emergency department (ED) is one of the most challenging problems facing ED clinicians. This revised technical report and accompanying policy statement reaffirm principles of patient- and family-centered care. Recent literature is examined regarding family presence, termination of resuscitation, bereavement responsibilities of ED clinicians, support of child fatality review efforts, and other issues inherent in caring for the patient, family, and staff when a child dies in the ED. Appendices are provided that offer an approach to bereavement activities in the ED, carrying out forensic responsibilities while providing compassionate care, communicating the news of …

Functional Health Status In Children And Adolescents After Fontan: Comparison Of Generic And Disease-Specific Assessments., Brian W. Mccrindle, Victor Zak, Victoria L. Pemberton, Linda M. Lambert, Victoria L. Vetter, Wyman W. Lai, Karen Uzark, Renee Margossian, Andrew M. Atz, Amanda Cook, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease.

METHODS: This was a cross-sectional observational study involving 325 single ventricle patients, aged 10-18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables …

Airway Obstruction In Congenital Central Hypoventilation Syndrome, Alexandra K Reverdin, Ricardo Mosquera, Giuseppe N Colasurdo, Cindy K Jon, Roya M Clements

Faculty, Staff and Student Publications

Congenital central hypoventilation syndrome (CCHS) is the failure of the autonomic system to control adequate ventilation while asleep with preserved ventilatory response while awake. We report a case of a patient with CCHS who presented with intrathoracic and extrathoracic airway obstruction after tracheostomy tube decannulation and phrenic nerve pacer placement. Nocturnal polysomnography (NPSG) revealed hypoxia, hypercapnia and obstructive sleep apnoea, which required bilevel positive airway pressure titration. Airway endoscopy demonstrated tracheomalacia and paretic true vocal cords in the paramedian position during diaphragmatic pacing. Laryngeal electromyography demonstrated muscular electrical impulses that correlated with diaphragmatic pacer settings. Thus, we surmise that the …

Pediatric Care Recommendations For Freestanding Urgent Care Facilities., Committee On Pediatric Emergency Medicine, Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

Treatment of children at freestanding urgent care facilities has become common in pediatric health care. Well-managed freestanding urgent care facilities can improve the health of the children in their communities, integrate into the medical community, and provide a safe, effective adjunct to, but not a replacement for, the medical home or emergency department. Recommendations are provided for optimizing freestanding urgent care facilities' quality, communication, and collaboration in caring for children.

Lean Body Mass May Explain Apparent Racial Differences In Carotid Intima-Media Thickness In Obese Children., Shahryar M. Chowdhury, Melissa H. Henshaw, Brad Friedman, J Philip Saul, Girish S. Shirali, Janet Carter, Bryana M. Levitan, Tom Hulsey

Manuscripts, Articles, Book Chapters and Other Papers

Background: Racial differences in carotid intima-media thickness (cIMT) have been suggested to be associated with the disproportionally high prevalence of cardiovascular disease in black adults. The objective of this study was to evaluate the effects of cardiovascular risk factors on the racial differences seen in cIMT in obese children.

Methods: Obese subjects aged 4 to 21 years were recruited prospectively. Height, weight, blood pressure, fasting insulin, glucose, lipid panel, high-sensitivity C-reactive protein, and body composition by dual-energy x-ray absorptiometry were obtained. B-mode carotid imaging was analyzed by a single blinded physician.

Results: A total of 120 subjects (46 white, 74 …

Withholding Or Termination Of Resuscitation In Pediatric Out-Of-Hospital Traumatic Cardiopulmonary Arrest., American College Of Surgeons Committee On Trauma, American College Of Emergency Physicians Pediatric Emergency Medicine Committee, National Association Of Ems Physicians, American Academy Of Pediatrics Committee On Pediatric Emergency Medicine,, Mary E. Fallat, Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

