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2017

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Articles 241 - 270 of 399

Full-Text Articles in Pediatrics

Waiting 2 Minutes After Sucrose Administration—Unnecessary?, Naomi Meesters, Sinno Simons, Joost Van Rosmalen, Irwin Reiss, John Van Den Anker, Monique Van Dijk Mar 2017

Waiting 2 Minutes After Sucrose Administration—Unnecessary?, Naomi Meesters, Sinno Simons, Joost Van Rosmalen, Irwin Reiss, John Van Den Anker, Monique Van Dijk

Pediatrics Faculty Publications

Background Worldwide, oral sucrose is standard of care in many neonatal intensive care units to relieve procedural pain in neonates. This study aims to determine if time interval between sucrose administration and heelstick correlates with pain scores.

Methods Neonates were prospectively studied with variable time intervals and assessed with the Premature Infant Pain Profile-Revised (PIPP-R).

Results 150 neonates were included with a median gestational age of 30+6 (IQR 27+6–33+2) weeks and a median time interval of 72 (IQR 39–115) seconds between sucrose administration and heelstick. In multiple regression analysis, this time interval was not significantly …


Guideline For The Prevention Of Oral And Oropharyngeal Mucositis In Children Receiving Treatment For Cancer Or Undergoing Haematopoietic Stem Cell Transplantation, Lillian Sung, Paula Robinson, Nathaniel Treister, Tina Baggott, Paul Gibson, Wim Tissing, John Wiernikowski, Jennifer Brinklow, L Lee Dupuis Mar 2017

Guideline For The Prevention Of Oral And Oropharyngeal Mucositis In Children Receiving Treatment For Cancer Or Undergoing Haematopoietic Stem Cell Transplantation, Lillian Sung, Paula Robinson, Nathaniel Treister, Tina Baggott, Paul Gibson, Wim Tissing, John Wiernikowski, Jennifer Brinklow, L Lee Dupuis

Paediatrics Publications

PURPOSE: To develop an evidence-based clinical practice guideline for the prevention of oral mucositis in children (0-18 years) receiving treatment for cancer or undergoing haematopoietic stem cell transplantation (HSCT).

METHODS: The Mucositis Prevention Guideline Development Group was interdisciplinary and included internationally recognised experts in paediatric mucositis. For the evidence review, we included randomised controlled trials (RCTs) conducted in either children or adults evaluating the following interventions selected according to prespecified criteria: cryotherapy, low level light therapy (LLLT) and keratinocyte growth factor (KGF). We also examined RCTs of any intervention conducted in children. For all systematic reviews, we synthesised the occurrence …


Haemophilus Influenzae Type B Invasive Disease In Amish Children, Missouri, Usa, 2014, Angela L. Myers, Mary Anne Jackson, Lixin Zhang, Douglas S. Swanson, Janet R. Gilsdorf Mar 2017

Haemophilus Influenzae Type B Invasive Disease In Amish Children, Missouri, Usa, 2014, Angela L. Myers, Mary Anne Jackson, Lixin Zhang, Douglas S. Swanson, Janet R. Gilsdorf

Manuscripts, Articles, Book Chapters and Other Papers

© 2017, Centers for Disease Control and Prevention (CDC). All rights reserved. During 5 months in 2014, three Amish children in Missouri, USA, were diagnosed with invasive Haemophilus influenzae type b infection. Two were rural neighbors infected with a genetically similar rare strain, sequence type 45. One child had recently traveled, raising the possibility of maintenance of this strain among unvaccinated carriers in Amish communities.


Mucopolysaccharidosis Type I Newborn Screening: Best Practices For Diagnosis And Management., Lorne A. Clarke, Andrea M. Atherton, Barbara K. Burton, Debra L. Day-Salvatore, Paige Kaplan, Nancy D. Leslie, C Ronald Ronald Scott, David W. Stockton, Janet A. Thomas, Joseph Muenzer Mar 2017

Mucopolysaccharidosis Type I Newborn Screening: Best Practices For Diagnosis And Management., Lorne A. Clarke, Andrea M. Atherton, Barbara K. Burton, Debra L. Day-Salvatore, Paige Kaplan, Nancy D. Leslie, C Ronald Ronald Scott, David W. Stockton, Janet A. Thomas, Joseph Muenzer

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.


