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Aberrant Brain Functional Connectivity In Newborns With Congenital Heart Disease Before Cardiac Surgery, Josepheen De Asis-Cruz, Mary T. Donofrio, Gilbert Vezina, Catherine Limperopoulos 2018 George Washington University

Aberrant Brain Functional Connectivity In Newborns With Congenital Heart Disease Before Cardiac Surgery, Josepheen De Asis-Cruz, Mary T. Donofrio, Gilbert Vezina, Catherine Limperopoulos

Pediatrics Faculty Publications

Newborns with congenital heart disease (CHD) requiring open heart surgery are at increased risk for neurodevelopmental disabilities. Recent quantitative MRI studies have reported disrupted growth, microstructure, and metabolism in fetuses and newborns with complex CHD. To date, no study has examined whether functional brain connectivity is altered in this high-risk population after birth, before surgery. Our objective was to compare whole-brain functional connectivity of resting state networks in healthy, term newborns (n = 82) and in term neonates with CHD before surgery (n = 30) using graph theory and network-based statistics. We report for the first time intact global network topology – efficient ...


Plasma Fibroblast Growth Factor-21 Levels In Patients With Inborn Errors Of Metabolism, Brian Kirmse, Juan Cabrera-Luque, Omar Ayyub, Kristina Cusmano-Ozog, Kimberly A. Chapman, Marshall L. Summar 2017 George Washington University

Plasma Fibroblast Growth Factor-21 Levels In Patients With Inborn Errors Of Metabolism, Brian Kirmse, Juan Cabrera-Luque, Omar Ayyub, Kristina Cusmano-Ozog, Kimberly A. Chapman, Marshall L. Summar

Pediatrics Faculty Publications

Fibroblast growth factor-21 (FGF21) levels are elevated in patients with primary mitochondrial disorders but have not been studied in patients with inborn errors of metabolism (IEM) known to have secondary mitochondrial dysfunction. We measured plasma FGF21 by ELISA in patients with and without IEM. FGF21 levels were higher in patients with IEM compared to without IEM (370 pg/dL vs. 0–65 pg/dL). Further study of FGF21 as a biomarker in IEM is warranted.


Confirmation That Mat1a P.Ala259val Mutation Causes Autosomal Dominant Hypermethioninemia, M J. Muriello, S Viall, T Bottiglieri, K Cusmano-Ozog, C R. Ferreira 2017 George Washington University

Confirmation That Mat1a P.Ala259val Mutation Causes Autosomal Dominant Hypermethioninemia, M J. Muriello, S Viall, T Bottiglieri, K Cusmano-Ozog, C R. Ferreira

Pediatrics Faculty Publications

Methionine adenosyltransferase (MAT) I/III deficiency is an inborn error of metabolism caused by mutations in MAT1A, encoding the catalytic subunit of MAT responsible for the synthesis of S-adenosylmethionine, and is characterized by persistent hypermethioninemia. While historically considered a recessive disorder, a milder autosomal dominant form of MAT I/III deficiency occurs, though only the most common mutation p.Arg264His has ample evidence to prove dominant inheritance. We report a case of hypermethioninemia caused by the p.Ala259Val substitution and provide evidence of autosomal dominant inheritance by showing both maternal inheritance of the mutation and concomitant hypermethioninemia. The p.Ala259Val ...


Impact Of Hpv Education On Knowledge, Perception, And Cultural Influence Among African American Parents, Christina Lattner, Christina Lattner 2017 DePaul University

Impact Of Hpv Education On Knowledge, Perception, And Cultural Influence Among African American Parents, Christina Lattner, Christina Lattner

Grace Peterson Nursing Research Colloquium

Abstract

Background: Infection with human papillomavirus (HPV), the sexually transmitted virus that causes cervical and penial cancer can be prevented. An effective vaccine has been approved for both boys and girls since 2006. Despite recommendation from the Center of Disease Control, vaccination initiation and completion rates among African American adolescents in the United States are lower than average in the nation.

Objectives: The purpose of this descriptive survey study was to identify African-American parents’ knowledge and perceptions of sexually transmitted HPV infection and the HPV vaccination. The secondary objective was to use a single-group pre-test post-test research design to examine ...