This multiorganizational literature review was undertaken to provide an evidence base for determining whether recommendations for out-of-hospital termination of resuscitation could be made for children who are victims of traumatic cardiopulmonary arrest. Although there is increasing acceptance of out-of-hospital termination of resuscitation for adult traumatic cardiopulmonary arrest when there is no expectation of a good outcome, children are routinely excluded from state termination-of-resuscitation protocols. The decision to withhold resuscitative efforts in a child under specific circumstances (decapitation or dependent lividity, rigor mortis, etc) is reasonable. If there is any doubt as to the circumstances or timing of the traumatic cardiopulmonary …

Variability Of M-Mode Versus Two-Dimensional Echocardiography Measurements In Children With Dilated Cardiomyopathy., Caroline K. Lee, Renee Margossian, Lynn A. Sleeper, Charles E. Canter, Shan Chen, Lloyd Y. Tani, Girish S. Shirali, Anita Szwast, Elif Seda Selamet Tierney, M Jay Campbell, Fraser Golding, Yanli Wang, Karen Altmann, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

M-mode and 2-dimensional (2D) echocardiographic imaging are routinely used to quantify left-ventricular (LV) size and function in pediatric patients with dilated cardiomyopathy (DCM). The reproducibility of and correlation between these techniques are unknown. This analysis sought to compare interreader, intrareader, and interacquisition reproducibility of M-mode versus 2D measurements in pediatric DCM patients. The Ventricular Volume Variability study of the Pediatric Heart Network is a multicenter, prospective, observational study assessing the course of chronic DCM in children. Two sonographers performed baseline image acquisitions locally, and two readers performed measurements at the echocardiographic core laboratory. One reader repeated measurements 1 month later. …

Headstrong Intervention For Pediatric Migraine Headache: A Randomized Clinical Trial., Michael A. Rapoff, Mark Connelly, Jennifer Bickel, Scott W. Powers, Andrew D. Hershey, Janelle R. Allen, Cynthia W. Karlson, Catrina C. Litzenburg, John M. Belmont

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The purpose of this study was to evaluate the efficacy of a self-guided CD-ROM program ("Headstrong") containing cognitive-behavioral self-management strategies versus an educational CD-ROM program for treating headaches, headache-related disability, and quality of life.

METHODS: Participants were 35 children ages 7-12 years with migraine recruited from one university medical center and two children's hospital headache clinics. Participants were randomly assigned to complete the Headstrong or educational control CD-ROM program over a 4-week period. Data on headache frequency, duration, and severity, migraine-related disability, and quality of life (QOL) were obtained at baseline, post-intervention, and 3-months post-intervention.

RESULTS: At post-intervention, Headstrong …

Whole Exome Sequencing Identifies Novel Genes For Fetal Hemoglobin Response To Hydroxyurea In Children With Sickle Cell Anemia., V. A. Sheehan, J. R. Crosby, A. Sabo, N. A. Mortier, T. A. Howard, D. M. Muzny, S. Dugan-Perez, Banu Aygun, K. A. Nottage, J. M. Flanagan, +3 Additional Authors

Journal Articles

Hydroxyurea has proven efficacy in children and adults with sickle cell anemia (SCA), but with considerable inter-individual variability in the amount of fetal hemoglobin (HbF) produced. Sibling and twin studies indicate that some of that drug response variation is heritable. To test the hypothesis that genetic modifiers influence pharmacological induction of HbF, we investigated phenotype-genotype associations using whole exome sequencing of children with SCA treated prospectively with hydroxyurea to maximum tolerated dose (MTD). We analyzed 171 unrelated patients enrolled in two prospective clinical trials, all treated with dose escalation to MTD. We examined two MTD drug response phenotypes: HbF (final …

Differential Expression Of The Nuclear-Encoded Mitochondrial Transcriptome In Pediatric Septic Shock., Scott L Weiss, Natalie Z Cvijanovich, Geoffrey L Allen, Neal J Thomas, Robert J. Freishtat, Nick Anas, +11 Additional Authors

Pediatrics Faculty Publications

INTRODUCTION: Increasing evidence supports a role for mitochondrial dysfunction in organ injury and immune dysregulation in sepsis. Although differential expression of mitochondrial genes in blood cells has been reported for several diseases in which bioenergetic failure is a postulated mechanism, there are no data about the blood cell mitochondrial transcriptome in pediatric sepsis.

