Pediatrics Commons^™

Survival Motor Neuron Protein Deficiency Impairs Myotube Formation By Altering Myogenic Gene Expression And Focal Adhesion Dynamics, Katherine V. Bricceno, Tara Martinez, Evgenia Leikina, Stephanie Duguez, Terence A. Partridge, Leonid V. Chernomordik, Kenneth H. Fischbeck, Charlotte J. Sumner, Barrington G. Burnett

Pediatrics Faculty Publications

While spinal muscular atrophy (SMA) is characterized by motor neuron degeneration, it is unclear whether and how much survival motor neuron (SMN) protein deficiency in muscle contributes to the pathophysiology of the disease. There is increasing evidence from patients and SMA model organisms that SMN deficiency causes intrinsic muscle defects. Here we investigated the role of SMN in muscle development using muscle cell lines and primary myoblasts. Formation of multinucleate myotubes by SMN-deficient muscle cells is inhibited at a stage preceding plasma membrane fusion. We found increased expression and reduced induction of key muscle development factors, such as MyoD and …

Qualitative Evaluation Of The Project P.A.T.H.S.: An Integration Of Findings Based On Program Implementers, Daniel T. L. Shek

Pediatrics Faculty Publications

An integration of the qualitative evaluation findings collected from program implementers conducting the Project P.A.T.H.S. (Positive Adolescent Training through Holistic Social Programmes) in different years (n = 177 participants in 36 focus groups) was carried out. General qualitative data analyses utilizing intra and interrater reliability techniques were performed. Results showed that the descriptors used to describe the program and the metaphors named by the informants that could stand for the program were generally positive in nature. Program participants also perceived the program to be beneficial to the development of the students in different psychosocial domains. The present study further supports …

Positive Youth Development Constructs: Conceptual Review And Application, Daniel T. L. Shek, Rachel C. F. Sun, Joav Merrick

Pediatrics Faculty Publications

No abstract provided.

Self-Harm And Suicidal Behaviors In Hong Kong Adolescents: Prevalence And Psychosocial Correlates, Daniel T. L. Shek, Lu Yu

Pediatrics Faculty Publications

The present paper examined the prevalence and psychosocial correlates of adolescent deliberate self-harm (DSH) and suicidal behavior in a representative sample of 3,328 secondary school students in Hong Kong. With reference to the previous year, 32.7% of the students reported at least one form of DSH, 13.7% of the respondents had suicide thoughts, 4.9% devised specific suicidal plans, and 4.7% had actually attempted suicide. Adolescent girls had significantly higher rates of DSH and suicidal behavior than did adolescent boys. Having remarried parents was related to an increased likelihood of DSH and suicide. While high levels of family functioning, overall positive …

Malnutrition In Young Pakistani Children, Shela Akbar Ali Hirani

School of Nursing & Midwifery

Background: Pakistan is a developing country with the second highest infant and child mortality rate in South Asia. In this region, malnutrition underlies much of the high infant and under five child morbidity and mortality rates. Although struggle to tackle the issue of malnutrition among young Pakistani children has been going on since many decades, till yet a realistic solution for this growing problem has not been found.

Objective: This paper aims at reviewing literature to analyse the biological, maternal, socio-cultural, environmental, and politico-economical determinants of malnutrition among young children in Pakistan so that need based interventions can …

Biotinidase Deficiency In Pakistani Children; What Needs To Be Known And Done, Bushra Afroze, Mohammad Wasay

Department of Medicine

No abstract provided.

Evaluation Of The Training Program Of The Project P.A.T.H.S.: Findings Based On The Perspective Of The Participants From Different Cohorts, Daniel T. L. Shek, Yammy L. Y. Chak

Pediatrics Faculty Publications

Subjective outcome evaluation findings based on the perspective of the participants participating in a 3-day training program of the Project P.A.T.H.S. are reported in this paper. The findings were based on the data collected from the training workshops conducted between 2005 and 2009 (N = 4.167). Results showed that the respondents had good and positive perceptions of the training program and found it very valuable, particularly with respect to training instructors and familiarization with the project. Besides, the training participants were able to acquire attitude, knowledge and skills that are conducive to the successful implementation of the program. Based on …

Subjective Outcome Evaluation And Factors Related To Perceived Effectiveness Of The Project P.A.T.H.S. In Hong Kong, Daniel T. L. Shek, Lu Yu, Vicky Y. T. Ho