Why American Parents Circumcise Their Sons: An Integrative Review Of Literature, Lacy Lauber 2017 DePaul University

Why American Parents Circumcise Their Sons: An Integrative Review Of Literature, Lacy Lauber

Grace Peterson Nursing Research Colloquium

Background: Since medical evidence supports both pros and cons of routine neonatal male circumcision, expectant and new parents are left to make the decision to circumcise their sons on their own. There is limited research as to why American parents choose to circumcise their newborn sons.

Objectives: The purpose of this integrative literature review is to identify the reasons behind parental decision making in neonatal male circumcision in the United States. Understanding motivating factors in parental decision-making can highlight areas of prenatal education for expectant parents.

Method: An integrative literature review was conducted utilizing these online databases: PubMed, The Cumulative ...


Complementary Art Interventions For Children Coping With Cancer: An Integrative Literature Review, Brenda Pacheco 2017 DePaul University

Complementary Art Interventions For Children Coping With Cancer: An Integrative Literature Review, Brenda Pacheco

Grace Peterson Nursing Research Colloquium

Background: Cancer can be a physically and emotionally damaging disease. While there are various conventional pediatric cancer treatments, complementary alternative medicine (CAM), specifically art interventions, may be helpful in the care of these patients.

Objective: The aims of this integrative review were; (1) determine if art interventions impact coping with cancer in a pediatric population, (2) determine if the duration of the art intervention influences the impact on coping.

Methods: Articles were found through the following databases: Cumulative Index of Nursing and Allied Health (CINAHL), PubMed, PsycInfo, and ProQuest Nursing & Allied Health. Key search terms used included cancer, neoplasms, oncology, leukemia, child, pediatric, childhood, art, music, dance, art craft, therapies, medicine, interventions, treatments, and program. Studies were published in English and included three art interventions (arts and crafts, music, and dance).

Results: After a thorough review of the literature and removal of duplicates, 258 studies were found. Seven studies conducted in the U.S., Vietnam, and Australia were selected for inclusion. The following themes were found in art interventions: effective distractor, form of self-expression, and positive facial affects.

Conclusion: The findings suggest evidence of reduced anxiety, decreased distress, and improved self-esteem amongst children with cancer who received various art interventions, however, there is a lack of evidence on the duration of art interventions and their impact. Further systematic studies should be conducted to explore the duration of art interventions that yield the most therapeutic results in stress coping in pediatric oncology patients.

Keywords: cancer; child; pediatric; art; music; dance; therapies; medicine; interventions; treatments


Niemann-Pick Type C Disease, Sameh Morkous MD, FAAN 2017 Lehigh Valley Health Network

Niemann-Pick Type C Disease, Sameh Morkous Md, Faan

Department of Pediatrics

No abstract provided.


Association Of Teen Mothers' And Grandmothers' Parenting Capacities With Child Development: A Study Protocol., Damali Wilson, Deborah Gross, Stacy Hodgkinson, Kirby Deater-Deckard 2017 George Washington University

Association Of Teen Mothers' And Grandmothers' Parenting Capacities With Child Development: A Study Protocol., Damali Wilson, Deborah Gross, Stacy Hodgkinson, Kirby Deater-Deckard

Pediatrics Faculty Publications

Children born to teen mothers may experience less responsive and supportive parenting and are at heightened risk for a range of social, developmental, and health issues. There is literature to support the positive impact of grandmothers on teen parents and their children. However, what if the teen's mother is also limited in her parenting capacities? How do parenting capacities across these two generations of mothers affect the developing child? In this ongoing study we are examining two important aspects of parenting capacities, attachment quality and executive functioning, in teen mothers (TM) and their biological, co- residing mothers or grandmothers ...


Effect Of Intranasally Delivered Rh-Vegf165 On Angiogenesis Following Cerebral Hypoxia-Ischemia In The Cerebral Cortex Of Newborn Piglets., Amit Jain, Panagiotis Kratimenos, Ioannis Koutroulis, Amishi Jain, Amulya Buddhavarapu, Jahan Ara 2017 George Washington University

Effect Of Intranasally Delivered Rh-Vegf165 On Angiogenesis Following Cerebral Hypoxia-Ischemia In The Cerebral Cortex Of Newborn Piglets., Amit Jain, Panagiotis Kratimenos, Ioannis Koutroulis, Amishi Jain, Amulya Buddhavarapu, Jahan Ara

Pediatrics Faculty Publications

Background: Vascular endothelial growth factor (VEGF) stimulates vascular genesis and angiogenesis. Cerebral Hypoxia-Ischemia (HI) leads to the reduction of vasculature in the cerebral cortex of newborn piglets.

