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Full-Text Articles in Medicine and Health Sciences

The Supply And Demand Of The Cardiovascular Workforce: Striking The Right Balance., Akhil Narang, Shashank S. Sinha, Bharath Rajagopalan, Nkechinyere N. Ijioma, Natalie Jayaram, Aaron P. Kithcart, Varsha K. Tanguturi, Michael W. Cullen Oct 2016

The Supply And Demand Of The Cardiovascular Workforce: Striking The Right Balance., Akhil Narang, Shashank S. Sinha, Bharath Rajagopalan, Nkechinyere N. Ijioma, Natalie Jayaram, Aaron P. Kithcart, Varsha K. Tanguturi, Michael W. Cullen

Manuscripts, Articles, Book Chapters and Other Papers

As the burden of cardiovascular disease in the United States continues to increase, uncertainty remains on how well-equipped the cardiovascular workforce is to meet the challenges that lie ahead. In a time when health care is rapidly shifting, numerous factors affect the supply and demand of the cardiovascular workforce. This Council Commentary critically examines several factors that influence the cardiovascular workforce. These include current workforce demographics and projections, evolving health care and practice environments, and the increasing burden of cardiovascular disease. Finally, we propose 3 strategies to optimize the workforce. These focus on cardiovascular disease prevention, the effective utilization of …


Biallelic Mutations In Tbcd, Encoding The Tubulin Folding Cofactor D, Perturb Microtubule Dynamics And Cause Early-Onset Encephalopathy., Elisabetta Flex, Marcello Niceta, Serena Cecchetti, Isabelle Thiffault, Margaret G. Au, Alessandro Capuano, Emanuela Piermarini, Anna A. Ivanova, Joshua W. Francis, Giovanni Chillemi, Balasubramanian Chandramouli, Giovanna Carpentieri, Charlotte A. Haaxma, Andrea Ciolfi, Simone Pizzi, Ganka V. Douglas, Kara Levine, Antonella Sferra, Maria Lisa Dentici, Rolph R. Pfundt, Jean-Baptist Lepichon, Emily G. Farrow, Frank Baas, Fiorella Piemonte, Bruno Dallapiccola, John M. Graham, Carol J. Saunders, Enrico Bertini, Richard A. Kahn, David A. Koolen, Marco Tartaglia Oct 2016

Biallelic Mutations In Tbcd, Encoding The Tubulin Folding Cofactor D, Perturb Microtubule Dynamics And Cause Early-Onset Encephalopathy., Elisabetta Flex, Marcello Niceta, Serena Cecchetti, Isabelle Thiffault, Margaret G. Au, Alessandro Capuano, Emanuela Piermarini, Anna A. Ivanova, Joshua W. Francis, Giovanni Chillemi, Balasubramanian Chandramouli, Giovanna Carpentieri, Charlotte A. Haaxma, Andrea Ciolfi, Simone Pizzi, Ganka V. Douglas, Kara Levine, Antonella Sferra, Maria Lisa Dentici, Rolph R. Pfundt, Jean-Baptist Lepichon, Emily G. Farrow, Frank Baas, Fiorella Piemonte, Bruno Dallapiccola, John M. Graham, Carol J. Saunders, Enrico Bertini, Richard A. Kahn, David A. Koolen, Marco Tartaglia

Manuscripts, Articles, Book Chapters and Other Papers

Microtubules are dynamic cytoskeletal elements coordinating and supporting a variety of neuronal processes, including cell division, migration, polarity, intracellular trafficking, and signal transduction. Mutations in genes encoding tubulins and microtubule-associated proteins are known to cause neurodevelopmental and neurodegenerative disorders. Growing evidence suggests that altered microtubule dynamics may also underlie or contribute to neurodevelopmental disorders and neurodegeneration. We report that biallelic mutations in TBCD, encoding one of the five co-chaperones required for assembly and disassembly of the αβ-tubulin heterodimer, the structural unit of microtubules, cause a disease with neurodevelopmental and neurodegenerative features characterized by early-onset cortical atrophy, secondary hypomyelination, microcephaly, thin …


Rationale And Design Of The Children's Oncology Group (Cog) Study Alte1621: A Randomized, Placebo-Controlled Trial To Determine If Low-Dose Carvedilol Can Prevent Anthracycline-Related Left Ventricular Remodeling In Childhood Cancer Survivors At High Risk For Developing Heart Failure., Saro H. Armenian, Melissa M. Hudson, Ming Hui Chen, Steven D. Colan, Lanie Lindenfeld, George Mills, Aida Siyahian, Sarah Gelehrter, Ha Dang, Wendy Hein, Daniel M M. Green, Leslie L. Robison, F Lennie Wong, Pamela S. Douglas, Smita Bhatia Oct 2016

