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Superelastic And Ph-Responsive Degradable Dendrimer Cryogels Prepared By Cryo-Aza-Michael Addition Reaction, Juan Wang, Hu Yang Dec 2018

Superelastic And Ph-Responsive Degradable Dendrimer Cryogels Prepared By Cryo-Aza-Michael Addition Reaction, Juan Wang, Hu Yang

Chemical and Biochemical Engineering Faculty Research & Creative Works

Dendrimers exhibit super atomistic features by virtue of their well-defined discrete quantized nanoscale structures. Here, we show that hyperbranched amine-terminated polyamidoamine (PAMAM) dendrimer G4.0 reacts with linear polyethylene glycol (PEG) diacrylate (575 g/mol) via the aza-Michael addition reaction at a subzero temperature (-20 °C), namely cryo-aza-Michael addition, to form a macroporous superelastic network, i.e., dendrimer cryogel. Dendrimer cryogels exhibit biologically relevant Young's modulus, high compression elasticity and super resilience at ambient temperature. Furthermore, the dendrimer cryogels exhibit excellent rebound performance and do not show significant stress relaxation under cyclic deformation over a wide temperature range (-80 to 100 °C). The …


A Multicenter Study To Evaluate Pulmonary Function In Osteogenesis Imperfecta., Allison Tam, Shan Chen, Evan Schauer, Ingo Grafe, Venkata Bandi, Jay R. Shapiro, Robert D. Steiner, Peter A. Smith, Michael B. Bober, Tracy Hart, David Cuthbertson, Jeffrey Krischer, Mary Mullins, Peter H. Byers, Robert A. Sandhaus, Michaela Durigova, Francis H. Glorieux, Frank Rauch, Vernon Reid Sutton, Brendan Lee, Members Of The Brittle Bone Disorders Consortium, Eric T. Rush, Sandesh C S Nagamani Dec 2018

A Multicenter Study To Evaluate Pulmonary Function In Osteogenesis Imperfecta., Allison Tam, Shan Chen, Evan Schauer, Ingo Grafe, Venkata Bandi, Jay R. Shapiro, Robert D. Steiner, Peter A. Smith, Michael B. Bober, Tracy Hart, David Cuthbertson, Jeffrey Krischer, Mary Mullins, Peter H. Byers, Robert A. Sandhaus, Michaela Durigova, Francis H. Glorieux, Frank Rauch, Vernon Reid Sutton, Brendan Lee, Members Of The Brittle Bone Disorders Consortium, Eric T. Rush, Sandesh C S Nagamani

Manuscripts, Articles, Book Chapters and Other Papers

Pulmonary complications are a significant cause for morbidity and mortality in osteogenesis imperfecta (OI). However, to date, there have been few studies that have systematically evaluated pulmonary function in individuals with OI. We analyzed spirometry measurements, including forced vital capacity (FVC) and forced expiratory volume in the first second (FEV1 ), in a large cohort of individuals with OI (n = 217) enrolled in a multicenter, observational study. We show that individuals with the more severe form of the disease, OI type III, have significantly reduced FVC and FEV1 which do not follow the expected trends of the …


Types Of Tobacco Consumption And The Oral Microbiome In The United Arab Emirates Healthy Future (Uaehfs) Pilot Study, Yvonne Vallès, Claire K. Inman, Brandilyn A. Peters, Raghib Ali, Laila Abdel Wareth, Abdishakur Abdulle, Habiba Alsafar, Fatme Al Anouti, Ayesha Al Dhaheri, Divya Galani, Muna Haji, Aisha Al Hamiz, Ayesha Al Hosani, Mohammed Al Houqani, Abdulla Al Junaibi, Marina Kazim, Tomas Kirchhoff, Wael Al Mahmeed, Fatma Al Maskari, Abdullah Alnaeemi, Naima Oumeziane, Ravichandran Ramasamy, Ann Marie Schmidt, Michael Weitzman, Eiman Al Zaabi, Scott Sherman, Richard B. Hayes, Jiyoung Ahn Dec 2018

Types Of Tobacco Consumption And The Oral Microbiome In The United Arab Emirates Healthy Future (Uaehfs) Pilot Study, Yvonne Vallès, Claire K. Inman, Brandilyn A. Peters, Raghib Ali, Laila Abdel Wareth, Abdishakur Abdulle, Habiba Alsafar, Fatme Al Anouti, Ayesha Al Dhaheri, Divya Galani, Muna Haji, Aisha Al Hamiz, Ayesha Al Hosani, Mohammed Al Houqani, Abdulla Al Junaibi, Marina Kazim, Tomas Kirchhoff, Wael Al Mahmeed, Fatma Al Maskari, Abdullah Alnaeemi, Naima Oumeziane, Ravichandran Ramasamy, Ann Marie Schmidt, Michael Weitzman, Eiman Al Zaabi, Scott Sherman, Richard B. Hayes, Jiyoung Ahn

All Works

© 2018, The Author(s). Cigarette smoking alters the oral microbiome; however, the effect of alternative tobacco products remains unclear. Middle Eastern tobacco products like dokha and shisha, are becoming globally widespread. We tested for the first time in a Middle Eastern population the hypothesis that different tobacco products impact the oral microbiome. The oral microbiome of 330 subjects from the United Arab Emirates Healthy Future Study was assessed by amplifying the bacterial 16S rRNA gene from mouthwash samples. Tobacco consumption was assessed using a structured questionnaire and further validated by urine cotinine levels. Oral microbiome overall structure and specific taxon …