Objective: The present study tests the hypothesis that post-hypoxia intranasal administration of recombinant human VEGF165 (rh-VEGF165) for 3 days increases the vascular density in the cerebral cortex of newborn piglets without promoting neovascularization.

Design/Methods: Ventilated newborn piglets were divided into three groups (n = 5/group): normoxic (Nx), hypoxic-ischemic (HI), and HI treated with intranasal rh-VEGF165rh-VEGF165 (HI-VEGF). HI piglets were exposed to HI (0.05 FiO2) for 30 min. Recombinant h-VEGF165 (100 ...


I’M Going To Tell You A Little About Myself: Illness Centrality, Self-Image And Identity In Cystic Fibrosis, Susan Horky LCSW, Laura Sherman LICSW, Julie K. Polvinen BA, Medhavi Saxena MD, Michael Rich MD 2017 University of Florida

I’M Going To Tell You A Little About Myself: Illness Centrality, Self-Image And Identity In Cystic Fibrosis, Susan Horky Lcsw, Laura Sherman Licsw, Julie K. Polvinen Ba, Medhavi Saxena Md, Michael Rich Md

Patient Experience Journal

This study assessed the illness centrality of adolescents with CF and the specific ways that CF may affect adolescents’ identities, through the qualitative analysis of video narratives. Adolescents with CF were loaned video cameras and asked to “show us your life outside the hospital” and to “teach your healthcare team about your CF.” Four major themes were identified related to illness centrality: CF is Central, CF is Compartmentalized, CF is Integrated into Self Image, CF is Denied. Integration and compartmentalization often co-existed. Four themes emerged related to the role of CF in self-image and identity: (1) Valence (positive or negative ...


Clinical Risks And Healthcare Utilization Of Hematopoietic Cell Transplantation For Sickle Cell Disease In The Usa Using Merged Databases., Staci D Arnold, Ruta Brazauskas, Naya He, Yimei Li, Richard Aplenc, Zhezhen Jin, Allistair Abraham, +several additional authors 2017 George Washington University

Clinical Risks And Healthcare Utilization Of Hematopoietic Cell Transplantation For Sickle Cell Disease In The Usa Using Merged Databases., Staci D Arnold, Ruta Brazauskas, Naya He, Yimei Li, Richard Aplenc, Zhezhen Jin, Allistair Abraham, +Several Additional Authors

Pediatrics Faculty Publications

Advances in allogeneic hematopoietic cell transplantation for sickle cell disease have improved outcomes, but there is limited analysis of healthcare utilization in this setting. We hypothesized that, compared to late transplantation, early transplantation (at age <10 >years) improves outcomes and decreases healthcare utilization. We performed a retrospective study of children transplanted for sickle cell disease in the USA during 2000-2013 using two large databases. Univariate and Cox models were used to estimate associations of demographics, sickle cell disease severity, and transplant-related variables with mortality and chronic graft-versus-host disease, while Wilcoxon, Kruskal-Wallis, or linear trend tests were applied for the estimates of ...


Hyperoxia Causes Mir-34a-Mediated Injury Via Angiopoietin-1 In Neonatal Lungs., Mansoor Syed, Pragnya Das, Aishwarya Pawar, Zubair H. Aghai, Anu Kaskinen, Zhen W. Zhuang, Namasivayam Ambalavanan, Gloria Pryhuber, Sture Andersson, Vineet Bhandari 2017 Yale University School of Medicine; Drexel University College of Medicine

Hyperoxia Causes Mir-34a-Mediated Injury Via Angiopoietin-1 In Neonatal Lungs., Mansoor Syed, Pragnya Das, Aishwarya Pawar, Zubair H. Aghai, Anu Kaskinen, Zhen W. Zhuang, Namasivayam Ambalavanan, Gloria Pryhuber, Sture Andersson, Vineet Bhandari

Department of Pediatrics Faculty Papers

Hyperoxia-induced acute lung injury (HALI) is a key contributor to the pathogenesis of bronchopulmonary dysplasia (BPD) in neonates, for which no specific preventive or therapeutic agent is available. Here we show that lung micro-RNA (miR)-34a levels are significantly increased in lungs of neonatal mice exposed to hyperoxia. Deletion or inhibition of miR-34a improves the pulmonary phenotype and BPD-associated pulmonary arterial hypertension (PAH) in BPD mouse models, which, conversely, is worsened by miR-34a overexpression. Administration of angiopoietin-1, which is one of the downstream targets of miR34a, is able to ameliorate the BPD pulmonary and PAH phenotypes. Using three independent cohorts ...


