Pediatrics Commons^™

The Impact Of Family History On Medullary Thyroid Cancer In Men2a Patients, Nicole D. Mohrbacher

Dissertations and Theses (Open Access)

The American Thyroid Association recently classified all MEN2A-associated codons into increasing risk levels A-C and stated that some patients may delay prophylactic thyroidectomy if certain criteria are met. One criterion is a less aggressive family history of MTC but whether families with the same mutated codon have variable MTC aggressiveness is not well described. We developed several novel measures of MTC aggressiveness and compared families with the same mutated codon to determine if there is significant inter-familial variability. Pedigrees of families with MEN2A were reviewed for codon mutated and proportion of RET mutation carriers with MTC. Individuals with MTC were …

Effects Of Perinatal Oxycodone Exposure On The Cardiovascular Response To Acute Stress In Male Rats At Weaning And In Young Adulthood, Thitinart Sithisarn, Henrietta S. Bada, Richard J. Charnigo, Sandra J. Legan, David C. Randall

Pediatrics Faculty Publications

Oxycodone (OXY) is one of the most commonly abused opiates during pregnancy. Perinatal opiate exposure (POE) is associated with neurobehavioral and hormone changes. Little is known about the effects of perinatal OXY on the cardiovascular (CV) responses to stress.

Objectives: to determine the effects of POE on: (1) CV responses to acute stress and ability to discriminate using a classical conditioning paradigm; (2) changes in CV response to the paradigm and retention of the ability to discriminate from postnatal day (PD) 40 to young adulthood.

Methods: Pregnant rats were given i.v. OXY or vehicle (CON) daily. OXY and CON males …

Coping With Pediatric Cancer: Conversational Methods Utilized By Parents And Children When Dealing With Pediatric Cancer, Chelsi Morgan Walls

Graduate Research Symposium (2010 - 2017)

This thesis sought to analyze how parents communicate with their child regarding pediatric cancer treatments. When dealing with pediatric cancer, it is vital that parents and their child communicate about the illness in order to effectively cope with the cancer. Using Uncertainty Management Theory, along with sub-concepts of the theory (i.e., appraisals, inferences, and illusions), this thesis sought to discover which coping mechanism (i.e., affect-management or buffering) would be chosen to manage the illness. Under UMT, appraisals were assessed, resulting in positive and negative appraisal, which indicated whether the individual handled the issue with either an inference based on general …

Better Drug Therapy For The Children Of Africa: Current Impediments To Success And Potential Strategies For Improvement, Stuart M. Macleod, Janet K. Finch, William Macharia, Gabriel M. Anabwani

Paediatrics and Child Health, East Africa

A commentary is presented on the urgent need for a comprehensive effort to improve the practice of pediatric therapeutics in Africa. A call for action is addressed to a variety of practitioners internationally, many of whom possess skills that could be fruitfully applied to the improvement of health outcomes for African children. Successful engagement with the many challenges requires the complementary effort of researchers in basic and clinical pharmacology and toxicology, nurses, pharmacists, physicians, clinical pharmacologists, clinical pharmacists, and political leaders and civil servants. While a comprehensive or systematic review of the relevant literature has not been attempted, the authors …

Caveolar Fatty Acids And Acylation Of Caveolin-1, Qian Cai, Ling Guo, Haiqing Gao, Xiang-An Li

Pediatrics Faculty Publications

PURPOSE: Caveolae are cholesterol and sphingolipids rich subcellular domains on plasma membrane. Caveolae contain a variety of signaling proteins which provide platforms for signaling transduction. In addition to enriched with cholesterol and sphingolipids, caveolae also contain a variety of fatty acids. It has been well-established that acylation of protein plays a pivotal role in subcellular location including targeting to caveolae. However, the fatty acid compositions of caveolae and the type of acylation of caveolar proteins remain largely unknown. In this study, we investigated the fatty acids in caveolae and caveolin-1 bound fatty acids.

