Pediatrics Commons^™

Integrin-Linked Kinase: A Scaffold Protein Unique Among Its Ilk, Lina Dagnino

Paediatrics Publications

Integrin-linked kinase (ILK) is a scaffolding protein with central roles in tissue development and homeostasis. Much debate has focused on whether ILK is a bona fide or a pseudo- kinase. This aspect of ILK function has been complicated by the large volumes of conflicting observations obtained from a wide variety of experimental approaches, from in vitro models, to analyses in invertebrates and in mammals. Key findings in support or against the notion that ILK is catalytically active are summarized. The importance of ILK as an adaptor protein is well established, and defining its role as a signaling hub will be …

The Prevalence And Factors Associated With Nonadherence With Arv Treatment And Cotrimoxazole Preventive Therapy Among Hiv Infected Adolescents Attending Out-Patient Hiv Clinics In Kenya, Reson Marima

Master of Medicine in Paediatrics and Child Health

Background: There is an increase in the burden of HIV infected adolescents, both those perinatally infected as well as those acquiring HIV during adolescence. Female adolescents continue to be at the highest risk for acquiring HIV; in many Southern African countries, they have a three fold risk of acquiring HIV compared to their male counterparts. Adherence to treatment among adolescents has been shown to range between 30-70%, which is unacceptably low for antiretroviral therapy (ART). Psychosocial wellbeing and social support have been shown to be possible contributing factors to adherence to ART. This relationship has not been previously evaluated among …

Effects Of Hypo And Iso - Tonic Intravenous Maintenance Fluids On Plasma Sodium Level In Hospitalized Children. A Double Blind Randomized Clinical Trial, Ismail Ahmed

Master of Medicine in Paediatrics and Child Health

Background: Hypotonic fluids are widely used in pediatrics and are the standard of care when giving maintenance fluids. However, there are several reports of risk of iatrogenic hyponatremia attributed to this practice in the literature. There is therefore uncertainty as to whether isotonic fluids would be the more appropriate fluid. Objectives: The primary objective was to compare effects of hypotonic and isotonic maintenance fluids on plasma sodium levels in children between the ages of 2 months and 15 years admitted at Aga Khan University Hospital (AKUH). The secondary objective was to compare effects of hypotonic and isotonic maintenance fluids on …

System-Wide Deployment Of Color Coded Pediatric Resuscitation Carts Supported By In Situ Simulation, Deborah Arnold Msn, Rn, Cmsrn, Jayne Febbraro Msn Rn Crnp, William Bond Md, Ms, Michele Dunstan, Margaret J. Chorazak, Melissa B. Walsh Bs, K Walker, T Dietz

Department of Education

No abstract provided.

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

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 …

Baseline Data For Maine Pediatric And Family Practices From Arra-Funded Hit Ambulatory Practice Survey Data, Kimberley S. Fox Mpa

Population Health & Health Policy

No abstract provided.

Rac1 Activation Induces Tumour Necrosis Factor-Α Expression And Cardiac Dysfunction In Endotoxemia, Ting Zhang, Xiangru Lu, Frank Beier, Qingping Feng

Paediatrics Publications

Induction of tumour necrosis factor-α (TNF-α) expression leads to myocardial depression during sepsis. However, the underlying molecular mechanisms are not fully understood. The aim of this study was to investigate the role of Rac1 in TNF-α expression and cardiac dysfunction during endotoxemia and to determine the involvement of phosphoinositide-3 kinase (PI3K) in lipopolysaccharide (LPS)-induced Rac1 activation. Our results showed that LPS-induced Rac1 activation and TNF-α expression in cultured neonatal mouse cardiomyocytes. The response was inhibited in Rac1 deficient cardiomyocytes or by a dominant-negative Rac1 (Rac1N17). To determine whether PI3K regulates Rac1 activation, cardiomyocytes were treated with LY294002, a PI3K selective …

Deletion Of Glycogen Synthase Kinase-3Β In Cartilage Results In Up-Regulation Of Glycogen Synthase Kinase-3Α Protein Expression, J. R. Gillespie, V. Ulici, H. Dupuis, A. Higgs, A. Dimattia, S. Patel, J. R. Woodgett, Frank Beier