External Cardioversion Of Supraventricular Tachycardia In Omphalo-Thoracopagus Conjoined Twins., M Rizwan Afzal, Lindsey Malloy-Walton, Svjetlana Tisma-Dupanovic, Andrea L. Miles, Sanket Shah, John Papagiannis Mar 2017

External Cardioversion Of Supraventricular Tachycardia In Omphalo-Thoracopagus Conjoined Twins., M Rizwan Afzal, Lindsey Malloy-Walton, Svjetlana Tisma-Dupanovic, Andrea L. Miles, Sanket Shah, John Papagiannis

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.


The Art And Skill Of Delivering Culturally Responsive Trauma-Focused Cognitive Behavioral Therapy In Tanzania And Kenya., Briana Woods-Jaeger, Christine M. Kava, Christopher F. Akiba, Leah Lucid, Shannon Dorsey Mar 2017

The Art And Skill Of Delivering Culturally Responsive Trauma-Focused Cognitive Behavioral Therapy In Tanzania And Kenya., Briana Woods-Jaeger, Christine M. Kava, Christopher F. Akiba, Leah Lucid, Shannon Dorsey

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: This study explored the facilitators, barriers, and strategies used to deliver a child mental health evidence-based treatment (EBT), trauma-focused cognitive behavioral therapy (TF-CBT), in a culturally responsive manner. In low- and middle-income countries most individuals with mental health problems do not receive treatment due to a shortage of mental health professionals. One approach to addressing this problem is task-sharing, in which lay counselors are trained to deliver mental health treatment. Combining this approach with a focus on EBT provides a strategy for bridging the mental health treatment gap. However, little is known about how western-developed EBTs are delivered in …


Assessment Of Dietary Intake Of Children With Chronic Kidney Disease., Wun Fung Hui, Aisha Betoko, Jonathan D. Savant, Alison G. Abraham, Larry A. Greenbaum, Bradley A. Warady, Marva M. Moxey-Mims, Susan L. Furth Mar 2017

Assessment Of Dietary Intake Of Children With Chronic Kidney Disease., Wun Fung Hui, Aisha Betoko, Jonathan D. Savant, Alison G. Abraham, Larry A. Greenbaum, Bradley A. Warady, Marva M. Moxey-Mims, Susan L. Furth

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Our aim was to characterize the nutrient intake of children with chronic kidney disease (CKD) relative to recommended intake levels.

METHODS: We conducted a cross-sectional study of dietary intake assessed by Food Frequency Questionnaire (FFQ) in The North American Chronic Kidney Disease in Children (CKiD) prospective cohort study. Nutrient intake was analyzed to estimate the daily consumption levels of various nutrients and compared with national guidelines for intake.

RESULTS: There were 658 FFQs available for analysis; 69.9 % of respondents were boys, with a median age [Interquartile range (IQR)] of 11 years (8-15). Median daily sodium, potassium, and phosphorus …


Longitudinal Cardiovascular Outcomes Of Sleep Disordered Breathing In Children: A Meta-Analysis And Systematic Review., Zarmina Ehsan, Stacey L. Ishman, Thomas R. Kimball, Nanhua Zhang, Yuanshu Zou, Raouf S. Amin Mar 2017

Longitudinal Cardiovascular Outcomes Of Sleep Disordered Breathing In Children: A Meta-Analysis And Systematic Review., Zarmina Ehsan, Stacey L. Ishman, Thomas R. Kimball, Nanhua Zhang, Yuanshu Zou, Raouf S. Amin

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: The presence of sleep disordered breathing (SDB) is known to impact long-term cardiovascular morbidity in adults; however, the long-term effects in children are poorly understood. We aimed to systematically review and synthesize studies published to date on the long-term effects of SDB in children.

Study Design: Meta-analysis and systematic review using PubMed, CINAHL, Embase, and Scopus (all indexed years).

Methods: We searched for English-language articles containing original human data from prospective studies, with ≥7 participants, in children ≤18 years of age. Data regarding study design, demographics, clinical characteristics, outcomes, level of evidence, and risk of bias were obtained. Articles …


Ethical Concerns When Minors Act As Standardized Patients., Erwin Jiayuan Khoo, Robert Schremmer, Douglas S. Diekema, John Lantos Mar 2017

Ethical Concerns When Minors Act As Standardized Patients., Erwin Jiayuan Khoo, Robert Schremmer, Douglas S. Diekema, John Lantos

Manuscripts, Articles, Book Chapters and Other Papers

When minors are asked to assist medical educators by acting as standardized patients (SPs), there is a potential for the minors to be exploited. Minors deserve protection from exploitation. Such protection has been written into regulations governing medical research and into child labor laws. But there are no similar guidelines for minors' work in medical education. This article addresses the question of whether there should be rules. Should minors be required to give their informed consent or assent? Are there certain practices that could cause harm for the children who become SPs? We present a controversial case and ask a …