Rationale And Design Of The Children's Oncology Group (Cog) Study Alte1621: A Randomized, Placebo-Controlled Trial To Determine If Low-Dose Carvedilol Can Prevent Anthracycline-Related Left Ventricular Remodeling In Childhood Cancer Survivors At High Risk For Developing Heart Failure., Saro H. Armenian, Melissa M. Hudson, Ming Hui Chen, Steven D. Colan, Lanie Lindenfeld, George Mills, Aida Siyahian, Sarah Gelehrter, Ha Dang, Wendy Hein, Daniel M M. Green, Leslie L. Robison, F Lennie Wong, Pamela S. Douglas, Smita Bhatia

Manuscripts, Articles, Book Chapters and Other Papers

Background: Anthracyclines are widely used in the treatment of childhood cancer. One of the well-recognized side-effects of anthracycline therapy is dose-dependent cardiomyopathy that may progress to heart failure (HF) years after completion of cancer-directed therapy. This study will evaluate the efficacy of low-dose beta-blocker (carvedilol) for HF risk reduction in childhood cancer survivors at highest risk for HF. The proposed intervention has the potential to significantly reduce chronic cardiac injury via interruption of neurohormonal systems responsible for left ventricular (LV) remodeling, resulting in improved cardiac function and decreased risk of HF. The intervention is informed by previous studies demonstrating efficacy …


User-Centered Design Of Multi-Gene Sequencing Panel Reports For Clinicians., Elizabeth Cutting, Meghan Banchero, Amber L. Beitelshees, James J. Cimino, Guilherme Del Fiol, Ayse P. Gurses, Mark A. Hoffman, Linda Jo Bone Jeng, Kensaku Kawamoto, Mark Kelemen, Harold Alan Pincus, Alan R. Shuldiner, Marc S. Williams, Toni I. Pollin, Casey Lynnette Overby Oct 2016

User-Centered Design Of Multi-Gene Sequencing Panel Reports For Clinicians., Elizabeth Cutting, Meghan Banchero, Amber L. Beitelshees, James J. Cimino, Guilherme Del Fiol, Ayse P. Gurses, Mark A. Hoffman, Linda Jo Bone Jeng, Kensaku Kawamoto, Mark Kelemen, Harold Alan Pincus, Alan R. Shuldiner, Marc S. Williams, Toni I. Pollin, Casey Lynnette Overby

Manuscripts, Articles, Book Chapters and Other Papers

The objective of this study was to develop a high-fidelity prototype for delivering multi-gene sequencing panel (GS) reports to clinicians that simulates the user experience of a final application. The delivery and use of GS reports can occur within complex and high-paced healthcare environments. We employ a user-centered software design approach in a focus group setting in order to facilitate gathering rich contextual information from a diverse group of stakeholders potentially impacted by the delivery of GS reports relevant to two precision medicine programs at the University of Maryland Medical Center. Responses from focus group sessions were transcribed, coded and …


Bioactivation Of Trimethoprim To Protein-Reactive Metabolites In Human Liver Microsomes., Jennifer Goldman, Yakov M. Koen, Steven A. Rogers, Kelin Li, J Steven Leeder, Robert P. Hanzlik Oct 2016

Bioactivation Of Trimethoprim To Protein-Reactive Metabolites In Human Liver Microsomes., Jennifer Goldman, Yakov M. Koen, Steven A. Rogers, Kelin Li, J Steven Leeder, Robert P. Hanzlik

Manuscripts, Articles, Book Chapters and Other Papers

The formation of drug-protein adducts via metabolic activation and covalent binding may stimulate an immune response or may result in direct cell toxicity. Protein covalent binding is a potentially pivotal step in the development of idiosyncratic adverse drug reactions (IADRs). Trimethoprim (TMP)-sulfamethoxazole (SMX) is a combination antibiotic that commonly causes IADRs. Recent data suggest that the contribution of the TMP component of TMP-SMX to IADRs may be underappreciated. We previously demonstrated that TMP is bioactivated to chemically reactive intermediates that can be trapped in vitro by N-acetyl cysteine (NAC), and we have detected TMP-NAC adducts (i.e., mercapturic acids) in the …


Early Cumulative Supplemental Oxygen Predicts Bronchopulmonary Dysplasia In High Risk Extremely Low Gestational Age Newborns., Katherine C. Wai, Michael A. Kohn, Roberta A. Ballard, William E. Truog, Dennis M. Black, Jeanette M. Asselin, Philip L. Ballard, Elizabeth E. Rogers, Roberta L. Keller, Trial Of Late Surfactant (Tolsurf) Study Group Oct 2016

Early Cumulative Supplemental Oxygen Predicts Bronchopulmonary Dysplasia In High Risk Extremely Low Gestational Age Newborns., Katherine C. Wai, Michael A. Kohn, Roberta A. Ballard, William E. Truog, Dennis M. Black, Jeanette M. Asselin, Philip L. Ballard, Elizabeth E. Rogers, Roberta L. Keller, Trial Of Late Surfactant (Tolsurf) Study Group

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To assess the prognostic accuracy of early cumulative supplemental oxygen (CSO) exposure for prediction of bronchopulmonary dysplasia (BPD) or death, and to evaluate the independent association of CSO with BPD or death.

