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Articles 1351 - 1380 of 1416

Full-Text Articles in Diseases

Unique Phenotype In A Patient With Charge Syndrome., Shobhit Jain, Hyung-Goo Kim, Felicitas Lacbawan, Irene Meliciani, Wolfgang Wenzel, Ingo Kurth, Josefina Sharma, Morris Schoeneman, Svetlana Ten, Lawrence C. Layman, Elka Jacobson-Dickman Oct 2011

Unique Phenotype In A Patient With Charge Syndrome., Shobhit Jain, Hyung-Goo Kim, Felicitas Lacbawan, Irene Meliciani, Wolfgang Wenzel, Ingo Kurth, Josefina Sharma, Morris Schoeneman, Svetlana Ten, Lawrence C. Layman, Elka Jacobson-Dickman

Manuscripts, Articles, Book Chapters and Other Papers

CHARGE is a phenotypically heterogeneous autosomal dominant disorder recognized as a cohesive syndrome since the identification of CHD7 as a genetic etiology. Classic features include: Coloboma, Heart defects, Atresia choanae, Retarded growth and development, Genitourinary abnormalities, and Ear anomalies and/or deafness. With greater accessibility to genetic analysis, a wider spectrum of features are emerging, and overlap with disorders such as DiGeorge syndrome, Kallmann syndrome, and Hypoparathyroidism Sensorineural Deafness and Renal Disease syndrome, is increasingly evident. We present a patient with a unique manifestation of CHARGE syndrome, including primary hypoparathyroidism and a limb anomaly; to our knowledge, he is also the …


Executive Summary: The Management Of Community-Acquired Pneumonia In Infants And Children Older Than 3 Months Of Age: Clinical Practice Guidelines By The Pediatric Infectious Diseases Society And The Infectious Diseases Society Of America., John S. Bradley, Carrie L. Byington, Samir S. Shah, Brian Alverson, Edward R. Carter, Christopher Harrison, Sheldon L. Kaplan, Sharon E. Mace, George H. Mccracken, Matthew R. Moore, Shawn D. St Peter, Jana A. Stockwell, Jack T. Swanson, Pediatric Infectious Diseases Society And The Infectious Diseases Society Of America Oct 2011

Executive Summary: The Management Of Community-Acquired Pneumonia In Infants And Children Older Than 3 Months Of Age: Clinical Practice Guidelines By The Pediatric Infectious Diseases Society And The Infectious Diseases Society Of America., John S. Bradley, Carrie L. Byington, Samir S. Shah, Brian Alverson, Edward R. Carter, Christopher Harrison, Sheldon L. Kaplan, Sharon E. Mace, George H. Mccracken, Matthew R. Moore, Shawn D. St Peter, Jana A. Stockwell, Jack T. Swanson, Pediatric Infectious Diseases Society And The Infectious Diseases Society Of America

Manuscripts, Articles, Book Chapters and Other Papers

Evidenced-based guidelines for management of infants and children with community-acquired pneumonia (CAP) were prepared by an expert panel comprising clinicians and investigators representing community pediatrics, public health, and the pediatric specialties of critical care, emergency medicine, hospital medicine, infectious diseases, pulmonology, and surgery. These guidelines are intended for use by primary care and subspecialty providers responsible for the management of otherwise healthy infants and children with CAP in both outpatient and inpatient settings. Site-of-care management, diagnosis, antimicrobial and adjunctive surgical therapy, and prevention are discussed. Areas that warrant future investigations are also highlighted.


Mast Cell Activation And Clinical Outcome In Pediatric Cholelithiasis And Biliary Dyskinesia., Craig A. Friesen, Nancy Neilan, James F. Daniel, Kim Radford, Jennifer Verrill Schurman, Ding-You Li, Linda Andre, Shawn D. St Peter, G W. Holcomb Iii Sep 2011

Mast Cell Activation And Clinical Outcome In Pediatric Cholelithiasis And Biliary Dyskinesia., Craig A. Friesen, Nancy Neilan, James F. Daniel, Kim Radford, Jennifer Verrill Schurman, Ding-You Li, Linda Andre, Shawn D. St Peter, G W. Holcomb Iii

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The current study was undertaken to determine the degree of activation of gallbladder mucosal mast cells, whether mast cell (MC) density or activation differ between patients with and without a positive clinical response to cholecystectomy, and whether either density or activation correlate with gallbladder emptying.