Parvovirus B19 Ns1 Protein Induces Cell Cycle Arrest At G2-Phase By Activating The Atr-Cdc25c-Cdk1 Pathway., Peng Xu, Zhe Zhou, Min Xiong, Wei Zou, Xuefeng Deng, Safder S. Ganaie, Steve Kleiboeker, Jianxin Peng, Kaiyu Liu, Shengqi Wang, Shui Qing Ye, Jianming Qiu Mar 2017

Parvovirus B19 Ns1 Protein Induces Cell Cycle Arrest At G2-Phase By Activating The Atr-Cdc25c-Cdk1 Pathway., Peng Xu, Zhe Zhou, Min Xiong, Wei Zou, Xuefeng Deng, Safder S. Ganaie, Steve Kleiboeker, Jianxin Peng, Kaiyu Liu, Shengqi Wang, Shui Qing Ye, Jianming Qiu

Manuscripts, Articles, Book Chapters and Other Papers

Human parvovirus B19 (B19V) infection of primary human erythroid progenitor cells (EPCs) arrests infected cells at both late S-phase and G2-phase, which contain 4N DNA. B19V infection induces a DNA damage response (DDR) that facilitates viral DNA replication but is dispensable for cell cycle arrest at G2-phase; however, a putative C-terminal transactivation domain (TAD2) within NS1 is responsible for G2-phase arrest. To fully understand the mechanism underlying B19V NS1-induced G2-phase arrest, we established two doxycycline-inducible B19V-permissive UT7/Epo-S1 cell lines that express NS1 or NS1mTAD2, and examined the function of the TAD2 domain during G2-phase arrest. The results confirm that the …


Impact Of Smartphone Digital Photography, Email, And Media Communication On Emergency Room Visits Post-Hypospadias Repair., Michael E. Chua, Megan A. Saunders, Paul R. Bowlin, Jessica M. Ming, Roberto Iglesias Lopes, Walid A. Farhat, Joana Dos Santos Mar 2017

Impact Of Smartphone Digital Photography, Email, And Media Communication On Emergency Room Visits Post-Hypospadias Repair., Michael E. Chua, Megan A. Saunders, Paul R. Bowlin, Jessica M. Ming, Roberto Iglesias Lopes, Walid A. Farhat, Joana Dos Santos

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Advances in communication technology are shaping our medical practice. To date, there is no clear evidence that this mode of communication will have any effect on unnecessary postoperative emergency room (ER) visits. We aim to evaluate the effect of email and media communication with application of smartphone digital photography on post-hypospadias repair ER visit rates.

METHODS: This prospective cohort study included all patients who underwent hypospadias repair performed by a single surgeon from October 2014 to November 2015. Patients were categorized into two groups: Group A consented for smartphone photography and email communication and Group B declined. Reason for …


Clinical Impact Of An Antibiotic Stewardship Program At A Children's Hospital., Brian R. Lee, Jennifer Goldman, Diana Yu, Angela Myers, Leslie M. Stach, Erin Hedican, Mary Anne Jackson, Jason G. Newland Mar 2017

Clinical Impact Of An Antibiotic Stewardship Program At A Children's Hospital., Brian R. Lee, Jennifer Goldman, Diana Yu, Angela Myers, Leslie M. Stach, Erin Hedican, Mary Anne Jackson, Jason G. Newland

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Antibiotic stewardship programs (ASP) improve appropriate antibiotic use. Data are limited on the clinical benefit of ASPs in children's hospitals. This study's objective was to determine the impact of an ASP on length of stay (LOS) and readmission rate among patients admitted to children's hospitals.

METHODS: Data from a prospective-audit-with-feedback ASP were used to examine the ASP review characteristics, including antibiotic(s) prescribed, clinical indication, recommendations made by the ASP, and agreement with recommendations. Propensity score analysis was utilized to determine the impact of the ASP on LOS and 30-day readmission based on whether the patient received an ASP recommendation …


Professional Practices, Training, And Funding Mechanisms: A Survey Of Pediatric Primary Care Psychologists, Kathryn W. Hoffses, Andrew R. Riley, Kathryn M. Menousek, Kriston B. Schellinger, Allison O. Grennan, Chrissy Cammarata, Jason L. Steadman Mar 2017