STUDY DESIGN: We performed a secondary analysis of the Trial of Late Surfactant, which enrolled 511 infants born at ≤28 weeks gestational age who were mechanically ventilated at 7-14 days of life. Our primary outcome was BPD or death at 36 weeks postmenstrual age, as determined by a physiological oxygen/flow challenge. Average daily supplemental oxygen (fraction of inspired oxygen - 0.21) was calculated. CSO was calculated as …


The Influence Of Televised Food Commercials On Children's Food Choices: Evidence From Ventromedial Prefrontal Cortex Activations., Amanda S. Bruce, Stephen W. Pruitt, Oh-Ryeong Ha, J Bradley C Cherry, Timothy R. Smith, Jared M. Bruce, Seung-Lark Lim Oct 2016

The Influence Of Televised Food Commercials On Children's Food Choices: Evidence From Ventromedial Prefrontal Cortex Activations., Amanda S. Bruce, Stephen W. Pruitt, Oh-Ryeong Ha, J Bradley C Cherry, Timothy R. Smith, Jared M. Bruce, Seung-Lark Lim

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To investigate how food commercials influence children's food choices.

STUDY DESIGN: Twenty-three children ages 8-14 years provided taste and health ratings for 60 food items. Subsequently, these children were scanned with the use of functional magnetic resonance imaging while making food choices (ie, "eat" or "not eat") after watching food and nonfood television commercials.

RESULTS: Our results show that watching food commercials changes the way children consider the importance of taste when making food choices. Children did not use health values for their food choices, indicating children's decisions were largely driven by hedonic, immediate rewards (ie, "tastiness"); however, children …


Developing Novel Machine Learning Algorithms To Improve Sedentary Assessment For Youth Health Enhancement., Gowtham Kumar Golla, Jordan A. Carlson, Jun Huan, Jacqueline Kerr, Tarrah Mitchell, Kelsey Borner Oct 2016

Developing Novel Machine Learning Algorithms To Improve Sedentary Assessment For Youth Health Enhancement., Gowtham Kumar Golla, Jordan A. Carlson, Jun Huan, Jacqueline Kerr, Tarrah Mitchell, Kelsey Borner

Manuscripts, Articles, Book Chapters and Other Papers

Sedentary behavior of youth is an important determinant of health. However, better measures are needed to improve understanding of this relationship and the mechanisms at play, as well as to evaluate health promotion interventions. Wearable accelerometers are considered as the standard for assessing physical activity in research, but do not perform well for assessing posture (i.e., sitting vs. standing), a critical component of sedentary behavior. The machine learning algorithms that we propose for assessing sedentary behavior will allow us to re-examine existing accelerometer data to better understand the association between sedentary time and health in various populations. We collected two …


Ontogeny Of Hepatic Drug Transporters As Quantified By Lc-Ms/Ms Proteomics., B Prasad, Andrea Gaedigk, M Vrana, R Gaedigk, J Steven Leeder, L Salphati, X Chu, G Xiao, Ceca Hop, R Evers, L Gan, J D Unadkat Oct 2016

Ontogeny Of Hepatic Drug Transporters As Quantified By Lc-Ms/Ms Proteomics., B Prasad, Andrea Gaedigk, M Vrana, R Gaedigk, J Steven Leeder, L Salphati, X Chu, G Xiao, Ceca Hop, R Evers, L Gan, J D Unadkat

Manuscripts, Articles, Book Chapters and Other Papers

Protein expression of major hepatic uptake and efflux drug transporters in human pediatric (n = 69) and adult (n = 41) livers was quantified by liquid chromatography / tandem mass spectroscopy (LC-MS/MS). Transporter protein expression of OCT1, OATP1B3, P-gp, and MRP3 was age-dependent. Particularly, significant differences were observed in transporter expression (P < 0.05) between the following age groups: neonates vs. adults (OCT1, OATP1B3, P-gp), neonates or infants vs. adolescents and/or adults (OCT1, OATP1B3, and P-gp), infants vs. children (OATP1B3 and P-gp), and adolescents vs. adults (MRP3). OCT1 showed the largest increase, of almost 5-fold, in protein expression with age. Ontogenic expression of OATP1B1 was confounded by genotype and was revealed only in livers harboring SLCO1B1*1A/*1A. In livers >1 year, tissues harboring SLCO1B1*14/*1A showed 2.5-fold higher (P < 0.05) protein expression than SLCO1B1*15/*1A. Integration of these ontogeny data in physiologically based pharmacokinetic (PBPK) models will be a crucial step in predicting hepatic drug disposition in children.