RESULTS: Fifteen biliary dyskinesia (BD) and 13 symptomatic cholelithiasis (CL) patients undergoing cholecystectomy were prospectively enrolled. Gallbladder wall MC density (by immunohistochemistry) and activation (by electron microscopy) were determined. Clinical response was evaluated 30 days post-cholecystectomy on a 5-point Likert-type scale. A complete or nearly complete clinical response was seen in 100% of CL …


Factors Associated With Serum Brain Natriuretic Peptide Levels After The Fontan Procedure., Andrew M. Atz, Victor Zak, Roger E. Breitbart, Steven D. Colan, Sara K. Pasquali, Daphne T. Hsu, Minmin Lu, Lynn Mahony, Stephen M. Paridon, Michael D. Puchalski, Tal Geva, Brian W. Mccrindle, Pediatric Heart Network Investigators, Girish S. Shirali Jul 2011

Factors Associated With Serum Brain Natriuretic Peptide Levels After The Fontan Procedure., Andrew M. Atz, Victor Zak, Roger E. Breitbart, Steven D. Colan, Sara K. Pasquali, Daphne T. Hsu, Minmin Lu, Lynn Mahony, Stephen M. Paridon, Michael D. Puchalski, Tal Geva, Brian W. Mccrindle, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Although a useful marker of heart failure in adults, the utility of brain natriuretic peptide concentration (BNP) for children after the Fontan procedure is not well studied.

DESIGN: BNP was measured in 510 patients who were 6-18 years old in the Pediatric Heart Network Fontan cross-sectional study at a median of 8.2 years after Fontan. Patients underwent echocardiography, exercise testing, magnetic resonance imaging (MRI) and functional health status questionnaires. Associations of BNP with baseline patient characteristics, medical history and cross-sectional assessment were examined with multivariable linear regression analyses.

RESULTS: The distribution of BNP was highly skewed, median 13.0 pg/mL …


Real-World Comparison Of Two Molecular Methods For Detection Of Respiratory Viruses, Syed Asad Ali, James E. Gern, Tina V. Hartert, Kathryn M. Edwards, Marie R. Griffin, E Kathryn Mille, Tebeb Gebretsadik, Tressa Pappas, Wai Ming Lee, John V. Williams Jun 2011

Real-World Comparison Of Two Molecular Methods For Detection Of Respiratory Viruses, Syed Asad Ali, James E. Gern, Tina V. Hartert, Kathryn M. Edwards, Marie R. Griffin, E Kathryn Mille, Tebeb Gebretsadik, Tressa Pappas, Wai Ming Lee, John V. Williams

Department of Paediatrics and Child Health

Background: Molecular polymerase chain reaction (PCR) based assays are increasingly used to diagnose viral respiratory infections and conduct epidemiology studies. Molecular assays have generally been evaluated by comparing them to conventional direct fluorescent antibody (DFA) or viral culture techniques, with few published direct comparisons between molecular methods or between institutions. We sought to perform a real-world comparison of two molecular respiratory viral diagnostic methods between two experienced respiratory virus research laboratories.
Methods: We tested nasal and throat swab specimens obtained from 225 infants with respiratory illness for 11 common respiratory viruses using both a multiplex assay (Respiratory MultiCode-PLx Assay [RMA]) …


Surgical Management Of Complete Atrioventricular Septal Defect: Associations With Surgical Technique, Age, And Trisomy 21., Andrew M. Atz, John A. Hawkins, Minmin Lu, Meryl S. Cohen, Steven D. Colan, James Jaggers, Ronald V. Lacro, Brian W. Mccrindle, Renee Margossian, Ralph S. Mosca, Lynn A. Sleeper, L Luann Minich, Pediatric Heart Network Investigators, Girish S. Shirali Jun 2011

Surgical Management Of Complete Atrioventricular Septal Defect: Associations With Surgical Technique, Age, And Trisomy 21., Andrew M. Atz, John A. Hawkins, Minmin Lu, Meryl S. Cohen, Steven D. Colan, James Jaggers, Ronald V. Lacro, Brian W. Mccrindle, Renee Margossian, Ralph S. Mosca, Lynn A. Sleeper, L Luann Minich, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes.

METHODS: The demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006.