Pediatrics Faculty Publications

Based on a sample of 24,457 participated students, the present study investigated participants' subjective evaluation of the Tier 2 Program of the Project P.A.T.H.S. in the 2009/2010 academic year. Participants generally held positive views toward the Tier 2 Program and program instructor and perceived the program to be beneficial to their development. Programs involving adolescents alone were evaluated more positively than programs involving parents and/or teachers. Students' grade and program type did not show significant impact on participants' subjective evaluation of the project. Consistent with previous reports, perceived effectiveness of the program was significantly predicted by students' perceptions about the …

The Project P.A.T.H.S. In Hong Kong--Lessons Learned And Implications For Positive Youth Development Programs, Daniel T. L. Shek, Rachel C. F. Sun

Pediatrics Faculty Publications

The Project P.A.T.H.S. is a positive youth development program which attempts to promote holistic development of junior secondary schools in Hong Kong. It is ground breaking in terms of the number of schools participating in the project and financial resources injected into the project. Based on the experiences gained from the implementation of the project and evaluation data collected from 2005 to 2011, several issues pertinent to the development of positive youth development programs in the Chinese culture are discussed. These issues include complexity of program development, importance of training, identification of factors governing program implementation, need for evaluation, and …

Helping Adolescents With Greater Psychosocial Needs: Subjective Outcome Evaluation Based On Different Cohorts, Daniel T. L. Shek, Tak Yan Lee

Pediatrics Faculty Publications

The Tier 2 Program of the Project P.A.T.H.S. (Positive Adolescent Training through Holistic Social Programmes) is designed to help students with greater psychosocial needs. This paper examines nine sets of subjective outcome evaluation data collected from 2005 to 2009 (n = 60, 241 participants). Based on the consolidated data with schools as units, results showed that participants generally had positive perceptions of the program, implementers, and benefits of the program. The subjective outcome evaluation instrument was found to be internally consistent. Multiple regression analysis revealed that perceived qualities of the program and the program implementers predicted perceived effectiveness of the …

Patient Safety In Developing Countries: Retrospective Estimation Of Scale And Nature Of Harm To Patients In Hospital, R. M. Wilson, P. Michel, S. Olsen, R. W. Gibberd, C. Vincent, R. El-Assady, O. Rasslan, S. Qsous, William Macharia, A. Sahel, S. Whittaker, M. Abdo-Ali, M. Letaief, N. A. Ahmed, A. Abdellatif, I. Larizgoitia

Paediatrics and Child Health, East Africa

Objective: To assess the frequency and nature of adverse events to patients in selected hospitals in developing or transitional economies.

Design: Retrospective medical record review of hospital admissions during 2005 in eight countries.

Setting: Ministries of Health of Egypt, Jordan, Kenya, Morocco, Tunisia, Sudan, South Africa and Yemen; the World Health Organisation (WHO) Eastern Mediterranean and African Regions (EMRO and AFRO), and WHO Patient Safety.

Participants: Convenience sample of 26 hospitals from which 15 548 patient records were randomly sampled.

Main outcome measures: Two stage screening. Initial screening based on 18 explicit criteria. Records that screened positive were then reviewed …

Overlap Subtype Of Chronic Graft-Versus-Host Disease Is Associated With An Adverse Prognosis, Functional Impairment, And Inferior Patient-Reported Outcomes: A Chronic Graft-Versus-Host Disease Consortium Study, Joseph Pidala, Georgia Vogelsang, Paul Martin, Xiaoyu Chai, Barry Storer, Steven Pavletic, Daniel Weisdorf, Madan Jagasia, Corey Cutler, Jeanne Palmer, David Jacobsohn, Sally Arai, Stephanie J. Lee

Pediatrics Faculty Publications

Background

The National Institutes of Health Consensus Conference proposed the term “overlap” graft-versus-host disease to describe the situation when both acute and chronic graft-versus-host disease are present.

Design and Methods

We examined whether the overlap subtype of graft-versus-host disease was associated with a different prognosis, functional limitations, or patient-reported outcomes compared to “classic” chronic graft-versus-host disease without any acute features.