Professional Practices, Training, And Funding Mechanisms: A Survey Of Pediatric Primary Care Psychologists, Kathryn W. Hoffses, Andrew R. Riley, Kathryn M. Menousek, Kriston B. Schellinger, Allison O. Grennan, Chrissy Cammarata, Jason L. Steadman

Department of Pediatrics Faculty Papers

The integration of mental health services in primary care settings has expanded rapidly in recent years with psychologists being at the forefront of efforts to promote healthy behaviors, reduce disease, and care for behavioral, emotional, and developmental needs to promote overall health and well-being for children and families (Asarnow, Kolko, Miranda,&Kazak, 2017; Stancin& Perrin, 2014). While there are many psychologists working in pediatric primary care (PPC), little is known about the specific activities that these psychologists engage in, the training they receive, or funding mechanisms that support their work. This study sought to address this gap in the literature through …


Spotlight On The Integrated Primary Care Special Interest Group, Kathryn W. Hoffses, Andrew R. Riley, Kathryn M. Menousek, Kriston B. Schellinger, Allison O. Grennan, Chrissy Cammarata, Jason L. Steadman Mar 2017

Spotlight On The Integrated Primary Care Special Interest Group, Kathryn W. Hoffses, Andrew R. Riley, Kathryn M. Menousek, Kriston B. Schellinger, Allison O. Grennan, Chrissy Cammarata, Jason L. Steadman

Department of Pediatrics Faculty Papers

No abstract provided.


Competent For Unsupervised Practice: Use Of Pediatric Residency Training Milestones To Assess Readiness, Su-Ting T. Li, Daniel J. Tancredi, Alan Schwartz, Ann P. Guillot, Ann E. Burke, R. Franklin Trimm, Susan Guralnick, John D. Mahan, Kimberly A. Gifford Mar 2017

Competent For Unsupervised Practice: Use Of Pediatric Residency Training Milestones To Assess Readiness, Su-Ting T. Li, Daniel J. Tancredi, Alan Schwartz, Ann P. Guillot, Ann E. Burke, R. Franklin Trimm, Susan Guralnick, John D. Mahan, Kimberly A. Gifford

University Faculty and Staff Publications

Purpose

To describe clinical skills progression during pediatric residency using the distribution of pediatric milestone assessments by subcompetency and year of training and to determine reasonable milestone expectations at time of graduation.

Method

Multi-institutional cohort study of the milestones reported to the Accreditation Council for Graduate Medical Education for all 21 pediatric subcompetencies. Most subcompetencies were measured using five milestone levels (1 = novice, 2 = advanced beginner, 3 = competent, 4 = proficient, 5 = master); 3 subcompetencies had only four levels defined.

Results

Milestone assessments for 2,030 pediatric residents in 47 programs during academic year 2013–2014 were obtained. …


Outpatient Parenteral Antimicrobial Therapy In Pediatric Medicaid Enrollees., Jennifer Goldman, Troy Richardson, Jason G. Newland, Brian R. Lee, Jeffrey S. Gerber, Matt Hall, Matthew Kronman, Adam L. Hersh Mar 2017

Outpatient Parenteral Antimicrobial Therapy In Pediatric Medicaid Enrollees., Jennifer Goldman, Troy Richardson, Jason G. Newland, Brian R. Lee, Jeffrey S. Gerber, Matt Hall, Matthew Kronman, Adam L. Hersh

Manuscripts, Articles, Book Chapters and Other Papers

Background: Outpatient parenteral antimicrobial therapy (OPAT) is overused in cases where highly bioavailable oral alternatives would be equally effective. However, the scope of OPAT use for children nationwide is poorly understood. Our objective was to characterize OPAT use and clinical outcomes for a large population of pediatric Medicaid enrollees treated with OPAT.

Methods: We analyzed the Truven MarketScan Medicaid claims database between 2009 and 2012. An OPAT episode was identified by capturing children with claims data indicating home infusion therapy for an intravenous antimicrobial. We characterized OPAT use by describing patient demographics, diagnoses, and antimicrobials prescribed. We categorized an antimicrobial …


Every Child, Every Time: Universal Child Abuse Screening Increases Awareness And State Reporting (Poster)., Lauren Dudas Md, Gwenda L. Petrohoy, Debra Esernio-Jenssen Md, Cheryl Lansenderfer Bsn, Joseph J. Stirparo Md, Michele Dunstan, Marybeth Browne Md Mar 2017

Every Child, Every Time: Universal Child Abuse Screening Increases Awareness And State Reporting (Poster)., Lauren Dudas Md, Gwenda L. Petrohoy, Debra Esernio-Jenssen Md, Cheryl Lansenderfer Bsn, Joseph J. Stirparo Md, Michele Dunstan, Marybeth Browne Md

Department of Surgery

No abstract provided.