RESULTS: The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect …


Renin-Angiotensin-Aldosterone Genotype Influences Ventricular Remodeling In Infants With Single Ventricle., Seema Mital, Wendy K. Chung, Steven D. Colan, Lynn A. Sleeper, Cedric Manlhiot, Cammon B. Arrington, James F. Cnota, Eric M. Graham, Michael E. Mitchell, Elizabeth Goldmuntz, Jennifer S. Li, Jami C. Levine, Teresa M. Lee, Renee Margossian, Daphne T. Hsu, Pediatric Heart Network Investigators, Girish S. Shirali May 2011

Renin-Angiotensin-Aldosterone Genotype Influences Ventricular Remodeling In Infants With Single Ventricle., Seema Mital, Wendy K. Chung, Steven D. Colan, Lynn A. Sleeper, Cedric Manlhiot, Cammon B. Arrington, James F. Cnota, Eric M. Graham, Michael E. Mitchell, Elizabeth Goldmuntz, Jennifer S. Li, Jami C. Levine, Teresa M. Lee, Renee Margossian, Daphne T. Hsu, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

Background: We investigated the effect of polymorphisms in the renin-angiotensin-aldosterone system (RAAS) genes on ventricular remodeling, growth, renal function, and response to enalapril in infants with single ventricle.

Methods and results: Single ventricle infants enrolled in a randomized trial of enalapril were genotyped for polymorphisms in 5 genes: angiotensinogen, angiotensin-converting enzyme, angiotensin II type 1 receptor, aldosterone synthase, and chymase. Alleles associated with renin-angiotensin-aldosterone system upregulation were classified as risk alleles. Ventricular mass, volume, somatic growth, renal function using estimated glomerular filtration rate, and response to enalapril were compared between patients with ≥2 homozygous risk genotypes (high risk), and those …


Natural History Study Of Arthrogryposis Multiplex Congenita, Amyoplasia Type, Trisha Nichols May 2011

Natural History Study Of Arthrogryposis Multiplex Congenita, Amyoplasia Type, Trisha Nichols

Dissertations and Theses (Open Access)

Arthrogryposis or Arthrogrypsosis Multiplex Congenita (AMC) are terms used to describe the clinical finding of multiple congenital contractures. There are more than 300 distinct disorders associated with arthrogryposis. Amyoplasia is the most common type of arthrogryposis and is often referred to as the “classic” type. There is no known cause of amyoplasia and no risk factors have been identified. Moreover, there is no established diagnostic criteria, which has led to inconsistency and confusion in the medical literature. The purpose of this study was to describe the natural history of amyoplasia, to determine if there are any identifiable risk factors and …


Evaluation Of Kawasaki Disease Risk-Scoring Systems For Intravenous Immunoglobulin Resistance., Lynn A. Sleeper, L Luann Minich, Brian M. Mccrindle, Jennifer S. Li, Wilbert Mason, Steven D. Colan, Andrew M. Atz, Beth F. Printz, Annette Baker, Victoria L. Vetter, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali May 2011

Evaluation Of Kawasaki Disease Risk-Scoring Systems For Intravenous Immunoglobulin Resistance., Lynn A. Sleeper, L Luann Minich, Brian M. Mccrindle, Jennifer S. Li, Wilbert Mason, Steven D. Colan, Andrew M. Atz, Beth F. Printz, Annette Baker, Victoria L. Vetter, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment.

STUDY DESIGN: We used data from a randomized trial of pulsed steroids for primary treatment of Kawasaki disease to assess operating characteristics of the 3 risk scores, and we examined whether steroid therapy lowers the risk of coronary artery abnormalities in patients prospectively classified as IVIG resistant.

RESULTS: For comparability with published cohorts, we analyzed the data of 99 patients who were not treated with steroids (16% IVIG-retreated) and identified male sex, lower …


Preventive Zinc Supplementation In Developing Countries: Impact On Mortality And Morbidity Due To Diarrhea, Pneumonia And Malaria, Mohammad Yawar Yakoob, Evropi Theodoratou, Afshan Jabeen, Aamer Imdad, Thomas P. Eisele, Joy Ferguson, Arnoupe Jhass, Igor Rudan, Harry Campbell, Robert E. Black, Zulfiqar Ahmed Bhutta Apr 2011

Preventive Zinc Supplementation In Developing Countries: Impact On Mortality And Morbidity Due To Diarrhea, Pneumonia And Malaria, Mohammad Yawar Yakoob, Evropi Theodoratou, Afshan Jabeen, Aamer Imdad, Thomas P. Eisele, Joy Ferguson, Arnoupe Jhass, Igor Rudan, Harry Campbell, Robert E. Black, Zulfiqar Ahmed Bhutta