Results

Prospective data were collected from 427 patients from nine centers. Patients were classified as having overlap (n=352) or classic chronic (n=75) graft-versus-host disease based on reported organ involvement. Overlap …

Mitogen-Activated Protein Kinase Phosphatase-1 Inhibits Myocardial Tnf-Α Expression And Improves Cardiac Function During Endotoxemia, Ting Zhang, Xiangru Lu, Paul Arnold, Yin Liu, Reshma Baliga, Hong Huang, John Anthony Bauer, Yusen Liu, Qingping Feng

Paediatrics Publications

Aims: Myocardial tumour necrosis factor-α (TNF-α) expression induces cardiac dysfunction in endotoxemia. The aim of this study was to investigate the role of mitogen-activated protein kinase phosphatase-1 (MKP1) pathway in myocardial TNF-α expression and cardiac function during endotoxemia. Methods and results: Lipopolysaccharide (LPS) increased MKP1 expression in the myocardium in vivo and in cultured neonatal cardiomyocytes in vitro. LPS-induced extracellular signal-regulated kinase (ERK) 1/2 and p38 phosphorylation in the myocardium was prolonged in MKP1 -/- mice. Myocardial TNF-α mRNA and protein levels were enhanced in MKP1 -/- compared with wild-type (WT) mice in endotoxemia, leading to a further decrease in …

Advocate For Immunizations., Angela L. Myers

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Transcriptional Regulation Of N-Acetylglutamate Synthase, Sandra Kirsch Heibel, Giselle Yvette Lopez, Maria Panglao, Sonal Sodha, Leonardo Marino-Ramirez, Mendel Tuchman, Ljubica Caldovic

Pediatrics Faculty Publications

The urea cycle converts toxic ammonia to urea within the liver of mammals. At least 6 enzymes are required for ureagenesis, which correlates with dietary protein intake. The transcription of urea cycle genes is, at least in part, regulated by glucocorticoid and glucagon hormone signaling pathways. N-acetylglutamate synthase (NAGS) produces a unique cofactor, N-acetylglutamate (NAG), that is essential for the catalytic function of the first and rate-limiting enzyme of ureagenesis, carbamyl phosphate synthetase 1 (CPS1). However, despite the important role of NAGS in ammonia removal, little is known about the mechanisms of its regulation. We identified two regions of high …

Cd11d Antibody Treatment Improves Recovery In Spinal Cord-Injured Mice, Nicole M. Geremia, Feng Bao, Trina E. Rosenzweig, Todd Hryciw, Lynne Weaver, Gregory A. Dekaban, Arthur Brown

Paediatrics Publications

Acute administration of a monoclonal antibody (mAb) raised against the CD11d subunit of the leukocyte CD11d/CD18 integrin after spinal cord injury (SCI) in the rat greatly improves neurological outcomes. This has been chiefly attributed to the reduced infiltration of neutrophils into the injured spinal cord in treated rats. More recently, treating spinal cord-injured mice with a Ly-6G neutrophil-depleting antibody was demonstrated to impair neurological recovery. These disparate results could be due to different mechanisms of action utilized by the two antibodies, or due to differences in the inflammatory responses between mouse and rat that are triggered by SCI. To address …

A Method To Determine Regional Mechanical Left Ventricular Dyssynchrony Based On High Temporal Resolution Short Axis Ssfp Cine Images, Jonathan Suever, Brandon K. Fornwalt, Michael Lloyd, John N. Oshinski

Pediatrics Presentations

Left ventricular (LV) mechanical dyssynchrony has been proposed as a parameter to select patients for cardiac resynchronization therapy (CRT) [Bax et al JACC 2005].Several recent studies have shown that placing the LV pacing lead in the most delayed regions yields a better response to CRT [Ansalone et al JACC 2002]. However, most imaging-based methods assess global LV dyssynchrony providing a single value for the entire LV. Regional maps of LV dyssynchrony are required for planning LV lead placement.

The objective of this study was to develop a method to create a map of regional left ventricular mechanical dyssynchrony based on …

Symptomatic Hypocalcemia During Urinary Alkalinization For Acute Aspirin Toxicity, Jennifer Flint, Bruce Banwart

Posters

A16y/o ingested 165 tablets of 325 mg ASA(1000 mg/kg). He presented to an outside facility 8 hours after ingestion alert with stable vital signs and ASA level 78 mg/dL. Initial lab values showed an arterial blood gas (ABG) pH 7.46, pCO2 26 mmHg, HCO3 18 mmol/L, ionized calcium 1.09 mmol/L, serum calcium 10 mg/dL, albumin 4.5 gm/dL, magnesium 2.1 mg/dL. Urinary alkalinization was initiated with a sodium bicarbonate infusion (NaHCO3) and he arrived to our facility 12 hours after ingestion with Kussmaul respirations and altered mentation. Repeat ASA 116mg/dL, ionized calcium 0.88 mmol/L and serum calcium 8.8 mg/dL. He became …

Characterization Of The Size And Location Of Dyssynchronous Regions In Patients Undergoing Crt, Jonathan Suever, Brandon K. Fornwalt, Michael Lloyd, John N. Oshinski

Pediatrics Presentations

The amount and location of left ventricular (LV) mechanical dyssynchrony affects an individual’s ability to respond positively to cardiac resynchronization therapy (CRT) [Bax et al JACC 2005]. By using high temporal resolution short-axis cines, it is possible to derive radial motion curves throughout the LV. These radial motion curves can be used to create maps showing dyssynchronous regions in patients enrolled for CRT.