The Effect Of Bony Parameters On The Pediatric Knee: Normal Versus Anterior Cruciate Ligament Injury Versus Tibial Spine Avulsion Fracture., Kenneth A. Shaw, Brian S. Dunoski, Neil J. Mardis, Donna M. Pacicca Oct 2016

The Effect Of Bony Parameters On The Pediatric Knee: Normal Versus Anterior Cruciate Ligament Injury Versus Tibial Spine Avulsion Fracture., Kenneth A. Shaw, Brian S. Dunoski, Neil J. Mardis, Donna M. Pacicca

Manuscripts, Articles, Book Chapters and Other Papers

Purpose  Anterior cruciate ligament (ACL) injuries can present as a ligamentous disruption or avulsion fracture of the tibial spine in pediatric patients. Differences in knee morphometric parameters have been investigated between pediatric cohorts with ACL disruptions and tibial spine avulsion fractures. However, no study to date has compared morphometric parameters in patients with tibial spine avulsion fracture against a control population. Methods  A retrospective review of pediatric patients undergoing knee magnetic resonance imaging (MRI) studies was performed, identifying 15 patients with tibial spine avulsion fracture between January 1, 2009, and January 1, 2013. Inclusionary criteria consisted of patients who sustained …


Decreasing Patient Cost And Travel Time Through Pediatric Rheumatology Telemedicine Visits., Elizabeth A. Kessler, Ashley K. Sherman, Mara L. Becker Sep 2016

Decreasing Patient Cost And Travel Time Through Pediatric Rheumatology Telemedicine Visits., Elizabeth A. Kessler, Ashley K. Sherman, Mara L. Becker

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: There is a critical shortage of pediatric rheumatologists in the US. Substantial travel to clinics can impose time and monetary burdens on families. The aim of this study was to evaluate the cost of in-person pediatric rheumatology visits for families and determine if telemedicine clinics resulted in time and cost savings. Factors associated with interest in telemedicine were also explored.

METHODS: Surveys were offered to parents and guardians of patients in Pediatric Rheumatology follow-up clinics in Kansas City, Missouri, the primary site of in-person care, and at a telemedicine outreach site 160 miles away, in Joplin, Missouri. Survey questions …


Predictors Of Infant Age At Enrollment In Early Infant Diagnosis Services In Kenya., Kathy Goggin, Catherine Wexler, Niaman Nazir, Vincent S. Staggs, Brad Gautney, Vincent Okoth, Samoel A Khamadi, Andrea Ruff, Michael Sweat, An-Lin Cheng, Sarah Finocchario-Kessler Sep 2016

Predictors Of Infant Age At Enrollment In Early Infant Diagnosis Services In Kenya., Kathy Goggin, Catherine Wexler, Niaman Nazir, Vincent S. Staggs, Brad Gautney, Vincent Okoth, Samoel A Khamadi, Andrea Ruff, Michael Sweat, An-Lin Cheng, Sarah Finocchario-Kessler

Manuscripts, Articles, Book Chapters and Other Papers

Despite the importance of early detection to signal lifesaving treatment initiation for HIV+ infants, early infant diagnosis (EID) services have received considerably less attention than other aspects of prevention of mother to child transmission care. This study draws on baseline data from an on-going cluster randomized study of an intervention to improve EID services at six government hospitals across Kenya. Two logistic regressions examined potential predictors of "on time" (infant ≤6 weeks of age) vs. "late" (≥7 weeks) and "on time" versus "very late" (≥12 weeks) EID engagement among 756 mother-infant pairs. A quarter of the infants failed to get …


Pediatric Statin Administration: Navigating A Frontier With Limited Data., Jonathan B. Wagner, Susan M. Abdel-Rahman Sep 2016

Pediatric Statin Administration: Navigating A Frontier With Limited Data., Jonathan B. Wagner, Susan M. Abdel-Rahman

Manuscripts, Articles, Book Chapters and Other Papers

Increasingly, children and adolescents with dyslipidemia qualify for pharmacologic intervention. As they are for adults, 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase inhibitors (statins) are the mainstay of pediatric dyslipidemia treatment when lifestyle modifications have failed. Despite the overall success of these drugs, the magnitude of variability in dose-exposure-response profiles contributes to adverse events and treatment failure. In children, the cause of treatment failures remains unclear. This review describes the updated guidelines for screening and management of pediatric dyslipidemia and statin disposition pathway to assist the provider in recognizing scenarios where alterations in dosage may be warranted to meet patients' specific needs.


A Hypothesis For Using Pathway Genetic Load Analysis For Understanding Complex Outcomes In Bilirubin Encephalopathy, Sean M. Riordan, Douglas C. Bittel, Jean-Baptist Lepichon, Silvia Gazzin, Claudio Tiribelli, Jon F. Watchko, Richard P. Wennberg, Steven Shapiro Aug 2016

A Hypothesis For Using Pathway Genetic Load Analysis For Understanding Complex Outcomes In Bilirubin Encephalopathy, Sean M. Riordan, Douglas C. Bittel, Jean-Baptist Lepichon, Silvia Gazzin, Claudio Tiribelli, Jon F. Watchko, Richard P. Wennberg, Steven Shapiro

Manuscripts, Articles, Book Chapters and Other Papers

© 2016 Riordan, Bittel, Le Pichon, Gazzin, Tiribelli, Watchko, Wennberg and Shapiro.