Woman and Child Health

Background:Zinc deficiency is commonly prevalent in children in developing countries and plays a role in decreased immunity and increased risk of infection. Preventive zinc supplementation in healthy children can reduce mortality due to common causes like diarrhea, pneumonia and malaria. The main objective was to determine all-cause mortality and cause-specific mortality and morbidity in children under five in developing countries for preventive zinc supplementation. Data sources/review methods: A literature search was carried out on PubMed, the Cochrane Library and the WHO regional databases to identify RCTs on zinc supplementation for greater than 3 months in children less than 5 …


Noncoronary Cardiac Abnormalities Are Associated With Coronary Artery Dilation And With Laboratory Inflammatory Markers In Acute Kawasaki Disease., Beth F. Printz, Lynn A. Sleeper, Jane W. Newburger, L Luann Minich, Timothy Bradley, Meryl S. Cohen, Deborah Frank, Jennifer S. Li, Renee Margossian, Girish S. Shirali, Masato Takahashi, Steven D. Colan, Pediatric Heart Network Investigators Jan 2011

Noncoronary Cardiac Abnormalities Are Associated With Coronary Artery Dilation And With Laboratory Inflammatory Markers In Acute Kawasaki Disease., Beth F. Printz, Lynn A. Sleeper, Jane W. Newburger, L Luann Minich, Timothy Bradley, Meryl S. Cohen, Deborah Frank, Jennifer S. Li, Renee Margossian, Girish S. Shirali, Masato Takahashi, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: We explored the association of noncoronary cardiac abnormalities with coronary artery dilation and with laboratory inflammatory markers early after Kawasaki disease (KD) diagnosis.

BACKGROUND: Left ventricular (LV) dysfunction, mitral regurgitation (MR), and aortic root dilation occur early after diagnosis; their associations with coronary artery dilation and inflammatory markers have not been well-described.

METHODS: Centrally interpreted echocardiograms were obtained at KD diagnosis and 1 and 5 weeks after diagnosis on 198 subjects in the National Institutes of Health-sponsored Pediatric Heart Network KD pulsed steroid trial. Regression models were constructed to investigate the relationships among early LV dysfunction, MR, and aortic …


No Significant Change In Sexual Behavior In Association With Human Papilloma Virus Vaccination In Young Girls, Wafa R.R. Al Romaih, Archana Srinivas, Said Shahtahmasebi, Hatim A. Omar Jan 2011

No Significant Change In Sexual Behavior In Association With Human Papilloma Virus Vaccination In Young Girls, Wafa R.R. Al Romaih, Archana Srinivas, Said Shahtahmasebi, Hatim A. Omar

Pediatrics Faculty Publications

The first human Papilloma virus (HPV) vaccine was approved in the United States in 2006 with the potential to reduce cervical cancer and genital warts. Since then, its efficacy in preventing HPV-related cancers in both males and females has been promising. Despite CDC recommendations, opponents of the vaccine assert that vaccinating pre-adolescents and adolescents will increase their sexual activity, as well as overtly condone risky sexual behavior. We analyzed clinic data of 499 adolescents with a mean age of 16 years to explore whether vaccination led to change in sexual behavior after one year. Our results showed no statistically significant …


Are Proton Pump Inhibitors A Safe And Effective Treatment For Gastroesophageal Reflux Disease In Infants Less Than Twelve Months Old?, Mariah Smith Jan 2011

Are Proton Pump Inhibitors A Safe And Effective Treatment For Gastroesophageal Reflux Disease In Infants Less Than Twelve Months Old?, Mariah Smith

PCOM Physician Assistant Studies Student Scholarship

OBJECTIVE: The objective of this systematic review is to determine whether or not proton pump inhibitors are a safe and effective treatment for gastroesophageal reflux disease in infants less than twelve months old.


The Effectiveness And Safety Of Antibiotic Treatment In Healing Tympanic Membrane Perforation In Aboriginal Children, Shannon M. Rice Jan 2011

The Effectiveness And Safety Of Antibiotic Treatment In Healing Tympanic Membrane Perforation In Aboriginal Children, Shannon M. Rice

PCOM Physician Assistant Studies Student Scholarship

The objective of this systematic review is to determine whether or not antibiotic treatment is safe and effective in healing tympanic membrane (TM) perforation in Aboriginal children diagnosed with chronic suppurative otitis media and otitis media with effusion less than 16 years of age.