The objective of this study was to characterize the size and location of areas of mechanical dyssynchrony in patients scheduled for CRT by comparing their radial wall motion curves to radial motion curves from normal subjects.

Longitudinal Impact Of The Project Paths On Adolescent Risk Behavior: What Happened After Five Years?, Daniel T. L. Shek, Lu Yu

Pediatrics Faculty Publications

The present study investigated the longitudinal impact of the Project PATHS, a large-scale curriculum-based positive youth development program in Hong Kong, on the development of adolescents' risk behavior over a period of five years. Using a longitudinal randomized controlled design, eight waves of data were collected from 19 experimental schools in which students participated in the Project PATHS (N = 2,850 at Wave 8) and 24 control schools without joining the Project PATHS (N = 3,640 at Wave 8). At each wave, students responded to measures assessing their current risk behaviors, including delinquency, use of different types of drug, and …

Mechanisms Of Fetal Programming In Hypertension., John E. Jones, Julie A. Jurgens, Sarah A. Evans, Riley C. Ennis, Van Anthony Villar, Pedro A. Jose

Pediatrics Faculty Publications

Events that occur in the early fetal environment have been linked to long-term health and lifespan consequences in the adult. Intrauterine growth restriction (IUGR), which may occur as a result of nutrient insufficiency, exposure to hormones, or disruptions in placental structure or function, may induce the fetus to alter its developmental program in order to adapt to the new conditions. IUGR may result in a decrease in the expression of genes that are responsible for nephrogenesis as nutrients are rerouted to the development of more essential organs. Fetal survival under these conditions often results in low birth weight and a …

Schools As Potential Vaccination Venue For Vaccines Outside Regular Epi Schedule: Results From A School Census In Pakistan., Sajid Bashir Soofi, Inam-Ul Haq, M. Imran Khan, Muhammad Bilal Siddiqui, Mushtaq Mirani, Rehman Tahir, Imtiaz Hussain, Mahesh K Puri, Zamir Hussain Suhag, Asif R Khowaja, Abdul Razzaq Lasi, John D Clemens, Michael Favorov, R. Leon Ochiai, Zulfiqar Ahmed Bhutta

Department of Paediatrics and Child Health

Background: Vaccines are the most effective public health intervention. Expanded Program on Immunization (EPI) provides routine vaccination in developing countries. However, vaccines that cannot be given in EPI schedule such as typhoid fever vaccine need alternative venues. In areas where school enrolment is high, schools provide a cost effective opportunity for vaccination. Prior to start of a school-based typhoid vaccination program, interviews were conducted with staff of educational institutions in two townships of Karachi, Pakistan to collect baseline information about the school system and to plan a typhoid vaccination program. Data collection teams administered a structured questionnaire to all schools …

2012 Cancer Care Annual Report, Children's Mercy Hospital

Cancer Center Annual Reports

Annual report of cancer care and oncology services at the Children's Mercy Hospital, Kansas City MO, a pediatric medical center.

Physiologically Based Pharmacokinetic Modeling In Pediatric Drug Development: A Clinician's Request For A More Integrated Approach., Karel M. Allegaert, Anne Smits, John N. Van Den Anker

Pediatrics Faculty Publications

No abstract provided.

Hypertension-Related Gene Polymorphisms Of G-Protein-Coupled Receptor Kinase 4 Are Associated With Nt-Probnp Concentration In Normotensive Healthy Adults, Junichi Yatabe, Midori S. Yatabe, Minoru Yoneda, Robin A. Felder, Pedro A. Jose, Hironobu Sanada

Pediatrics Faculty Publications

G protein-coupled receptor kinase 4 (GRK4) with activating polymorphisms desensitize the natriuric renal tubular D1 dopamine receptor, and these GRK4 polymorphisms are strongly associated with salt sensitivity and hypertension. Meanwhile, N-terminal pro-B-type natriuretic peptide (NT-proBNP) may be useful in detecting slight volume expansion. However, relations between hypertension-related gene polymorphisms including GRK4 and cardiovascular indices such as NT-proBNP are not clear, especially in healthy subjects. Therefore, various hypertension-related polymorphisms and cardiovascular indices were analyzed in 97 normotensive, healthy Japanese adults. NT-proBNP levels were significantly higher in subjects with two or more GRK4 polymorphic alleles. Other hypertension-related gene polymorphisms, such as those …