Genetic-based susceptibility to bilirubin neurotoxicity and chronic bilirubin encephalopathy (kernicterus) is still poorly understood. Neonatal jaundice affects 60-80% of newborns, and considerable effort goes into preventing this relatively benign condition from escalating into the development of kernicterus making the incidence of this potentially devastating condition very rare in more developed countries. The current understanding of the genetic background of kernicterus is largely comprised of mutations related to alterations of bilirubin production, elimination, or both. Less is known about mutations that may predispose or protect against CNS bilirubin neurotoxicity. …


Failure To Thrive Hospitalizations And Risk Factors For Readmission To Children's Hospitals., Henry T. Puls, Matt Hall, Jessica L. Bettenhausen, Matthew B. Johnson, Christina Peacock, Jean L. Raphael, Jason G. Newland, Jeffrey D. Colvin Aug 2016

Failure To Thrive Hospitalizations And Risk Factors For Readmission To Children's Hospitals., Henry T. Puls, Matt Hall, Jessica L. Bettenhausen, Matthew B. Johnson, Christina Peacock, Jean L. Raphael, Jason G. Newland, Jeffrey D. Colvin

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Risk factors for failure to thrive (FTT) readmissions, including medical complexity, have not been described. We sought to characterize children hospitalized for FTT and identify risk factors associated with FTT-specific readmissions during the current era of increasing medical complexity among hospitalized children.

METHODS: This retrospective cohort study used the Pediatric Health Information System database of 43 freestanding children's hospitals across the United States. The cohort included childrenmodels, we assessed the association of demographic, clinical, diagnostic, and treatment characteristics with FTT-specific readmission.

RESULTS: There were 10 499 FTT hospitalizations, with 14.1% being readmitted for FTT within 3 years and 4.8% …


Clinical Detection Of Deletion Structural Variants In Whole-Genome Sequences., Aaron C. Noll, Neil A. Miller, Laurie D. Smith, Byunggil Yoo, Stephanie Fiedler, Linda D. Cooley, Laurel K. Willig, Josh E. Petrikin, Julie Cakici, John Lesko, Angela Newton, Kali Detherage, Isabelle Thiffault, Carol J. Saunders, Emily G. Farrow, Stephen F. Kingsmore Aug 2016

Clinical Detection Of Deletion Structural Variants In Whole-Genome Sequences., Aaron C. Noll, Neil A. Miller, Laurie D. Smith, Byunggil Yoo, Stephanie Fiedler, Linda D. Cooley, Laurel K. Willig, Josh E. Petrikin, Julie Cakici, John Lesko, Angela Newton, Kali Detherage, Isabelle Thiffault, Carol J. Saunders, Emily G. Farrow, Stephen F. Kingsmore

Manuscripts, Articles, Book Chapters and Other Papers

Optimal management of acutely ill infants with monogenetic diseases requires rapid identification of causative haplotypes. Whole-genome sequencing (WGS) has been shown to identify pathogenic nucleotide variants in such infants. Deletion structural variants (DSVs, >50 nt) are implicated in many genetic diseases, and tools have been designed to identify DSVs using short-read WGS. Optimisation and integration of these tools into a WGS pipeline could improve diagnostic sensitivity and specificity of WGS. In addition, it may improve turnaround time when compared with current CNV assays, enhancing utility in acute settings. Here we describe DSV detection methods for use in WGS for rapid …


Reliability Of Pressure Ulcer Rates: How Precisely Can We Differentiate Among Hospital Units, And Does The Standard Signal-Noise Reliability Measure Reflect This Precision?, Vincent S. Staggs, Emily Cramer Aug 2016

Reliability Of Pressure Ulcer Rates: How Precisely Can We Differentiate Among Hospital Units, And Does The Standard Signal-Noise Reliability Measure Reflect This Precision?, Vincent S. Staggs, Emily Cramer

Manuscripts, Articles, Book Chapters and Other Papers

Hospital performance reports often include rankings of unit pressure ulcer rates. Differentiating among units on the basis of quality requires reliable measurement. Our objectives were to describe and apply methods for assessing reliability of hospital-acquired pressure ulcer rates and evaluate a standard signal-noise reliability measure as an indicator of precision of differentiation among units. Quarterly pressure ulcer data from 8,199 critical care, step-down, medical, surgical, and medical-surgical nursing units from 1,299 US hospitals were analyzed. Using beta-binomial models, we estimated between-unit variability (signal) and within-unit variability (noise) in annual unit pressure ulcer rates. Signal-noise reliability was computed as the ratio …


Magic Study: Aims, Design And Methods Using Systemchange™ To Improve Immunosuppressive Medication Adherence In Adult Kidney Transplant Recipients., Cynthia L. Russell, Shirley Moore, Donna Hathaway, An-Lin Cheng, Guoqing Chen, Kathy Goggin Jul 2016