How Safe And Effective Is Infliximab In The Treatment Of Children With Moderate To Severe Crohn’S Disease?, Jackie Linh Jan 2011

How Safe And Effective Is Infliximab In The Treatment Of Children With Moderate To Severe Crohn’S Disease?, Jackie Linh

PCOM Physician Assistant Studies Student Scholarship

Objective: The objective of this systematic review is to determine whether or not “how safe and effective is infliximab in treatment of children with moderate to severe Crohn’s disease?”


Predictors Of Coronary Artery Visualization In Kawasaki Disease., Renee Margossian, Minmin Lu, L Luann Minich, Timothy J. Bradley, Meryl S. Cohen, Jennifer S. Li, Beth F. Printz, Girish S. Shirali, Lynn A. Sleeper, Jane W. Newburger, Steven D. Colan, Pediatric Heart Network Investigators Jan 2011

Predictors Of Coronary Artery Visualization In Kawasaki Disease., Renee Margossian, Minmin Lu, L Luann Minich, Timothy J. Bradley, Meryl S. Cohen, Jennifer S. Li, Beth F. Printz, Girish S. Shirali, Lynn A. Sleeper, Jane W. Newburger, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Background: Echocardiography is the imaging modality of choice for the evaluation of coronary artery (CA) abnormalities in Kawasaki disease. Small series have established high specificity and sensitivity for detecting abnormalities, yet visualization rates of individual CA segments and factors associated with success are unknown.

Methods: In the Pediatric Heart Network's randomized trial of primary steroid treatment for Kawasaki disease, echocardiograms were interpreted locally and by a core laboratory. Univariate and multivariate predictors of CA visualization by the local lab as determined by the core lab were explored, and agreement of CA size measured locally and by the core lab was …


Overuse Of Antibiotics In Children For Upper Respiratory Infections (Uris): A Dilemma, Malika Taufiq, Rukhsana Wamiq Zuberi Jan 2011

Overuse Of Antibiotics In Children For Upper Respiratory Infections (Uris): A Dilemma, Malika Taufiq, Rukhsana Wamiq Zuberi

Department for Educational Development

No abstract provided.


Relation Of Size Of Secondary Ventricles To Exercise Performance In Children After Fontan Operation., Ashwin Prakash, Thomas G. Travison, Mark A. Fogel, Lynne M. Hurwitz, Andrew J. Powell, Beth F. Printz, Michael D. Puchalski, Girish S. Shirali, Shi-Joon Yoo, Tal Geva, Pediatric Heart Network Investigators Dec 2010

Relation Of Size Of Secondary Ventricles To Exercise Performance In Children After Fontan Operation., Ashwin Prakash, Thomas G. Travison, Mark A. Fogel, Lynne M. Hurwitz, Andrew J. Powell, Beth F. Printz, Michael D. Puchalski, Girish S. Shirali, Shi-Joon Yoo, Tal Geva, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

The effects of the nondominant or secondary ventricle on the Fontan circulation are not known. The present study used cardiac magnetic resonance imaging to investigate the relations between secondary ventricular size and global cardiac performance. The Fontan cross-sectional study collected data from 7 centers participating in the Pediatric Heart Network. Subjects with complete cardiac magnetic resonance imaging data and an identifiable secondary ventricle were included in the analysis. Relationships between body surface area-adjusted parameters of the secondary ventricle (mass, end-diastolic volume, mass/volume ratio, and stroke volume) and the following measures were assessed. These measures included the percentage of predicted peak …


Prenatal Diagnosis And Risk Factors For Preoperative Death In Neonates With Single Right Ventricle And Systemic Outflow Obstruction: Screening Data From The Pediatric Heart Network Single Ventricle Reconstruction Trial(∗)., Andrew M. Atz, Thomas G. Travison, Ismee A. Williams, Gail D. Pearson, Peter C. Laussen, William T. Mahle, Amanda L. Cook, Joel A. Kirsh, Mark Sklansky, Svetlana Khaikin, Caren Goldberg, Michele Frommelt, Catherine Krawczeski, Michael D. Puchalski, Jeffrey P. Jacobs, Jeanne M. Baffa, Jack Rychik, Richard G. Ohye, Pediatric Heart Network Investigators, Girish S. Shirali Dec 2010