X-Linked Agammaglobulinemia - First Case With Bruton Tyrosine Kinase Mutation From Pakistan, Samreen Kulsom Zaidi, Sonia Qureshi, Farah Naz Qamar Mar 2017

X-Linked Agammaglobulinemia - First Case With Bruton Tyrosine Kinase Mutation From Pakistan, Samreen Kulsom Zaidi, Sonia Qureshi, Farah Naz Qamar

Department of Paediatrics and Child Health

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency with more than 600 mutations in Bruton tyrosine kinase (Bkt) gene which are responsible for early-onset agammaglobulinemia and repeated infections. Herein we present a case of a 3-year-old boy with history of repeated diarrhoea and an episode of meningoencephalitis with hemiplegia. The workup showed extremely low levels of immunoglobulin with low CD+19 cells. Genetic analysis showed Btk mutation 18 c.1883delCp.T628fs. To the best of our knowledge this is the first report of a case of XLA confirmed by molecular technique from Pakistan.


A Structured Blood Conservation Program In Pediatric Cardiac Surgery, A Budak, Kevin Mccusker, S Gunaydin Mar 2017

A Structured Blood Conservation Program In Pediatric Cardiac Surgery, A Budak, Kevin Mccusker, S Gunaydin

NYMC Faculty Publications

OBJECTIVE: The limitation of alternative transfusion practices in infants increases the benefits of blood conservation. We analyzed the efficacy of a structured program to reduce transfusions and transfusion-associated complications in cardiac surgery

PATIENTS AND METHODS: Our pediatric surgery database was reviewed retrospectively, comparing outcomes from two different time periods, after the implementation of an effective blood conservation program beginning in March 2014. A total of 214 infants (8.1±3.4 months) who underwent biventricular repair utilizing CPB (Group 1 – Blood conservation) were studied in a 12-month period (March 2014-February 2015) after the implementation of the new program, and compared with 250 …


Childhood Obesity: The Role Of The Mental Health Professional, Barry Panzer Mar 2017

Childhood Obesity: The Role Of The Mental Health Professional, Barry Panzer

NYMC Faculty Publications

This work consists of two major components: understanding the nature of childhood obesity and providing clinical services. Factors responsible for the current epidemic will be outlined, as will the current definition of the disorder. Statistical data regarding the epidemiology of weight disorders in childhood will be provided in order to give a perspective of the problem. Various obesity trajectories and their differential diagnostic and treatment issues will be thoroughly explored. The intervention section intends to help clinicians to evaluate salient factors in assessing the obese child and to identify appropriate goals and treatment methods. The course will provide vital information …


Determinants Of Neonatal Mortality In Rural And Urban Nigeria: Evidence From A Population-Based National Survey, Emmanuel O. Adewuyi, Yun Zhao Mar 2017

Determinants Of Neonatal Mortality In Rural And Urban Nigeria: Evidence From A Population-Based National Survey, Emmanuel O. Adewuyi, Yun Zhao

Research outputs 2014 to 2021

Background: Significant reduction in the global burden of neonatal mortality was achieved through the millennium development goals. In Nigeria, however, only a marginal reduction was realized. This study assesses the rural–urban differences in neonatal mortality rate (NMR) and the associated risk factors in Nigeria.

Methods: The dataset from the 2013 Nigeria demographic and health survey (NDHS), disaggregated by rural–urban residence (n = 20 449 and 9935, respectively), was explored using univariate, bivariate, and multivariable analysis. Complex samples analysis was applied to adjust for the unequal selection probabilities due to the multi-stage cluster sampling method used in the 2013 NDHS. The …


Embryonic Transcription Factor Expression In Mice Predicts Medial Amygdala Neuronal Identity And Sex-Specific Responses To Innate Behavioral Cues., Julieta E Lischinsky, Katie Sokolowski, Li Peijun, Shigeyuki Esumi, Yasmin Kamal, Meredith Goodrich, Livio Oboti, Timothy R Hammond, Meera Krishnamoorthy, Daniel Feldman, Molly Huntsman, Judy Liu, Joshua G Corbin Feb 2017