METHODS: We conducted a focused analysis using a multicenter genome-wide expression database of 180 children ≤ 10 years of age with septic shock and 53 healthy controls. Using total RNA isolated from whole blood within 24 hours of PICU admission for septic shock, we evaluated 296 nuclear-encoded …

American And Brazilian Children With Primary Urolithiasis: Similarities And Disparities., Maria Goretti Moreira Guimarães Penido, Marcelo De Sousa Tavares, Milena Maria Moreira Guimarães, Tarak Srivastava, Uri S. Alon

Manuscripts, Articles, Book Chapters and Other Papers

Objectives. Considering the differences in location, socioeconomic background, and cultural background, the aim of this study was to try to identify possible factors associated with the increased incidence of urolithiasis by comparing American and Brazilian children with stones.
Methods. Data of 222 American and 190 Brazilian children with urolithiasis were reviewed including age, gender, body mass index, imaging technique used (ultrasound and computed tomography), and 24-hour urine volume and chemistries.
Results. There were no differences between age and gender at diagnosis. Brazilian children were leaner but in no population did obesity rate exceed that of the general population. Ultrasound was …

Early Recovery In Post Varicella Transverse Myelitis, Prem Chand, Shahnaz Brahim, Syed Sohail Zaidi, Nida Amjad

Department of Paediatrics and Child Health

A two and a half year old Kenyan girl presented with recurrent chest infections and difficulty in swallowing. Her clinical and laboratory workup was suggestive of lower respiratory tract infection for which she received a course of antibiotics; however, she remains symptomatic after the management of her suspected diagnosis. Therefore, further radiological workup including a chest CT scan and barium study were performed. This showed a homogeneous mass surrounded by ascending and descending aorta in the posterior mediastinum that was compressing the middle esophagus. She had a posterolateral thoracotomy which revealed a cystic mass with smooth surfaces (5 x 5 …

Diagnosis And Acute Management Of Patients With Concussion At Children's Hospitals., Jeffrey D. Colvin, Cary Thurm, Brian M. Pate, Jason G. Newland, Matt Hall, William P. Meehan Iii

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: To describe the number of hospital admissions for concussion at paediatric hospitals in the USA. To describe the use of imaging and medications for acute concussion paediatric patients.

Design: Cross-sectional study.

Setting: Children's hospitals participating in the Pediatric Health Information System in the USA during a 10-year period.

Patients: All emergency department (ED) visits and inpatient admissions with the primary diagnosis of concussion, defined as International Classification of Diseases, Ninth Revision, Clinical Modification codes for: (1) concussion, (2) postconcussion syndrome or (3) skull fracture without mention of intracranial injury with concussion.

Main outcome measures: The proportion of concussion patients …

Multicenter Study Of Pectus Excavatum, Final Report: Complications, Static/Exercise Pulmonary Function, And Anatomic Outcomes., Robert E. Kelly, Robert B. Mellins, Robert C. Shamberger, Karen K. Mitchell, M Louise Lawson, Keith T. Oldham, Richard G. Azizkhan, Andre V. Hebra, Donald Nuss, Michael J. Goretsky, Ronald J. Sharp, George W. Holcomb, Walton K T Shim, Stephen M. Megison, R Lawrence Moss, Annie H. Fecteau, Paul M. Colombani, Dan Cooper, Traci Bagley, Amy Quinn, Alan B. Moskowitz, James F. Paulson

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: A multicenter study of pectus excavatum was described previously. This report presents our final results.

STUDY DESIGN: Patients treated surgically at 11 centers were followed prospectively. Each underwent a preoperative evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, complications, and perioperative pain. One year after treatment, patients underwent repeat chest CT scan, pulmonary function tests, and body image survey. A subset of 50 underwent exercise pulmonary function testing.