METHODS: Caveolae were isolated from Chinese hamster …

Interventions To Reduce Neonatal Mortality From Neonatal Tetanus In Low And Middle Income Countries - A Systematic Review, Adeel Ahmed Khan, Aysha Zahidie, F. Rabbani

Community Health Sciences

Background

In 1988, WHO estimated around 787,000 newborns deaths due to neonatal tetanus. Despite few success stories majority of the Low and Middle Income Countries (LMICs) are still struggling to reduce neonatal mortality due to neonatal tetanus. We conducted a systematic review to understand the interventions that have had a substantial effect on reducing neonatal mortality rate due to neonatal tetanus in LMICs and come up with feasible recommendations for decreasing neonatal tetanus in the Pakistani setting.

Methods

We systemically reviewed the published literature (Pubmed and Pubget databases) to identify appropriate interventions for reducing tetanus related neonatal mortality. A total …

Disseminating Childhood Home Injury Risk Reduction Information In Pakistan: Results From A Community-Based Pilot Study, Aruna Chandran, Uzma Rahim Khan, Nukhba Zia, Asher Feroze, Sarah Stewart De Ramirez, Cheng-Ming Huang, Junaid A. Razzak, Adnan A. Hyder

Department of Emergency Medicine

Background: Most childhood unintentional injuries occur in the home; however, very little home injury prevention information is tailored to developing countries. Utilizing our previously developed information dissemination tools and a hazard assessment checklist tailored to a low-income neighborhood in Pakistan, we pilot tested and compared the effectiveness of two dissemination tools.

Methods: Two low-income neighborhoods were mapped, identifying families with a child aged between 12 and 59 months. In June and July 2010, all enrolled households underwent a home hazard assessment at the same time hazard reduction education was being given using an in-home tutorial or a pamphlet. …

School-Based Injury Outcomes In Children From A Low-Income Setting: Results From The Pilot Injury Surveillance In Rawalpindi City, Pakistan, Uzma Rahim Khan, Junaid A. Bhatti, Nukhba Zia, Umar Farooq

Department of Emergency Medicine

Background

School-based injuries account for one in five unintentional childhood injuries. Little is known about the epidemiology of school-based injuries in low-income settings. The objective of our study was to compare emergency department (ED) outcomes of the school-based injuries with respect to age, sex, and injury mechanisms in a Pakistani urban setting.

Findings

A pilot injury surveillance study was conducted at the EDs of three major tertiary-care hospitals of Rawalpindi city from July 2007 to June 2008 and included children of less than 15 years injured at school. The World Health Organization’s questionnaire for injury surveillance was used. There were …

Microglia-Derived Tnfα Induces Apoptosis In Neural Precursor Cells Via Transcriptional Activation Of The Bcl-2 Family Member Puma, J. Guadagno, X. Xu, M. Karajgikar, A. Brown, S.P. Cregan

Paediatrics Publications

Neuroinflammation is a common feature of acute neurological conditions such as stroke and spinal cord injury, as well as neurodegenerative conditions such as Parkinson’s disease, Alzheimer’s disease, and amyotrophic lateral sclerosis. Previous studies have demonstrated that acute neuroinflammation can adversely affect the survival of neural precursor cells (NPCs) and thereby limit the capacity for regeneration and repair. However, the mechanisms by which neuroinflammatory processes induce NPC death remain unclear. Microglia are key mediators of neuroinflammation and when activated to induce a pro-inflammatory state produce a number of factors that could affect NPC survival. Importantly, in the present study we demonstrate …

Focused Abdominal Sonography For Trauma (Fast) In Blunt Paediatric Abdominal Trauma, Ahmad Vaqas Faruque, Saqib Hamid Qazi, Muhammad Arif Mateen Khan, Wassem Akhtar, Amina Majeed

Section of Paediatric Surgery

Objective: To evaluate the role of focussed abdominal sonography for trauma in blunt paediatric abdominal trauma patients, and to see if the role of computed tomography scan could be limited to only those cases in which sonography was positive.
Methods: The retrospective study covered 10 years, from January 1,2000 to December 31,2009, and was conducted at the Department of Radiology and Department of Emergency Medicine, Aga Khan University Hospital, Karachi. It comprised cases of 174 children from birth to 14 years who had presented with blunt abdominal trauma and had focussed abdominal sonography for trauma done at the hospital. The …

The Young And Suicide, Hatim A. Omar, Joav Merrick

Pediatrics Faculty Publications

No abstract provided.