Paediatrics Publications

The rate of endochondral bone growth determines final height in humans and is tightly controlled. Glycogen synthase kinase-3 (GSK-3) is a negative regulator of several signaling pathways that govern bone growth, such as insulin/IGF and Wnt/β-catenin. The two GSK-3 proteins, GSK-3 and GSK-3β, display both overlapping and distinct roles in different tissues. Here we show that pharmacological inhibition of GSK-3 signaling in a mouse tibia organ culture system results in enhanced bone growth, accompanied by increased proliferation of growth plate chondrocytes and faster turnover of hypertrophic cartilage to bone. GSK-3 inhibition rescues some, but not all, effects of phosphatidylinositide 3-kinase …

Maternal Protein Restriction Elevates Cholesterol In Adult Rat Offspring Due To Repressive Changes In Histone Modifications At The Cholesterol 7Α-Hydroxylase Promoter, Gurjeev Sohi, Kelly Marchand, Andrew Revesz, Edith Arany, Daniel B. Hardy

Paediatrics Publications

Adverse events in utero, such as intrauterine growth restriction (IUGR), can permanently alter epigenetic mechanisms leading to the metabolic syndrome, which encompasses a variety of symptoms including augmented cholesterol. The major site for cholesterol homeostasis occurs via the actions of hepatic cholesterol 7α-hydroxylase (Cyp7a1), which catabolizes cholesterol to bile acids. To determine whether posttranslational histone modifications influence the long-term expression of Cyp7a1 in IUGR, we used a protein restriction model in rats. This diet during pregnancy and lactation led to IUGR offspring with decreased liver to body weight ratios, followed by increased circulating and hepatic cholesterol levels in both sexes …

Effects Of Environment On Children's Motor Scores, Eligibility Status, And Administration Times, Derrick Mittelstadt, Abigail Parker, Kirsten Pickett, Heather Temkin

UNLV Theses, Dissertations, Professional Papers, and Capstones

Best practices for assessing developmental skills in young children focus on naturalistic observation in everyday settings, but the effects of environment on test scores, eligibility status and administration time have not been explored. The Peabody Developmental Motor Scales-Second Edition (PDMS-2) was administered to 34 children aged 18 to 59 months in natural and pull-out settings. PDMS-2 total, gross, and fine motor quotient (TMQ, GMQ, and FMQ) scores were significantly lower in the natural environment (p’s≤.014). Based on our results, more children would qualify for services when tested in natural environments using TMQ and GMQ scores. It also took significantly longer …

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

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 …

Adolescent Medicine, Carol L. Watwood

DLPS Faculty Publications

No abstract provided.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, And Adenitis (Pfapa) Is A Disorder Of Innate Immunity And Th1 Activation Responsive To Il-1 Blockade., Silvia Stojanov, Sivia Lapidus, Puja Chitkara, Henry Feder, Juan C Salazar, Thomas A Fleisher, Margaret R Brown, Kathryn M Edwards, Michael M Ward, Robert A Colbert, Hong-Wei Sun, Geryl M Wood, Beverly K Barham, Anne Jones, Ivona Aksentijevich, Raphaela Goldbach-Mansky, Balu Athreya, Karyl S Barron, Daniel L Kastner

Department of Pediatrics Faculty Papers

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever disease in children. However, the pathogenesis is unknown. Using a systems biology approach we analyzed blood samples from PFAPA patients whose genetic testing excluded hereditary periodic fevers (HPFs), and from healthy children and pediatric HPF patients. Gene expression profiling could clearly distinguish PFAPA flares from asymptomatic intervals, HPF flares, and healthy controls. During PFAPA attacks, complement (C1QB, C2, SERPING1), IL-1-related (IL-1B, IL-1RN, CASP1, IL18RAP), and IFN-induced (AIM2, IP-10/CXCL10) genes were significantly overexpressed, but T cell-associated transcripts (CD3, CD8B) were down-regulated. On the …

Does Improving Maternal Knowledge Of Vaccines Impact Infant Immunization Rates? A Community-Based Randomized-Controlled Trial In Karachi, Pakistan, Aatekah Owais, Beenish Hanif, Amna R. Siddiqui, Ajmal Agha, Anita K. M. Zaidi

Department of Paediatrics and Child Health

Background: In Pakistan, only 59-73% of children 12-23 months of age are fully immunized. This randomized, controlled trial was conducted to assess the impact of a low-literacy immunization promotion educational intervention for mothers living in low-income communities of Karachi on infant immunization completion rates. Methods: Three hundred and sixty-six mother-infant pairs, with infants aged Results: Baseline characteristics among the two groups were similar. At 4 month assessment, among 179 mother-infant pairs in the intervention group, 129 (72.1%) had received all 3 doses of DPT/Hepatitis B vaccine, whereas in the control group 92/178 (51.7%) had received all 3 doses. Multivariable analysis …