Hiv-1 Resistant Cdk2-Knockdown Macrophage-Like Cells Generated From 293t Cell-Derived Human Induced Pluripotent Stem Cells, Marina Jerebtsova, Namita Kumari, Min Xu, Gustavo Brito Alvim De Melo, Xiaomei Niu, Kuan-Teh Jeang, Sergei Nekhai

Pediatrics Faculty Publications

A major challenge in studies of human diseases involving macrophages is low yield and heterogeneity of the primary cells and limited ability of these cells for transfections and genetic manipulations. To address this issue, we developed a simple and efficient three steps method for somatic 293T cells reprogramming into monocytes and macrophage-like cells. First, 293T cells were reprogrammed into induced pluripotent stem cells (iPSCs) through a transfection-mediated expression of two factors, Oct-4 and Sox2, resulting in a high yield of iPSC. Second, the obtained iPSC were differentiated into monocytes using IL-3 and M-CSF treatment. And third, monocytes were differentiated into …

Evaluation Of Sedimentation Rate Methodology Reveals An Unusual Pediatric Subpopulation With Lupus Or Lupus-Like Syndrome And Hemolytic Anemia, Edward C. Wong, Deborah Yuan, Claas Hinze, Lawrence Jung

Pediatrics Faculty Publications

Purpose

Sedimentaion rate is often used to manage pediatric patients with rheumatologic disease. Most management decisions are dependent on studies which have used Wintrobe or Westergren sedimentation rate methodologies. However, these methods suffer from the need for relatively large amounts of blood and long turn-around times. Determination of sedimentation rate using laser kinetic rate determination has allowed calibration to Westergren methods, low volume of blood needed for testing and very rapid results. We sought to compare the Wintrobe method to the ESR Stat method (kinetic method; HemaTechnologies, Lebanon NJ) to determine suitability of the ESR Stat method for patient testing. …

The Test Characteristics Of Head Circumference Measurements For Pathology Associated With Head Enlargement: A Retrospective Cohort Study, Carrie Daymont, Moira Zabel, Chris Feudtner, David M. Rubin

Pediatrics Faculty Publications

Background

The test characteristics of head circumference (HC) measurement percentile criteria for the identification of previously undetected pathology associated with head enlargement in primary care are unknown.

Methods

Electronic patient records were reviewed to identify children age 3 days to 3 years with new diagnoses of intracranial expansive conditions (IEC) and metabolic and genetic conditions associated with macrocephaly (MGCM). We tested the following HC percentile threshold criteria: ever above the 95th, 97th, or 99.6thpercentile and ever crossing 2, 4, or 6 increasing major percentile lines. The Centers for Disease Control and World Health Organization growth curves were used, as well …

Reasons For Low Pandemic H1n1 2009 Vaccine Acceptance Within A College Sample, Russell D. Ravert, Linda Y. Fu

Pediatrics Faculty Publications

This study examined health beliefs associated with novel influenza A (H1N1) immunization among US college undergraduates during the 2009-2010 pandemic. Undergraduates (ages 18–24 years) from a large Midwestern University were invited to complete an online survey during March, 2010, five months after H1N1 vaccines became available. Survey items measured H1N1 vaccine history and H1N1-related attitudes based on the health belief literature. Logistic regression was used to identify attitudes associated with having received an H1N1 vaccine, and thematic analysis of student comments was conducted to further understand influences on vaccine decisions. Among the 296 students who participated in the survey, 15.2% …

Nonsteroidal Anti-Inflammatory Drugs Are An Important Cause Of Acute Kidney Injury In Children, Jason M. Misurac, Chad A. Knoderer, Jeffrey D. Leiser, Corina Nilescu, Amy C. Wilson, Sharon P. Andreoli

Scholarship and Professional Work – COPHS

Objective

To characterize nonsteroidal anti-inflammatory drug (NSAID)-associated acute kidney injury (AKI) in children.

Study design

We conducted a retrospective chart review of children diagnosed with AKI through the use of International Classification of Diseases, Ninth Revision diagnosis code 584.5 or 584.9 from January 1999 to June 2010. Medical records were reviewed to confirm the diagnosis of AKI and to quantify NSAID administration. Pediatric RIFLE criteria were used to codify AKI. Patients were not classified as having NSAID-associated AKI if they had a diagnosis explaining AKI or comorbid clinical conditions predisposing to AKI development.

Results

Patients (N = 1015) were identified …