Lps And Pan-Induced Podocyte Injury In An In Vitro Model Of Minimal Change Disease: Changes In Tlr Profile., Tarak Srivastava, Mukut Sharma, Kok-Hooi Yew, Ram Sharma, R Scott Duncan, Moin A. Saleem, Ellen T. Mccarthy, Alexander Kats, Patricia A. Cudmore, Uri S. Alon, Christopher J. Harrison

Manuscripts, Articles, Book Chapters and Other Papers

Minimal change disease (MCD), the most common idiopathic nephrotic syndrome in children, is characterized by proteinuria and loss of glomerular visceral epithelial cell (podocyte) ultrastructure. Lipopolysaccharide (LPS) and puromycin aminonucleoside (PAN) are used to study podocyte injury in models of MCD in vivo and in vitro. We hypothesized that LPS and PAN influence components of the innate immune system in podocytes such as the Toll-Like Receptor (TLRs), TLR adapter molecules, and associated cytokines. Our results show that cultured human podocytes constitutively express TLRs 1-6 and TLR-10, but not TLRs 7-9. LPS (25 μg/ml) or PAN (60 μg/ml) caused comparable derangement …

The Political Economy Of Undernutrition National Report: Pakistan, Shehla Zaidi, Zulfiqar Ahmed Bhutta, Shandana Khan Mohmand, Andres Mejia Acosta

Community Health Sciences

No abstract provided.

Prevalence Of Hypertension In Healthy School Children In Pakistan And Its Relationship With Body Mass Index, Proteinuria And Hematuria, Arshalooz Jamila Rahman, Farah Naz Qamar, Sohail Ashraf, Zubair Ali Khowaja, Shafaq Binte Tariq, Hira Naeem

Department of Paediatrics and Child Health

To determine the prevalence of high blood pressure (BP) in healthy school Pakistani children and its association with high body mass index (BMI), asymptomatic hematuria and proteinuria, we studied 661 public school children and measured their body weight, height and BP and urine dipstick for hematuria performed on a single occasion. Hypertension (BP >95 th centile) and pre-hypertension (BP >90 th centile) were defined based on the US normative BP tables. Over-weight and obesity were defined according to the World Health Organization (WHO) classification of BMI. The mean age of the children was 14 ± 1.3 years. The mean BMI …

Age-Related Accumulation Of T Cells With Markers Of Relatively Stronger Autoreactivity Leads To Functional Erosion Of T Cells., Zohreh Tatari-Calderone, Milica Stojakovic, Ramita Dewan, Gama L. Bouder, Dragana Jankovic, Stanislav Vukmanovic

Pediatrics Faculty Publications

Background

Thymic involution is a prominent characteristic of an aging immune system. When thymic function is reduced/absent, the peripheral T cell pool is subject to the laws of peripheral T cell homeostasis that favor survival/expansion of T cell receptors with relatively higher functional avidity for self-peptide/MHC complexes. Due to difficulties in assessing the TCR avidity in polyclonal population of T cells, it is currently not known whether high avidity T cells preferentially survive in aging individuals, and what impact this might have on the function of the immune system and development of autoimmune diseases.

Results

The phenotype of T cells …

Prenatal Diagnosis Of Long Qt Syndrome: Implications For Delivery Room And Neonatal Management, E. Anne Greene, Charles I. Berul, Mary T. Donofrio

Pediatrics Faculty Publications

This case describes the prenatal diagnosis and integrated peripartum management of a foetus with 2:1 atrioventricular block and torsade de pointes due to congenital long QT syndrome. The unique issues related to the detection of intrauterine conduction abnormalities and ventricular arrhythmias, along with the immediate postnatal care, have been described as an interesting teaching case with successful outcome.