Pediatric Residency Training And Behavioral Health: Models And Outcomes, Jeffrey Shahidullah PhD, Paul Kettlewell PhD, Mohammed Palejwala MA, Kathryn DeHart MD, Kris Rooney MD, Laura Diaz MD, Joanna Carlson MD 2017 Rutgers University - New Brunswick/Piscataway

Pediatric Residency Training And Behavioral Health: Models And Outcomes, Jeffrey Shahidullah Phd, Paul Kettlewell Phd, Mohammed Palejwala Ma, Kathryn Dehart Md, Kris Rooney Md, Laura Diaz Md, Joanna Carlson Md

Department of Pediatrics

No abstract provided.


Adversity And Academic Performance Among Adolescent Youth: A Community-Based Participatory Research Study, Sukhdip Purewal Boparai, Tiffani Marie, Eduardo Aguayo, Jordan Brooks, Estefany Juarez, Sheana Soriano, Alasia Waters, Jaquez Donaldson, Joseph Reagans, Gracee Anguiano, Allison Ipsen 2017 Center for Youth Wellness

Adversity And Academic Performance Among Adolescent Youth: A Community-Based Participatory Research Study, Sukhdip Purewal Boparai, Tiffani Marie, Eduardo Aguayo, Jordan Brooks, Estefany Juarez, Sheana Soriano, Alasia Waters, Jaquez Donaldson, Joseph Reagans, Gracee Anguiano, Allison Ipsen

Journal of Adolescent and Family Health

Researchers and youth stakeholders devised a survey on 27 adversities based on youth expertise, clinical practice, and adversity literature. The aim of the study was to understand the prevalence of individual and cumulative adversities, and association of adversities to age, gender, race/ethnicity and academic performance among a community sample of urban high school students. All participants experienced two or more adversities and experienced greater overall adversity than youth in population-based studies. Youth-proposed stressors were among the most prevalent, and females, older youth, and African American youth reported disproportionately greater number of adversities. Specific types of adversities were endorsed differentially ...


Evaluation Of Mothers And Infants With Presumed Exposure To Zika Virus: A Look Into The Lehigh Valley Experience, Tibisay Villalobos MD, Michelle Maron RN, Jeanette Taveras DO 2017 Lehigh Valley Health Network

Evaluation Of Mothers And Infants With Presumed Exposure To Zika Virus: A Look Into The Lehigh Valley Experience, Tibisay Villalobos Md, Michelle Maron Rn, Jeanette Taveras Do

Department of Pediatrics

No abstract provided.


Virus-Specific T Cells For The Immunocompromised Patient, A. Houghtelin, Catherine M. Bollard 2017 George Washington University

Virus-Specific T Cells For The Immunocompromised Patient, A. Houghtelin, Catherine M. Bollard

Pediatrics Faculty Publications

While progress has been made in the treatment of both hematologic cancers and solid tumors, chemorefractory or relapsed disease often portends a dismal prognosis, and salvage chemotherapy or radiation expose patients to intolerable toxicities and may not be effective. Hematopoietic stem cell transplant offers the promise of cure for many patients, and while mismatched, unrelated or haploidentical donors are increasingly available, the recipients are at higher risk of severe immunosuppression and immune dysregulation due to graft versus host disease. Viral infections remain a primary cause of severe morbidity and mortality in this patient population. Again, many therapeutic options for viral ...