Prenatal Diagnosis And Risk Factors For Preoperative Death In Neonates With Single Right Ventricle And Systemic Outflow Obstruction: Screening Data From The Pediatric Heart Network Single Ventricle Reconstruction Trial(∗)., Andrew M. Atz, Thomas G. Travison, Ismee A. Williams, Gail D. Pearson, Peter C. Laussen, William T. Mahle, Amanda L. Cook, Joel A. Kirsh, Mark Sklansky, Svetlana Khaikin, Caren Goldberg, Michele Frommelt, Catherine Krawczeski, Michael D. Puchalski, Jeffrey P. Jacobs, Jeanne M. Baffa, Jack Rychik, Richard G. Ohye, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: The purpose of this analysis was to assess preoperative risk factors before the first-stage Norwood procedure in infants with hypoplastic left heart syndrome and related single-ventricle lesions and to evaluate practice patterns in prenatal diagnosis, as well as the role of prenatal diagnosis in outcome.

METHODS: Data from all live births with morphologic single right ventricle and systemic outflow obstruction screened for the Pediatric Heart Network's Single Ventricle Reconstruction Trial were used to investigate prenatal diagnosis and preoperative risk factors. Demographics, gestational age, prenatal diagnosis status, presence of major extracardiac congenital abnormalities, and preoperative mortality rates were recorded.

RESULTS: …


Should We Be Concerned About Jejunoileal Atresia During Repair Of Duodenal Atresia?, Shawn D. St Peter, Danny C. Little, Katherine A. Barsness, Daniel R. Copeland, Casey M. Calkins, Suzanne Yoder, Steve S. Rothenberg, Saleem Islam, Kuojen Tsao, Daniel J. Ostlie Nov 2010

Should We Be Concerned About Jejunoileal Atresia During Repair Of Duodenal Atresia?, Shawn D. St Peter, Danny C. Little, Katherine A. Barsness, Daniel R. Copeland, Casey M. Calkins, Suzanne Yoder, Steve S. Rothenberg, Saleem Islam, Kuojen Tsao, Daniel J. Ostlie

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: During repair for duodenal atresia, it has been emphasized that inspection of the small bowel to identify a second atresia is required. The laparoscopic approach for repair of duodenal atresia has been criticized for its limitation to perform this step. Given that duodenal atresia and jejunoileal atresias do not share common embryologic origins, we question the validity of this concern. Therefore, we conducted a multicenter retrospective review of duodenal atresia patients to quantify the incidence of jejunoileal atresia in this population.

METHODS: After institutional review board approval (IRB #07-12-187X), a retrospective review was conducted on all patients who have …


Childhood Injuries In Pakistan: Results From Two Communities, Seema Zainulabdin Lasi, Ghazala Rafique, Habib Peermohammad Aug 2010

Childhood Injuries In Pakistan: Results From Two Communities, Seema Zainulabdin Lasi, Ghazala Rafique, Habib Peermohammad

Human Development Programme

The study aimed at determining the incidence, nature, and extent of childhood injuries in two suburban and rural communities of Pakistan. The findings of the study are based on a cross-sectional survey of 2,292 children aged 1-8 years. Information was sought retrospectively from the primary caregiver on the occurrence of injury that required formal or informal medical consultation during the past three months. The incidence rate of non-fatal injuries that required care outside home for children aged 1-8 years was 19.7 injuries per 100 person (child)-years of exposure [95% confidence interval (CI) 16.41-23.51]: 26.5 injuries per 100 person (child)-years of …


Single Lung Ventilation In A Young Child For Repair Of Coarctation Of Aorta, Mohammad Hamid, Mansoor Ahmed Khan, Muneer Amanullah Aug 2010

Single Lung Ventilation In A Young Child For Repair Of Coarctation Of Aorta, Mohammad Hamid, Mansoor Ahmed Khan, Muneer Amanullah

Department of Anaesthesia

Single lung ventilation in small children is extremely challenging. In this case, a paediatric bronchial blocker was successfully inserted in a 19-month-old child to provide single lung ventilation using a modified insertion technique. It provided excellent working conditions during thoracotomy and our method of insertion may help in reducing the cost.