Conditional Sox9 Ablation Reduces Chondroitin Sulfate Proteoglycan Levels And Improves Motor Function Following Spinal Cord Injury, William M. Mckillop, Magdalena Dragan, Andreas Schedl, Arthur Brown

Paediatrics Publications

Chondroitin sulfate proteoglycans (CSPGs) found in perineuronal nets and in the glial scar after spinal cord injury have been shown to inhibit axonal growth and plasticity. Since we have previously identified SOX9 as a transcription factor that upregulates the expression of a battery of genes associated with glial scar formation in primary astrocyte cultures, we predicted that conditional Sox9 ablation would result in reduced CSPG expression after spinal cord injury and that this would lead to increased neuroplasticity and improved locomotor recovery. Control and Sox9 conditional knock-out mice were subject to a 70 kdyne contusion spinal cord injury at thoracic …

Comparison Of Clinical And Csf Profiles In Children With Tuberculous And Pyogenic Meningitis; Role Of Csf Protein: Glucose Ratio As Diagnostic Marker Of Tuberculous Meningitis, Farah Naz Qamar, Arshalooz Jamila Rahman, Sania Iqbal, Khadija Humayun

Department of Paediatrics and Child Health

Objectives: To compare the clinical and laboratory features of tuberculous meningitis with bacterial meningitis and to determine if protein glucose ratio in the cerebrospinal fluid can be predictive of the condition.
Methods: It was a retrospective descriptive study at the Paediatric Ward of Aga Khan University Hospital, Karachi, for which records of 46 patients with tuberculous meningitis and 33 with bacterial meningitis were reviewed. All records related to the study period that was from January 2005 to October 2010. The records were retrieved in December 2010. Tuberculous meningitis was defined as illness > or = 14 days, basal enhancement or tuberculoma …

Renal Function And Proteinuria After Successful Immunosuppressive Therapies In Patients With Fsgs., Ronald J. Hogg, Aaron Friedman, Tom Greene, Milena Radeva, Milos N. Budisavljevic, Jennifer Gassman, Debbie S. Gipson, J Ashley Jefferson, Eunice G. John, Frederick J. Kaskel, Asha Moudgil, Marva Moxey-Mims, Luis A. Ortiz, Jeffrey R. Schelling, William Schnaper, Tarak Srivastava, Howard Trachtman, V Matti Vehaskari, Craig Wong, Robert P. Woronieki, Scott K. Van Why, Anna Zolotnitskaya

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND AND OBJECTIVES: In the FSGS Clinical Trial, 22 cyclosporine-treated and 20 mycophenolate/dexamethasone-treated patients experienced a complete or partial remission after 26 weeks, completed 52 weeks of treatment, and were studied through 78 weeks. Herein, changes in the urine protein/creatinine ratio (UP/C) and estimated GFR (eGFR) throughout the entire study period are defined.

DESIGN, SETTING, PARTICIPANTS, AND MEASUREMENTS: The FSGS Clinical Trial, which was conducted from November 2004 to January 2010, enrolled patients aged 2-40 years, with eGFR ≥40 ml/min per 1.73 m(2) and UP/C >1 mg/mg after ≥4 weeks of corticosteroid therapy. Both groups received lisinopril or losartan throughout …

Association Of Impaired Linear Growth And Worse Neurodevelopmental Outcome In Infants With Single Ventricle Physiology: A Report From The Pediatric Heart Network Infant Single Ventricle Trial., Chitra Ravishankar, Victor Zak, Ismee A. Williams, David C. Bellinger, J William Gaynor, Nancy S. Ghanayem, Catherine D. Krawczeski, Daniel J. Licht, Lynn Mahony, Jane W. Newburger, Victoria L. Pemberton, Richard V. Williams, Renee Sananes, Amanda L. Cook, Teresa Atz, Svetlana Khaikin, Daphne T. Hsu, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To describe neurodevelopmental outcomes in infants with single ventricle (SV) physiology and determine factors associated with worse outcomes.

STUDY DESIGN: Neurodevelopmental outcomes for infants with SV enrolled in a multicenter drug trial were assessed at 14 months of age using the Bayley Scales of Infant Development-II. Multivariable regression analysis was used to identify factors associated with worse outcomes.