Magic Study: Aims, Design And Methods Using Systemchange™ To Improve Immunosuppressive Medication Adherence In Adult Kidney Transplant Recipients., Cynthia L. Russell, Shirley Moore, Donna Hathaway, An-Lin Cheng, Guoqing Chen, Kathy Goggin

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Among adult kidney transplant recipients, non-adherence to immunosuppressive medications is the leading predictor of poor outcomes, including rejection, kidney loss, and death. An alarming one-third of kidney transplant patients experience medication non-adherence even though the problem is preventable. Existing adherence interventions have proven marginally effective for those with acute and chronic illnesses and ineffective for adult kidney transplant recipients. Our purpose is to describe the design and methods of the MAGIC (Medication Adherence Given Individual SystemCHANGE™) trial

METHODS/DESIGN: We report the design of a randomized controlled trial with an attention-control group to test an innovative 6-month SystemCHANGE™ intervention designed …


Outcomes After Pediatric Fundoplication: Defining The Redo Population, J E. Baerg, E E. Perrone, R A. Vannix, D L. Thorpe, A Gasior, Shawn D. St Peter Jul 2016

Outcomes After Pediatric Fundoplication: Defining The Redo Population, J E. Baerg, E E. Perrone, R A. Vannix, D L. Thorpe, A Gasior, Shawn D. St Peter

Manuscripts, Articles, Book Chapters and Other Papers

Objective: The aims were to compare outcome variables in children with gastroesophageal reflux disease (GERD) and one Nissen fundoplication to children with redo fundoplications and define the pediatric redo population. Methods: After IRB approval (#5100277), a case control study was conducted of children younger than 18 years, from two children’s hospitals, with one Nissen fundoplication (control group) or a redo performed between January 1995 and March 2011. Complete data were collected by phone calls to caregivers in December 2012. Only redo operations performed after recurrence of GERD symptoms and wrap herniation into the mediastinum confirmed by contrast radiograph were included. …


Decreased Pregnane X Receptor Expression In Children With Active Crohn's Disease, Valentina Shakhnovich, Carrie A. Vyhlidal, Craig A. Friesen, Amber Hildreth, Vivekanand Singh, James Daniel, Gregory L. Kearns, J Steven Leeder Jul 2016

Decreased Pregnane X Receptor Expression In Children With Active Crohn's Disease, Valentina Shakhnovich, Carrie A. Vyhlidal, Craig A. Friesen, Amber Hildreth, Vivekanand Singh, James Daniel, Gregory L. Kearns, J Steven Leeder

Manuscripts, Articles, Book Chapters and Other Papers

Copyright © 2016 The Author(s). Expression of the pregnane X receptor (PXR) has been reported to be decreased in animal models of inflammatory bowel disease (IBD). To investigate the differential expression of PXR in children with Crohn's disease, a type of IBD, RNA was extracted from archived intestinal biopsies from 18 children with Crohn's disease (CD) and 12 age- and sex-matched controls (aged 7-17yrs). The aim of this investigation was to compare the relative mRNA expression of PXR, cytochrome p450 3A4 (CYP3A4), and villin 1 (VIL1) (a marker of epithelial cell integrity) in the inflamed terminal ileum (TI) versus noninflamed …


Validation Of Noninvasive Measures Of Left Ventricular Mechanics In Children: A Simultaneous Echocardiographic And Conductance Catheterization Study., Shahryar M. Chowdhury, Ryan J. Butts, Carolyn L. Taylor, Varsha M. Bandisode, Karen S. Chessa, Anthony M. Hlavacek, Girish S. Shirali, G Hamilton Baker Jul 2016

Validation Of Noninvasive Measures Of Left Ventricular Mechanics In Children: A Simultaneous Echocardiographic And Conductance Catheterization Study., Shahryar M. Chowdhury, Ryan J. Butts, Carolyn L. Taylor, Varsha M. Bandisode, Karen S. Chessa, Anthony M. Hlavacek, Girish S. Shirali, G Hamilton Baker

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The accuracy of echocardiography in evaluating left ventricular contractility has not been validated in children. The objective of this study was to compare echocardiographic measures of contractility with those derived from pressure-volume loop (PVL) analysis in children.