Enalapril In Infants With Single Ventricle: Results Of A Multicenter Randomized Trial., Daphne T. Hsu, Victor Zak, Lynn Mahony, Lynn A. Sleeper, Andrew M. Atz, Jami C. Levine, Piers C. Barker, Chitra Ravishankar, Brian W. Mccrindle, Richard V. Williams, Karen Altmann, Nancy S. Ghanayem, Renee Margossian, Wendy K. Chung, William L. Border, Gail D. Pearson, Mario P. Stylianou, Seema Mital, Pediatric Heart Network Investigators, Girish S. Shirali Jul 2010

Enalapril In Infants With Single Ventricle: Results Of A Multicenter Randomized Trial., Daphne T. Hsu, Victor Zak, Lynn Mahony, Lynn A. Sleeper, Andrew M. Atz, Jami C. Levine, Piers C. Barker, Chitra Ravishankar, Brian W. Mccrindle, Richard V. Williams, Karen Altmann, Nancy S. Ghanayem, Renee Margossian, Wendy K. Chung, William L. Border, Gail D. Pearson, Mario P. Stylianou, Seema Mital, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Angiotensin-converting enzyme inhibitor therapy improves clinical outcome and ventricular function in adults with heart failure. Infants with single-ventricle physiology have poor growth and are at risk for abnormalities in ventricular systolic and diastolic function. The ability of angiotensin-converting enzyme inhibitor therapy to preserve ventricular function and improve somatic growth and outcomes in these infants is unknown.

METHODS AND RESULTS: The Pediatric Heart Network conducted a double-blind trial involving 230 infants with single-ventricle physiology randomized to receive enalapril (target dose 0.4 mg . kg(-1) . d(-1)) or placebo who were followed up until 14 months of age. The primary end …


Initial Experience With A Miniaturized Multiplane Transesophageal Probe In Small Infants Undergoing Cardiac Operations., Sinai C. Zyblewski, Girish S. Shirali, Geoffrey A. Forbus, Tain-Yen Hsia, Scott M. Bradley, Andrew M. Atz, Meryl S. Cohen, Eric M. Graham Jun 2010

Initial Experience With A Miniaturized Multiplane Transesophageal Probe In Small Infants Undergoing Cardiac Operations., Sinai C. Zyblewski, Girish S. Shirali, Geoffrey A. Forbus, Tain-Yen Hsia, Scott M. Bradley, Andrew M. Atz, Meryl S. Cohen, Eric M. Graham

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: There has been reluctance to use intraoperative transesophageal echocardiography (TEE) in small infants. We assessed the utility and safety of a new miniaturized multiplane micro-TEE probe in small infants undergoing cardiac operations.

DESCRIPTION: Hemodynamic and ventilation variables were prospectively recorded before and after micro-TEE insertion and removal in infants weighing 5 kg or less undergoing cardiac operations.

EVALUATION: The study included 42 patients with a mean weight of 3.6 +/- 0.9 kg (range, 1.7 to 5 kg). All probe insertions were successful. There were no complications or clinically significant changes in hemodynamic or ventilation variables. Information provided by TEE …


Evaluating The Impact Of The Guatemalan Nursing Program On Staff, Organizational, And Clinical Outcomes, Sara Williamson Day May 2010

Evaluating The Impact Of The Guatemalan Nursing Program On Staff, Organizational, And Clinical Outcomes, Sara Williamson Day

Theses and Dissertations (ETD)

There is great disparity between the outcome of childhood cancer in developed and developing countries. Nurses, who comprise the largest group of health care professionals globally, are essential to successful treatment. Pediatric oncology education is generally unavailable for nurses in developing countries. This lack of education is likely to contribute to the disparity in survival rates, as undereducated nurses are unable to meet the demands of pediatric cancer care. A second critical problem in developing countries is the workload of the nurse, with a patient nurse ratio reported as high as 30 patients per one nurse.

In 2006, the quality …


Acute Necrotizing Encephalopathy Of Childhood: A Fatal Complication Of Swine Flu, Muhammad Rehan Khan, Prem Kumar Maheshwari, Syed Asad Ali, Anwarul Haque Feb 2010

Acute Necrotizing Encephalopathy Of Childhood: A Fatal Complication Of Swine Flu, Muhammad Rehan Khan, Prem Kumar Maheshwari, Syed Asad Ali, Anwarul Haque

Department of Paediatrics and Child Health

Acute necrotizing encephalopathy of childhood (ANEC) is a rare condition characterized by the presence of multifocal symmetrical brain lesions involving mainly thalami, brainstem, cerebellum and white matter. ANEC is a serious and life threatening complication of simple viral infections. We present a case of a young child who developed this condition with classical clinical and radiological findings consistent with ANEC, secondary to swine flu (H1N1). He needed ventilatory support and had profound motor and intellectual deficit on discharge. We report this case with aim of raising awareness about this fatal complication of swine flu which has become a global health …