Embryonic Transcription Factor Expression In Mice Predicts Medial Amygdala Neuronal Identity And Sex-Specific Responses To Innate Behavioral Cues., Julieta E Lischinsky, Katie Sokolowski, Li Peijun, Shigeyuki Esumi, Yasmin Kamal, Meredith Goodrich, Livio Oboti, Timothy R Hammond, Meera Krishnamoorthy, Daniel Feldman, Molly Huntsman, Judy Liu, Joshua G Corbin

Pediatrics Faculty Publications

The medial subnucleus of the amygdala (MeA) plays a central role in processing sensory cues required for innate behaviors. However, whether there is a link between developmental programs and the emergence of inborn behaviors remains unknown. Our previous studies revealed that the telencephalic preoptic area (POA) embryonic niche is a novel source of MeA destined progenitors. Here, we show that the POA is comprised of distinct progenitor pools complementarily marked by the transcription factors Dbx1 and Foxp2. As determined by molecular and electrophysiological criteria this embryonic parcellation predicts postnatal MeA inhibitory neuronal subtype identity. We further find that Dbx1-derived and …


Anemia In Children With Palmar Pallor Aged 02 Months To 05 Years, Saroop Chand, Farzana Shaikh, Chetan Das, Yasmeen Memon, Mohammad Akbar Nizamani, Zulfiqar Ali Qutrio Baloch Feb 2017

Anemia In Children With Palmar Pallor Aged 02 Months To 05 Years, Saroop Chand, Farzana Shaikh, Chetan Das, Yasmeen Memon, Mohammad Akbar Nizamani, Zulfiqar Ali Qutrio Baloch

Department of Paediatrics and Child Health

Objective:
To determine the frequency of anemia in children with palmar pallor aged 02 months to 05 years.
Patients and Methods:
This cross sectional descriptive study of six months (01-12-2012 to 31-05-2013) was conducted in the department of paediatrics at Liaquat University Hospital Hyderabad. All the children, from 02 months to 05 years, of either gender had palmar pallor on examination were recruited and evaluated for anemia by assessing the level of haemoglobin and categorized anemia as mild, moderate and severe. The data was analyzed in SPSS version 16.00, the frequency and percentage was calculated. The chi-square test was applied …


Genetic Drivers Of Kidney Defects In The Digeorge Syndrome., Esther Lopez-Rivera, Yangfan P. Liu, Miguel Verbitsky, Blair R. Anderson, Valentina P. Capone, Edgar A. Otto, Zhonghai Yan, Adele Mitrotti, Jeremiah Martino, Nicholas J. Steers, David A. Fasel, Katarina Vukojevic, Rong Deng, Silvia E. Racedo, Qingxue Liu, Max Werth, Rik Westland, Asaf Vivante, Gabriel S. Makar, Monica Bodria, Matthew G. Sampson, Christopher E. Gillies, Virginia Vega-Warner, Mariarosa Maiorana, Donald S. Petrey, Barry Honig, Vladimir J. Lozanovski, Rémi Salomon, Laurence Heidet, Wassila Carpentier, Dominique Gaillard, Alba Carrea, Loreto Gesualdo, Daniele Cusi, Claudia Izzi, Francesco Scolari, Joanna A E Van Wijk, Adela Arapovic, Mirna Saraga-Babic, Marijan Saraga, Nenad Kunac, Ali Samii, Donna M. Mcdonald-Mcginn, Terrence B. Crowley, Elaine H. Zackai, Dorota Drozdz, Monika Miklaszewska, Marcin Tkaczyk, Przemyslaw Sikora, Maria Szczepanska, Malgorzata Mizerska-Wasiak, Grazyna Krzemien, Agnieszka Szmigielska, Marcin Zaniew, John M. Darlow, Prem Puri, David Barton, Emilio Casolari, Susan L. Furth, Bradley A. Warady, Zoran Gucev, Hakon Hakonarson, Hana Flogelova, Velibor Tasic, Anna Latos-Bielenska, Anna Materna-Kiryluk, Landino Allegri, Craig S. Wong, Iain A Drummond, Vivette D'Agati, Akira Imamoto, Jonathan M. Barasch, Friedhelm Hildebrandt, Krzysztof Kiryluk, Richard P. Lifton, Bernice E. Morrow, Cecile Jeanpierre, Virginia E. Papaioannou, Gian Marco Ghiggeri, Ali G. Gharavi, Nicholas Katsanis, Simone Sanna-Cherchi Feb 2017