METHODS: Patients with relatively normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. The gold-standard measure of contractility, end-systolic elastance (Ees), was obtained via balloon occlusion of one or both vena cavae. Echocardiograms were performed immediately after PVL analysis under the same anesthetic conditions. Single-beat estimations of echocardiographic Ees were calculated using four different …


Asymptomatic Left Ventricular Myxoma In A 12-Year-Old Male., Meghan Chlebowski, James O'Brien, Casey Hertzenberg, Jonathan B. Wagner Jun 2016

Asymptomatic Left Ventricular Myxoma In A 12-Year-Old Male., Meghan Chlebowski, James O'Brien, Casey Hertzenberg, Jonathan B. Wagner

Manuscripts, Articles, Book Chapters and Other Papers

Cardiac myxoma is the most common cardiac tumor in patients of all ages; the majority are encountered as single left atrial tumors. Left ventricular myxomas are exceedingly rare, having been recorded in a small number of case reports involving children worldwide. We report a case of a left ventricular myxoma with left ventricular outflow tract obstruction in a previously healthy, asymptomatic adolescent black male. Transthoracic echocardiograms revealed a single, large (2.5 × 5-cm), lobulated, mobile mass within the left ventricular cavity that oscillated into the outflow tract, thereby causing moderate obstruction during systole. Advanced images delineated the location and tissue …


Harnessing Teams And Technology To Improve Outcomes In Infants With Single Ventricle., Girish S. Shirali, Lori A. Erickson, Johnathan Apperson, Kathy Goggin, David D. Williams, Kimberly J. Reid, Andrea Bradley-Ewing, Dawn Tucker, Michael Bingler, John Spertus, Leslie Rabbitt, Richard Stroup May 2016

Harnessing Teams And Technology To Improve Outcomes In Infants With Single Ventricle., Girish S. Shirali, Lori A. Erickson, Johnathan Apperson, Kathy Goggin, David D. Williams, Kimberly J. Reid, Andrea Bradley-Ewing, Dawn Tucker, Michael Bingler, John Spertus, Leslie Rabbitt, Richard Stroup

Manuscripts, Articles, Book Chapters and Other Papers

Infants with single ventricle require staged cardiac surgery, with stage I typically performed shortly after birth, stage II at 4 to 6 months of age, and stage III at 3 to 5 years of age. There is a high risk of interstage mortality and morbidity after infants are discharged from the hospital between stages I and II. Traditional home monitoring requires caregivers to record measurements of weight and oxygen saturation into a binder and requires families to assume a surveillance role. We have developed a tablet PC-based solution that provides secure and nearly instantaneous transfer of patient information to a …


Medical Therapy For Inappropriate Sexual Behaviors In A Teen With Autism Spectrum Disorder., Loyal Coshway, Julia Broussard, Kruti Acharya, Karen Fried, Michael E. Msall, John Lantos, Leena Nahata Apr 2016

Medical Therapy For Inappropriate Sexual Behaviors In A Teen With Autism Spectrum Disorder., Loyal Coshway, Julia Broussard, Kruti Acharya, Karen Fried, Michael E. Msall, John Lantos, Leena Nahata

Manuscripts, Articles, Book Chapters and Other Papers

Teens with autism spectrum disorder often exhibit sexual behaviors in public that are disturbing to parents, teachers, and peers. Some have proposed that such behaviors can be curtailed with hormonal suppression. There is information on the Internet suggesting that such medications work, and some reports in the peer-reviewed medical literature support these claims. Such medications can have serious side effects. In this paper, we present a case in which parents requested such treatment of their teenage son with autism spectrum disorder.


Considering Decision-Making And Sexuality In Menstrual Suppression Of Teens And Young Adults With Intellectual Disabilities., Kruti Acharya, John D. Lantos Apr 2016

Considering Decision-Making And Sexuality In Menstrual Suppression Of Teens And Young Adults With Intellectual Disabilities., Kruti Acharya, John D. Lantos

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.


Chondroinduction From Naturally Derived Cartilage Matrix: A Comparison Between Devitalized And Decellularized Cartilage Encapsulated In Hydrogel Pastes., Emily C. Beck, Marilyn Barragan, Tony B. Libeer, Sarah L. Kieweg, Gabriel L. Converse, Richard A. Hopkins, Cory J. Berkland, Michael S. Detamore Apr 2016

Chondroinduction From Naturally Derived Cartilage Matrix: A Comparison Between Devitalized And Decellularized Cartilage Encapsulated In Hydrogel Pastes., Emily C. Beck, Marilyn Barragan, Tony B. Libeer, Sarah L. Kieweg, Gabriel L. Converse, Richard A. Hopkins, Cory J. Berkland, Michael S. Detamore

Manuscripts, Articles, Book Chapters and Other Papers

Hydrogel precursors are liquid solutions that are prone to leaking after surgical placement. This problem was overcome by incorporating either decellularized cartilage (DCC) or devitalized cartilage (DVC) microparticles into traditional photocrosslinkable hydrogel precursors in an effort to achieve a paste-like hydrogel precursor. DCC and DVC were selected specifically for their potential to induce chondrogenesis of stem cells, given that materials that are chondroinductive on their own without growth factors are a revolutionary goal in orthopedic medicine. We hypothesized that DVC, lacking the additional chemical processing steps in DCC to remove cell content, would lead to a more chondroinductive hydrogel with …


Urgent Care And Emergency Department Visits In The Pediatric Medicaid Population., Amanda Montalbano, Jonathan Rodean, Juhi Kangas, Brian R. Lee, Matt Hall Apr 2016

Urgent Care And Emergency Department Visits In The Pediatric Medicaid Population., Amanda Montalbano, Jonathan Rodean, Juhi Kangas, Brian R. Lee, Matt Hall

Manuscripts, Articles, Book Chapters and Other Papers

Background: Urgent care (UC) is one of the fastest growing venues of health care delivery. We compared clinical and cost attributes of pediatric UC and emergency department (ED) visits that did not result in admission.