Chelation Therapy As A Treatment For Autism, Cassandra Baty, Rachel Terharr

Festival of Communities: UG Symposium (Posters)

As medical advances continue to be made, there has yet to be an effectively consistent cure to the commonly known condition, Autism. Some researchers and physicians state that by using chelation therapy, it could help cure mercury poisoning in autistic individuals who were affected by vaccines or other sources. Because of the lack of research and knowledge of chelation therapy, there has been some controversy as to the ethics of providing chelation therapy to autistic children. The families that are put through these trials are faced with high costs, and no guarantee that their child could be cured. But to …

Effect Of Preventive Zinc Supplementation On Linear Growth In Children Under 5 Years Of Age In Developing Countries: A Meta-Analysis Of Studies For Input To The Lives Saved Tool, Aamer Imdad, Zulfiqar Ahmed Bhutta

Woman and Child Health

Introduction:Zinc plays an important role in cellular growth, cellular differentiation and metabolism. The results of previous meta-analyses evaluating effect of zinc supplementation on linear growth are inconsistent. We have updated and evaluated the available evidence according to Grading of Recommendations, Assessment, Development and Evaluation (GRADE) criteria and tried to explain the difference in results of the previous reviews.

Methods:

A literature search was done on PubMed, Cochrane Library, IZiNCG database and WHO regional data bases using different terms for zinc and linear growth (height). Data were abstracted in a standardized form. Data were analyzed in two ways i.e. weighted …

Impact Of Maternal Education About Complementary Feeding And Provision Of Complementary Foods On Child Growth In Developing Countries, Aamer Imdad, Mohammad Yawar Yakoob, Zulfiqar Ahmed Bhutta

Woman and Child Health

Background:Childhood undernutrition is prevalent in low and middle income countries. It is an important indirect cause of child mortality in these countries. According to an estimate, stunting (height for age Z score < -2) and wasting (weight for height Z score < -2) along with intrauterine growth restriction are responsible for about 2.1 million deaths worldwide in children < 5 years of age. This comprises 21 % of all deaths in this age group worldwide. The incidence of stunting is the highest in the first two years of life especially after six months of life when exclusive breastfeeding alone cannot fulfill the energy needs of a rapidly growing child. Complementary feeding for an infant refers to timely introduction of safe and nutritional foods in addition to breast-feeding (BF) i.e. clean and nutritionally rich additional foods introduced at about six months of infant age. Complementary feeding strategies encompass a wide variety of interventions designed to improve not only the quality and quantity of these foods but also improve the feeding behaviors. In this review, we evaluated the effectiveness of two most commonly applied strategies of complementary feeding i.e. timely provision of appropriate complementary foods (+/- nutritional counseling) and education to mothers about practices of complementary feeding on growth. Recommendations have been made for input to the Lives Saved Tool (LiST) model by following standardized guidelines developed by Child Health Epidemiology Reference Group (CHERG).

Methods:

We conducted a systematic review of published randomized and quasi-randomized trials on PubMed, Cochrane Library and WHO regional databases. The included studies were abstracted and graded according to Study Design, limitations, intervention details and outcome effects. The primary outcomes were change in weight and height during the study period among children 6-24 months of age. We hypothesized that provision of complementary food and education of mother …

Impact Of Vitamin A Supplementation On Infant And Childhood Mortality, Aamer Imdad, Mohammad Yawar Yakoob, Christopher Sudfeld, Batool A. Haider, Robert E. Black, Zulfiqar Ahmed Bhutta

Woman and Child Health

Introduction:Vitamin A is important for the integrity and regeneration of respiratory and gastrointestinal epithelia and is involved in regulating human immune function. It has been shown previously that vitamin A has a preventive effect on all-cause and disease specific mortality in children under five. The Purpose of this paper was to get a point estimate of efficacy of vitamin A supplementation in reducing cause specific mortality by using Child Health Epidemiology Reference Group (CHERG) guidelines.

Methods:

A literature search was done on PubMed, Cochrane Library and WHO regional data bases using various free and Mesh terms for vitamin A …