Genetic Drivers Of Kidney Defects In The Digeorge Syndrome., Esther Lopez-Rivera, Yangfan P. Liu, Miguel Verbitsky, Blair R. Anderson, Valentina P. Capone, Edgar A. Otto, Zhonghai Yan, Adele Mitrotti, Jeremiah Martino, Nicholas J. Steers, David A. Fasel, Katarina Vukojevic, Rong Deng, Silvia E. Racedo, Qingxue Liu, Max Werth, Rik Westland, Asaf Vivante, Gabriel S. Makar, Monica Bodria, Matthew G. Sampson, Christopher E. Gillies, Virginia Vega-Warner, Mariarosa Maiorana, Donald S. Petrey, Barry Honig, Vladimir J. Lozanovski, Rémi Salomon, Laurence Heidet, Wassila Carpentier, Dominique Gaillard, Alba Carrea, Loreto Gesualdo, Daniele Cusi, Claudia Izzi, Francesco Scolari, Joanna A E Van Wijk, Adela Arapovic, Mirna Saraga-Babic, Marijan Saraga, Nenad Kunac, Ali Samii, Donna M. Mcdonald-Mcginn, Terrence B. Crowley, Elaine H. Zackai, Dorota Drozdz, Monika Miklaszewska, Marcin Tkaczyk, Przemyslaw Sikora, Maria Szczepanska, Malgorzata Mizerska-Wasiak, Grazyna Krzemien, Agnieszka Szmigielska, Marcin Zaniew, John M. Darlow, Prem Puri, David Barton, Emilio Casolari, Susan L. Furth, Bradley A. Warady, Zoran Gucev, Hakon Hakonarson, Hana Flogelova, Velibor Tasic, Anna Latos-Bielenska, Anna Materna-Kiryluk, Landino Allegri, Craig S. Wong, Iain A Drummond, Vivette D'Agati, Akira Imamoto, Jonathan M. Barasch, Friedhelm Hildebrandt, Krzysztof Kiryluk, Richard P. Lifton, Bernice E. Morrow, Cecile Jeanpierre, Virginia E. Papaioannou, Gian Marco Ghiggeri, Ali G. Gharavi, Nicholas Katsanis, Simone Sanna-Cherchi

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The DiGeorge syndrome, the most common of the microdeletion syndromes, affects multiple organs, including the heart, the nervous system, and the kidney. It is caused by deletions on chromosome 22q11.2; the genetic driver of the kidney defects is unknown.

METHODS: We conducted a genomewide search for structural variants in two cohorts: 2080 patients with congenital kidney and urinary tract anomalies and 22,094 controls. We performed exome and targeted resequencing in samples obtained from 586 additional patients with congenital kidney anomalies. We also carried out functional studies using zebrafish and mice.

RESULTS: We identified heterozygous deletions of 22q11.2 in 1.1% …


Topical Anaesthetics For Pain Control During Repair Of Dermal Laceration., Baraa O. Tayeb, Anthony Eidelman, Cristy L. Eidelman, Ewan D. Mcnicol, Daniel B. Carr Feb 2017

Topical Anaesthetics For Pain Control During Repair Of Dermal Laceration., Baraa O. Tayeb, Anthony Eidelman, Cristy L. Eidelman, Ewan D. Mcnicol, Daniel B. Carr

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Topical local anaesthetics provide effective analgesia for patients undergoing numerous superficial procedures, including repair of dermal lacerations. The need for cocaine in topical anaesthetic formulations has been questioned because of concern about adverse effects, thus novel preparations of cocaine-free anaesthetics have been developed. This review was originally published in 2011 and has been updated in 2017.

OBJECTIVES: To assess whether benefits of non-invasive topical anaesthetic application occur at the expense of decreased analgesic efficacy. To compare the efficacy of various single-component or multi-component topical anaesthetic agents for repair of dermal lacerations. To determine the clinical necessity for topical application …


Evaluation Of Treatment-Related Mortality Among Paediatric Cancer Deaths: A Population Based Analysis., Jason D Pole, Paul Gibson, Marie-Chantal Ethier, Tanya Lazor, Donna L Johnston, Carol Portwine, Mariana Silva, Sarah Alexander, Lillian Sung Feb 2017

Evaluation Of Treatment-Related Mortality Among Paediatric Cancer Deaths: A Population Based Analysis., Jason D Pole, Paul Gibson, Marie-Chantal Ethier, Tanya Lazor, Donna L Johnston, Carol Portwine, Mariana Silva, Sarah Alexander, Lillian Sung

Paediatrics Publications

BACKGROUND: Objectives were to describe the proportion of deaths due to treatment-related mortality (TRM) and to identify risk factors and probable causes of TRM among paediatric cancer deaths in a population-based cohort.