RESULTS: Neurodevelopmental testing was performed at 14 ± 1 months in 170/185 subjects in the trial. Hypoplastic left heart syndrome was present in 59% and 75% had undergone the Norwood operation. Mean Psychomotor Developmental Index (PDI) and mental developmental index (MDI) …

Neuropsychiatric Effects Of Tenofovir In Comparison With Other Antiretroviral Drugs., Kathleen Ferrer, Natella Rakhmanina

Pediatrics Faculty Publications

Tenofovir is a widely used antiretroviral medication indicated to treat adults and children infected with HIV. Current guidelines for the management of HIV infection recommend tenofovir disoproxil fumarate (TDF) as a component of the preferred first-line combination antiretroviral therapy. The efficacy, tolerability, prolonged half-life allowing for once-daily administration, and availability as a component of several fixed-dose formulations make TDF an attractive choice for treatment-naive and treatment-experienced HIV-infected patients. TDF is also widely used as a component of postexposure prophylaxis in noninfected individuals. Most importantly, it has been recently approved for use as pre-exposure prophylaxis for noninfected adults and adolescents to …

Ihoc Summary For Providers - Improving Health Outcomes For Children In Maine And Vermont, Joanie Klayman Lcsw, Amy Dix, Kevin S. Flanigan Md

Population Health & Health Policy

In February 2010, Maine and Vermont were awarded a five-year child health quality improvement grant. The project focuses on using quality measures and information technology to improve health outcomes for children. The goal is to improve timely access to quality care for children who are insured by Medicaid.

Wound Care Challenges In Children And Adults With Spina Bifida: An Open-Cohort Study, Katherine Ottolini, Amy B. Harris, June K. Amling, Ann M. Kennelly, Leslie A. Phillips, Laura L. Tosi

Pediatrics Faculty Publications

Skin breakdown is a frequent concern for individuals with spina bifida. We explored wound incidence in patients with spina bifida and how it varies across a person's life span and functional neurologic level. We examined the settings in which skin breakdown most commonly occurred, looking for evidence of chronic, non-healing wounds. We also sought to develop criteria to improve wound monitoring. We identified reported wound episodes in an open-cohort study over a 13-year period, examining the hospital and outpatient clinical records of spina bifida patients at Children's National Medical Center (CNMC). Current age, age at wound presentation, sex, weight, functional …

The Association Of Cd81 Polymorphisms With Alloimmunization In Sickle Cell Disease, Zohreh Tatari-Calderone, Ryad Tamouza, Gama P. Lebouder, Ramita Dewan, Naomi L. Luban, Jacqueline Lasserre, Jacqueline Maury, Francois Lionnet, Rajagopal Krishnamoorthy, Robert Girot, Stanislav Vukmanovic

Pediatrics Faculty Publications

The goal of the present work was to identify the candidate genetic markers predictive of alloimmunization in sickle cell disease (SCD). Red blood cell (RBC) transfusion is indicated for acute treatment, prevention, and abrogation of some complications of SCD. A well-known consequence of multiple RBC transfusions is alloimmunization. Given that a subset of SCD patients develop multiple RBC allo-/autoantibodies, while others do not in a similar multiple transfusional setting, we investigated a possible genetic basis for alloimmunization. Biomarker(s) which predicts (predict) susceptibility to alloimmunization could identify patients at risk before the onset of a transfusion program and thus may have …

Activation Of Human Herpesvirus Replication By Apoptosis, Alka Prasad, Jil Remick, Steven L. Zeichner

Pediatrics Faculty Publications

A central feature of herpesvirus biology is the ability of herpesviruses to remain latent within host cells. Classically, exposure to inducing agents, like activating cytokines or phorbol esters that stimulate host cell signal transduction events, and epigenetic agents (e.g., butyrate) was thought to end latency. We recently showed that Kaposi's sarcoma-associated herpesvirus (KSHV, or human herpesvirus-8 [HHV-8]) has another, alternative emergency escape replication pathway that is triggered when KSHV's host cell undergoes apoptosis, characterized by the lack of a requirement for the replication and transcription activator (RTA) protein, accelerated late gene kinetics, and production of virus with decreased infectivity. Caspase-3 …