Methods: Our study examined 5 925 568 ED and UC visits of children under 19 years old in the 2010 through 2012 Marketscan Medicaid Multi-State Database. Basic demographics, diagnoses, severity, and payments were compared. Between ED and UC visits, χ(2) tests were used for proportions and Wilcoxon rank-sum tests were used for continuous variables.

Results: The UC and ED had the same most common diagnoses. …


The Genomic Cds Sandbox: An Assessment Among Domain Experts., Ayesha Aziz, Kensaku Kawamoto, Karen Eilbeck, Marc S Williams, Robert R Freimuth, Mark A Hoffman, Luke V Rasmussen, Casey L Overby, Brian H Shirts, James M Hoffman, Brandon M Welch Apr 2016

The Genomic Cds Sandbox: An Assessment Among Domain Experts., Ayesha Aziz, Kensaku Kawamoto, Karen Eilbeck, Marc S Williams, Robert R Freimuth, Mark A Hoffman, Luke V Rasmussen, Casey L Overby, Brian H Shirts, James M Hoffman, Brandon M Welch

Manuscripts, Articles, Book Chapters and Other Papers

Genomics is a promising tool that is becoming more widely available to improve the care and treatment of individuals. While there is much assertion, genomics will most certainly require the use of clinical decision support (CDS) to be fully realized in the routine clinical setting. The National Human Genome Research Institute (NHGRI) of the National Institutes of Health recently convened an in-person, multi-day meeting on this topic. It was widely recognized that there is a need to promote the innovation and development of resources for genomic CDS such as a CDS sandbox. The purpose of this study was to evaluate …


Reliability And Validity Of An Observational Measure Of Client Decision-Making: The Client Language Assessment - Proximal/Distal (Cla-Pd)., Molly Magill, Timothy R. Apodaca, Mitchell Karno, Jacques Gaume, Ayla Durst, Justin Walthers, Robert L. Stout, Carlo Diclemente Apr 2016

Reliability And Validity Of An Observational Measure Of Client Decision-Making: The Client Language Assessment - Proximal/Distal (Cla-Pd)., Molly Magill, Timothy R. Apodaca, Mitchell Karno, Jacques Gaume, Ayla Durst, Justin Walthers, Robert L. Stout, Carlo Diclemente

Manuscripts, Articles, Book Chapters and Other Papers

OVERVIEW: The Client Language Assessment - Proximal/Distal (CLA-PD) is a language rating system for measuring client decision-making in interventions that target a specified behavior change (e.g., alcohol or other drug use). In the CLA-PD, there are five dimensions of change language (Reason, Ability, Commitment, Taking Steps, Other) adapted from the client portion of the Motivational Interviewing Skill Code (MISC). For the CLA-PD, language codes are sub-divided to discriminate statements regarding the primary, or target behavior change (distal change) from the intermediate coping activities (proximal change) that are prescribed to facilitate that target behavior change. The goal of the CLA-PD is …


Rebound Growth Of Infantile Hemangiomas After Propranolol Therapy., Sonal D. Shah, Eulalia Baselga, Catherine Mccuaig, Elena Pope, Julien Coulie, Laurence M. Boon, Maria C. Garzon, Anita N. Haggstrom, Denise Adams, Beth A. Drolet, Brandon D. Newell, Julie Powell, Maria Teresa García-Romero, Carol Chute, Esther Roe, Dawn H. Siegel, Barbara Grimes, Ilona J. Frieden Apr 2016

Rebound Growth Of Infantile Hemangiomas After Propranolol Therapy., Sonal D. Shah, Eulalia Baselga, Catherine Mccuaig, Elena Pope, Julien Coulie, Laurence M. Boon, Maria C. Garzon, Anita N. Haggstrom, Denise Adams, Beth A. Drolet, Brandon D. Newell, Julie Powell, Maria Teresa García-Romero, Carol Chute, Esther Roe, Dawn H. Siegel, Barbara Grimes, Ilona J. Frieden

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND AND OBJECTIVES: Propranolol is first-line therapy for problematic infantile hemangiomas (IHs). Rebound growth after propranolol discontinuation is noted in 19% to 25% of patients. Predictive factors for rebound are not completely understood and may alter the management approach. The goal of the study was to describe a cohort of patients with IHs treated with propranolol and to identify predictors for rebound growth.

METHODS: A multicenter retrospective cohort study was conducted in patients with IHs treated with propranolol. Patient demographic characteristics, IH characteristics, and specifics of propranolol therapy were obtained. Episodes of rebound growth were recorded. Patients' responses to propranolol …