Randomised Controlled Trial Of Lactobacillus Rhamnosus (Lgg) Versus Placebo In Children Presenting To The Emergency Department With Acute Gastroenteritis: The Pecarn Probiotic Study Protocol., David Schnadower, Phillip I Tarr, Casper T Charles, Marc H Gorelick, Michael J Dean, Karen J O'Connell, Prashant Mahajan, Thomas H Chun, Seema R Bhatt, Cindy G Roskind, Elizabeth C Powell, Alexander J Rogers, Cheryl Vance, Robert E Sapien, Feng Gao, Stephen B Freedman 2017 George Washington University

Randomised Controlled Trial Of Lactobacillus Rhamnosus (Lgg) Versus Placebo In Children Presenting To The Emergency Department With Acute Gastroenteritis: The Pecarn Probiotic Study Protocol., David Schnadower, Phillip I Tarr, Casper T Charles, Marc H Gorelick, Michael J Dean, Karen J O'Connell, Prashant Mahajan, Thomas H Chun, Seema R Bhatt, Cindy G Roskind, Elizabeth C Powell, Alexander J Rogers, Cheryl Vance, Robert E Sapien, Feng Gao, Stephen B Freedman

Pediatrics Faculty Publications

INTRODUCTION: Acute gastroenteritis (AGE) is a common and burdensome condition that affects millions of children worldwide each year. Currently available strategies are limited to symptomatic management, treatment and prevention of dehydration and infection control; no disease-modifying interventions exist. Probiotics, defined as live microorganisms beneficial to the host, have shown promise in improving AGE outcomes, but existing studies have sufficient limitations such that the use of probiotics cannot currently be recommended with confidence. Here we present the methods of a large, rigorous, randomised, double-blind placebo-controlled study to assess the effectiveness and side effect profile of Lactobacillus rhamnosus GG (LGG) (ATCC 53103 ...


Procalcitonin: The Pros And Cons In Pediatrics, Kris Rooney MD 2017 Lehigh Valley Health Network

Procalcitonin: The Pros And Cons In Pediatrics, Kris Rooney Md

Department of Pediatrics

No abstract provided.


Radiation-Free Cmr Diagnostic Heart Catheterization In Children., Kanishka Ratnayaka, Joshua P Kanter, Anthony Z Faranesh, Elena K Grant, Laura J Olivieri, Russell R Cross, Ileen F Cronin, Karin S Hamann, Adrienne E Campbell-Washburn, Kendall J O'Brien, Toby Rogers, Michael S Hansen, Robert J Lederman 2017 George Washington University

Radiation-Free Cmr Diagnostic Heart Catheterization In Children., Kanishka Ratnayaka, Joshua P Kanter, Anthony Z Faranesh, Elena K Grant, Laura J Olivieri, Russell R Cross, Ileen F Cronin, Karin S Hamann, Adrienne E Campbell-Washburn, Kendall J O'Brien, Toby Rogers, Michael S Hansen, Robert J Lederman

Pediatrics Faculty Publications

BACKGROUND: Children with heart disease may require repeated X-Ray cardiac catheterization procedures, are more radiosensitive, and more likely to survive to experience oncologic risks of medical radiation. Cardiovascular magnetic resonance (CMR) is radiation-free and offers information about structure, function, and perfusion but not hemodynamics. We intend to perform complete radiation-free diagnostic right heart catheterization entirely using CMR fluoroscopy guidance in an unselected cohort of pediatric patients; we report the feasibility and safety.

METHODS: We performed 50 CMR fluoroscopy guided comprehensive transfemoral right heart catheterizations in 39 pediatric (12.7 ± 4.7 years) subjects referred for clinically indicated cardiac catheterization. CMR ...


Hemimegalencephaly With Intractable Epilepsy: A Case Report, Prem Chand, Muhammad Faraz Raghib, Muhammad Sohail Salat 2017 Aga Khan University

Hemimegalencephaly With Intractable Epilepsy: A Case Report, Prem Chand, Muhammad Faraz Raghib, Muhammad Sohail Salat

Department of Paediatrics and Child Health

Isolated Hemimegalencephaly (iHME) is a rare form of congenital malformation of cortical development.It is characterized by enlargement of all or part of one cerebral hemisphere. It typically presents with intractable seizures, mental retardation, developmental delay, contralateral hemiparesis and hemianopia. The patient was a five and half month's old baby girl who presented first with focal seizures at 10th day of life. No other physical or behavioral abnormality was noted. However, Initial EEG showed excessive sharp EEG transients more over the right hemisphere, repeated EEG showed spikes, polyspikes, sharps and slow wave discharges predominately over right hemisphere. MRI brain ...


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