Oximetry Signal Processing Identifies Rem Sleep-Related Vulnerability Trait In Asthmatic Children, Geovanny F. Perez, Maria J. Gutierrez, Shehlanoor Huseni, Krishna Pancham, Carlos E. Rodriguez-Martinez, Cesar L. Nino, Gustavo Nino

Pediatrics Faculty Publications

Rationale. The sleep-related factors that modulate the nocturnal worsening of asthma in children are poorly understood. This study addressed the hypothesis that asthmatic children have a REM sleep-related vulnerability trait that is independent of OSA.

Methods. We conducted a retrospective cross-sectional analysis of pulse-oximetry signals obtained during REM and NREM sleep in control and asthmatic children (n=134). Asthma classification was based on preestablished clinical criteria. Multivariate linear regression model was built to control for potential confounders (significance level p ≤ 0.05).

Results. Our data demonstrated that (1) baseline nocturnal respiratory parameters were not significantly different in asthmatic versus control children, …

Low Plasma Citrulline Levels Are Associated With Acute Respiratory Distress Syndrome In Patients With Severe Sepsis, Lorraine B. Ware, Jordan A. Magarik, Nancy Wickersham, Gary Cunningham, Todd W. Rice, Brian W. Christman, Arthur P. Wheeler, Gordon R. Bernard, Marshall Summar

Pediatrics Faculty Publications

Introduction: The role of nitric oxide synthase (NOS) in the pathophysiology of acute respiratory distress syndrome (ARDS) is not well understood. Inducible NOS is upregulated during physiologic stress; however, if NOS substrate is insufficient then NOS can uncouple and switch from NO generation to production of damaging peroxynitrites. We hypothesized that NOS substrate levels are low in patients with severe sepsis and that low levels of the NOS substrate citrulline would be associated with end organ damage including ARDS in severe sepsis.

Methods: Plasma citrulline, arginine and ornithine levels and nitrate/nitrite were measured at baseline in 135 patients with severe …

Can Coronary Artery Involvement In Kawasaki Disease Be Predicted?, Sunil J. Ghelani, Neha Kwatra, Christopher F. Spurney

Pediatrics Faculty Publications

Background: Coronary artery involvement is seen in approximately 15–20% of children with Kawasaki disease. There is conflicting literature regarding the clinical and laboratory findings associated with coronary artery involvement. In this retrospective study, we attempt identification of predictive factors for coronary artery involvement at our institute and review the existing literature.

Methods and results: A review of 203 patients (65% males) with Kawasaki disease was performed, of whom 33 (16.3%) had coronary artery involvement. High erythrocyte sedimentation rate, high platelet count, low hematocrit, low albumin levels, and refractory Kawasaki disease showed significant association with coronary artery involvement. High …

A Randomized, Controlled Trial Of Catheter-Related Infectious Event Rates Using Antibiotic-Impregnated Catheters Versus Conventional Catheters In Pediatric Cardiovascular Surgery Patients, Elaine G. Cox, Chad A. Knoderer, Aimee Jennings, John W. Brown, Mark D. Rodefeld, Scott G. Walker, Mark W. Turrentine

Scholarship and Professional Work – COPHS

We conducted a randomized, controlled clinical trial to determine whether a difference in catheter-associated blood stream infection (CABSI) incidence existed between children who underwent cardiac surgery and had a central venous catheter impregnated with minocycline and rifampin versus those who had a conventional, nonimpregnated catheter after cardiac surgery. Due to a lower number of infections than expected, the study was terminated early. Among 288 evaluable patients, the rates of CABSI and line-related complications were similar between the 2 groups.

Evaluation Of Vancomycin Dosing And Corresponding Drug Concentrations In Neonatal Intensive Care Unit (Nicu) Patients, Christine Lam Pharmd, Jenny Boucher Bs, Pharmd, Jd, Bcps

Department of Pharmacy

No abstract provided.