METHODS: We included children with cancer ⩽18 years diagnosed and treated in Ontario who died between January 2003 and December 2012. Deaths were identified using a provincial registry, the Pediatric Oncology Group of Ontario Networked Information System. Probable causes of TRM were described.

RESULTS: Among the 964 deaths identified, 821 were included. The median age at diagnosis was 6.6 years (range 0-18.8) and 51.8% had at least one …


Using Quality Improvement Methods To Increase Use Of Pain Prevention Strategies For Childhood Vaccination., Jennifer Verrill Schurman, Amanda D. Deacy, Rebecca J. Johnson, Jolynn Parker, Kristi Williams, Dustin Wallace, Mark Connelly, Lynn Anson, Kevin Mroczka Feb 2017

Using Quality Improvement Methods To Increase Use Of Pain Prevention Strategies For Childhood Vaccination., Jennifer Verrill Schurman, Amanda D. Deacy, Rebecca J. Johnson, Jolynn Parker, Kristi Williams, Dustin Wallace, Mark Connelly, Lynn Anson, Kevin Mroczka

Manuscripts, Articles, Book Chapters and Other Papers

AIM: To increase evidence-based pain prevention strategy use during routine vaccinations in a pediatric primary care clinic using quality improvement methodology.

METHODS: Specific intervention strategies (i.e., comfort positioning, nonnutritive sucking and sucrose analgesia, distraction) were identified, selected and introduced in three waves, using a Plan-Do-Study-Act framework. System-wide change was measured from baseline to post-intervention by: (1) percent of vaccination visits during which an evidence-based pain prevention strategy was reported as being used; and (2) caregiver satisfaction ratings following the visit. Additionally, self-reported staff and caregiver attitudes and beliefs about pain prevention were measured at baseline and 1-year post-intervention …


Intestinal Microbiota And Weight-Gain In Preterm Neonates, Silvia Arboleya, Pablo Martinez-Camblor, Gonzalo Solís, Marta Suárez Feb 2017

Intestinal Microbiota And Weight-Gain In Preterm Neonates, Silvia Arboleya, Pablo Martinez-Camblor, Gonzalo Solís, Marta Suárez

Dartmouth Scholarship

The involvement of the gut microbiota on weight-gain and its relationship with childhood undernutrition and growth has been reported. Thus, the gut microbiota constitutes a potential therapeutic target for preventing growth impairment. However, our knowledge in this area is limited. In this study we aimed at evaluating the relationship among early microbiota, growth, and development in preterm infants. To this end we assessed the levels of specific microorganisms by qPCR, and those of short chain fatty acids by mean of gas-chromatography, in feces from 63 preterm newborns and determined their weight-gain during the first months. The statistical analyses performed indicate …


Ux007 For The Treatment Of Long Chain-Fatty Acid Oxidation Disorders: Safety And Efficacy In Children And Adults Following 24weeks Of Treatment., J Vockley, B Burton, G T Berry, N Longo, J Phillips, Pranoot Tanpaiboon, +Several Additional Authors Feb 2017

Ux007 For The Treatment Of Long Chain-Fatty Acid Oxidation Disorders: Safety And Efficacy In Children And Adults Following 24weeks Of Treatment., J Vockley, B Burton, G T Berry, N Longo, J Phillips, Pranoot Tanpaiboon, +Several Additional Authors

Pediatrics Faculty Publications

BACKGROUND: Long-chain fatty acid oxidation disorders (LC-FAOD) lead to accumulation of high concentrations of potentially toxic fatty acid intermediates. Newborn screening and early intervention have reduced mortality, but most patients continue to experience frequent hospitalizations and significant morbidity despite treatment. The deficient energy state can cause serious liver, muscle, and heart disease, and may be associated with an increased risk of sudden death. Triheptanoin is a medium odd-chain fatty acid. Anaplerotic metabolites of triheptanoin have the potential to replace deficient tricarboxylic acid (TCA) cycle intermediates, resulting in net glucose production as a novel energy source for the treatment of LC-FAOD. …


Junctional Ectopic Tachycardia Secondary To Myocarditis Associated With Sudden Cardiac Arrest, Michael E. A. Cunningham, Robin W. Doroshow, Laura Oliveri, Jeffrey P. Moak Feb 2017

Junctional Ectopic Tachycardia Secondary To Myocarditis Associated With Sudden Cardiac Arrest, Michael E. A. Cunningham, Robin W. Doroshow, Laura Oliveri, Jeffrey P. Moak

Pediatrics Faculty Publications

No abstract provided.