RESULTS: Of 327 patients, 284 underwent Nuss procedure and 43 underwent open procedure without mortality. Of 182 patients with complete follow-up (56%), …

Early Diagnosis And Treatment Of Invasive Pulmonary Aspergillosis In A Patient With Cystic Fibrosis, Ricardo Alberto Mosquera, Lila Estrada, Roya Mohebpour Clements, Cindy K Jon

Faculty, Staff and Student Publications

Invasive pulmonary aspergillosis is a rare and fatal complication in patients with cystic fibrosis (CF) who lack concomitant risk factors. The few documented cases in children have all resulted in deaths during hospitalisation. We present the case of a 12-year-old boy with CF who was admitted for an exacerbation which was unresponsive to antibiotic therapy. The findings on imaging raised concerns about a possible fungal infection. As a result, voriconazole therapy was started prior to his respiratory deterioration. He was later found to be β-D glucan and Aspergillus Ag galactomannan positive confirming the suspicion for invasive pulmonary aspergillosis. Three months …

Predictors Of Disease Progression In Pediatric Dilated Cardiomyopathy., Kimberly M. Molina, Peter Shrader, Steven D. Colan, Seema Mital, Renee Margossian, Lynn A. Sleeper, Girish S. Shirali, Piers Barker, Charles E. Canter, Karen Altmann, Elizabeth Radojewski, Elif Seda Selamet Tierney, Jack Rychik, Lloyd Y. Tani, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Background: Despite medical advances, children with dilated cardiomyopathy (DCM) remain at high risk of death or need for cardiac transplantation. We sought to identify predictors of disease progression in pediatric DCM.

Methods and results: The Pediatric Heart Network evaluated chronic DCM patients with prospective echocardiographic and clinical data collection during an 18-month follow-up. Inclusion criteria were age <22 years and DCM disease duration >2 months. Patients requiring intravenous inotropic/mechanical support or listed status 1A/1B for transplant were excluded. Disease progression was defined as an increase in transplant listing status, hospitalization for heart failure, intravenous inotropes, mechanical support, or death. Predictors of disease progression were identified using …

Phenotypic Parameters Predict Time To Normalization In Infants With Hypogammaglobulinemia., Robert C. Van Winkle, Walter W. Hauck, Stephen J. Mcgeady

Department of Pediatrics Faculty Papers

PURPOSE: Infants with recurrent infection may be found to have hypogammaglobulinemia without impaired specific antibody responses. Many will be diagnosed with transient hypogammaglobulinemia of infancy.

METHODS: This study used a parametric survival analysis of 100 infants with hypogammaglobulinemia to predict time to normalization.

RESULTS: Aggregate initial immunoglobulins (IgG + IgA + IgM), as a percentage of age-adjusted normal, predicted time to resolution: median time to resolution for the infants in the lowest quartile of aggregate levels (≤81 % of age-adjusted lower limits) was greater than 5 years, with 34 % resolving in 3 years. For infants in the highest quartile …

Factors Impacting Echocardiographic Imaging After The Fontan Procedure: A Report From The Pediatric Heart Network Fontan Cross-Sectional Study., Richard V. Williams, Renee Margossian, Minmin Lu, Andrew M. Atz, Timothy J. Bradley, Michael Jay Campbell, Steven D. Colan, Dianne Gallagher, Wyman W. Lai, Gail D. Pearson, Ashwin Prakash, Girish S. Shirali, Meryl S. Cohen, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Echocardiographic image quality in Fontan survivors may be limited by a variety of factors. We sought to describe echocardiographic quality and factors associated with study quality in subjects participating in the Pediatric Heart Network Fontan Cross-Sectional Study. Echocardiograms were obtained at 7 clinical sites using a standard protocol. Quality grading and analysis were performed by a core laboratory. Univariate and multivariable modeling were performed to assess factors associated with quality and ability to obtain images sufficient for prespecified quantitative analysis. A total of 543 echocardiograms were obtained. The quality of echocardiograms improved over the duration of the study. The great …

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 …