Partial And Transitional Atrioventricular Septal Defect Outcomes., L Luann Minich, Andrew M. Atz, Steven D. Colan, Lynn A. Sleeper, Seema Mital, James Jaggers, Renee Margossian, Ashwin Prakash, Jennifer S. Li, Meryl S. Cohen, Ronald V. Lacro, Gloria L. Klein, John A. Hawkins, Pediatric Heart Network Investigators, Girish S. Shirali Feb 2010

Partial And Transitional Atrioventricular Septal Defect Outcomes., L Luann Minich, Andrew M. Atz, Steven D. Colan, Lynn A. Sleeper, Seema Mital, James Jaggers, Renee Margossian, Ashwin Prakash, Jennifer S. Li, Meryl S. Cohen, Ronald V. Lacro, Gloria L. Klein, John A. Hawkins, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Surgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort.

METHODS: We studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change.

RESULTS: Median age at surgery was 1.8 years; median weight z-score was -0.88. Median days for ventilation were 1, intensive …


Hereditary 1,25-Dihydroxyvitamin D-Resistant Rickets With Alopecia Resulting From A Novel Missense Mutation In The Dna-Binding Domain Of The Vitamin D Receptor., Peter J. Malloy, Jining Wang, Tarak Srivastava, David Feldman Jan 2010

Hereditary 1,25-Dihydroxyvitamin D-Resistant Rickets With Alopecia Resulting From A Novel Missense Mutation In The Dna-Binding Domain Of The Vitamin D Receptor., Peter J. Malloy, Jining Wang, Tarak Srivastava, David Feldman

Manuscripts, Articles, Book Chapters and Other Papers

The rare genetic recessive disease, hereditary vitamin D resistant rickets (HVDRR), is caused by mutations in the vitamin D receptor (VDR) that result in resistance to the active hormone 1,25-dihydroxyvitamin D(3) (1,25(OH)(2)D(3) or calcitriol). In this study, we examined the VDR from a young boy with clinical features of HVDRR including severe rickets, hypocalcemia, hypophosphatemia and partial alopecia. The pattern of alopecia was very unusual with areas of total baldness, adjacent to normal hair and regions of scant hair. The child failed to improve on oral calcium and vitamin D therapy but his abnormal chemistries and his bone X-rays normalized …


Evaluating Children With Otitis Media For Bacteremia Or Urinary Tract Infection., Daniel Yawman, Patrick Mahar, Aaron Blumkin, Gregory P. Conners Jan 2010

Evaluating Children With Otitis Media For Bacteremia Or Urinary Tract Infection., Daniel Yawman, Patrick Mahar, Aaron Blumkin, Gregory P. Conners

Manuscripts, Articles, Book Chapters and Other Papers

Background. It is unclear if clinicians evaluate for concurrent bacteremia or UTI in young patients diagnosed with acute otitis media (AOM). Objectives. To describe how often, and under which circumstances, emergency providers investigate for bacteremia or UTI in 2-36 month olds with AOM. Methods. Cases of AOM were analyzed from the 2001-2004 National Hospital Ambulatory Medical Care Survey (NHAMCS)-Emergency Department dataset. Results. AOM was diagnosed in 17% of the 10,847 recorded visits of 2-36 month olds. Of these visits, laboratory testing included: CBC: 7%, Blood culture: 4%, urinalysis or urine culture: 5%, and any of these tests: 9%. Rates of …


Clinico-Morphological Correlations In The Categorization Of Holes Between The Ventricles., Brad A. Friedman, Anthony Hlavacek, Karen Chessa, Girish S. Shirali, Eowyn Corcrain, Diane Spicer, Robert H. Anderson, Sinai Zyblewski Jan 2010

Clinico-Morphological Correlations In The Categorization Of Holes Between The Ventricles., Brad A. Friedman, Anthony Hlavacek, Karen Chessa, Girish S. Shirali, Eowyn Corcrain, Diane Spicer, Robert H. Anderson, Sinai Zyblewski

Manuscripts, Articles, Book Chapters and Other Papers

Controversy still exists in the categorization of holes between the ventricles, although they are the most common congenital cardiac malformation. Advanced imaging techniques such as three-dimensional echocardiography and computed tomographic angiography offer superb anatomical details of these defects. In this review, we have sought to collate the features highlighted in different categorizations and identify their similarities, but also emphasize their differences. We hope that an analysis of this type, now achievable during life, using advanced imaging, might lead to the appearance of a unified system for diagnosis and description of holes between the ventricles.