Pediatric Nasogastric Tube Placement And Verification: Best Practice Recommendations From The Novel Project., Sharon Y. Irving, Gina Rempel, Beth Lyman, Wednesday Marie A Sevilla, Ladonna Northington, Peggi Guenter, American Society For Parenteral And Enteral Nutrition Dec 2018

Pediatric Nasogastric Tube Placement And Verification: Best Practice Recommendations From The Novel Project., Sharon Y. Irving, Gina Rempel, Beth Lyman, Wednesday Marie A Sevilla, Ladonna Northington, Peggi Guenter, American Society For Parenteral And Enteral Nutrition

Manuscripts, Articles, Book Chapters and Other Papers

The placement of a nasogastric tube (NGT) in a pediatric patient is a common practice that is generally perceived as a benign bedside procedure. There is potential risk for NGT misplacement with each insertion. A misplaced NGT compromises patient safety, increasing the risk for serious and even fatal complications. There is no standardized method for verification of the initial NGT placement or reverification assessment of NGT location prior to use. Measurement of the acidity or pH of the gastric aspirate is the most frequently used evidence-based method to verify NGT placement. The radiograph, when properly obtained and interpreted, is considered …


Risk Stratification Of Febrile Infants ≤60 Days Old Without Routine Lumbar Puncture., Paul L. Aronson, Marie E. Wang, Eugene D. Shapiro, Samir S. Shah, Adrienne G. Deporre, Russell J Mcculloh, Christopher M. Pruitt, Sanyukta Desai, Lise E. Nigrovic, Richard D. Marble, Rianna C. Leazer, Sahar N. Rooholamini, Laura F. Sartori, Fran Balamuth, Christopher Woll, Mark I. Neuman, Febrile Young Infant Research Collaborative Dec 2018

Risk Stratification Of Febrile Infants ≤60 Days Old Without Routine Lumbar Puncture., Paul L. Aronson, Marie E. Wang, Eugene D. Shapiro, Samir S. Shah, Adrienne G. Deporre, Russell J Mcculloh, Christopher M. Pruitt, Sanyukta Desai, Lise E. Nigrovic, Richard D. Marble, Rianna C. Leazer, Sahar N. Rooholamini, Laura F. Sartori, Fran Balamuth, Christopher Woll, Mark I. Neuman, Febrile Young Infant Research Collaborative

Manuscripts, Articles, Book Chapters and Other Papers

Video Abstract: media-1vid110.1542/5840460609001PEDS-VA_2018-1879

OBJECTIVES: To evaluate the Rochester and modified Philadelphia criteria for the risk stratification of febrile infants with invasive bacterial infection (IBI) who do not appear ill without routine cerebrospinal fluid (CSF) testing.

METHODS: We performed a case-control study of febrile infants ≤60 days old presenting to 1 of 9 emergency departments from 2011 to 2016. For each infant with IBI (defined as a blood [bacteremia] and/or CSF [bacterial meningitis] culture with growth of a pathogen), controls without IBI were matched by site and date of visit. Infants were excluded if they appeared ill or had a …


A Prospective Adaptive Utility Trial To Validate Performance Of A Novel Urine Exosome Gene Expression Assay To Predict High-Grade Prostate Cancer In Patients With Prostate-Specific Antigen 2-10ng/Ml At Initial Biopsy, James Mckiernan, Michael J Donovan, Eric Margolis, Alan Partin, Ballentine Carter, Gordon Brown, Phillipp Torkler, Mikkel Noerholm, Johan Skog, Neal Shore, Gerry Andriole, Ian Thompson, Peter Carroll Dec 2018

A Prospective Adaptive Utility Trial To Validate Performance Of A Novel Urine Exosome Gene Expression Assay To Predict High-Grade Prostate Cancer In Patients With Prostate-Specific Antigen 2-10ng/Ml At Initial Biopsy, James Mckiernan, Michael J Donovan, Eric Margolis, Alan Partin, Ballentine Carter, Gordon Brown, Phillipp Torkler, Mikkel Noerholm, Johan Skog, Neal Shore, Gerry Andriole, Ian Thompson, Peter Carroll

Open Access Publications

BACKGROUND: Discriminating indolent from clinically significant prostate cancer (PCa) in the initial biopsy setting remains an important issue. Prospectively evaluated diagnostic assays are necessary to ensure efficacy and clinical adoption.

OBJECTIVE: Performance and utility assessment of ExoDx Prostate (IntelliScore) (EPI) urine exosome gene expression assay versus standard clinical parameters for discriminating Grade Group (GG) ≥2 PCa from GG1 PCa and benign disease on initial biopsy.

DESIGN, SETTING, AND PARTICIPANTS: A two-phase adaptive clinical utility study (NCT03031418) comparing EPI results with biopsy outcomes in men, with age ≥50 yr and prostate-specific antigen (PSA) 2-10ng/ml, scheduled for initial prostate biopsy. After EPI …


New Advances In Amblyopia Therapy Ii: Refractive Therapies, Courtney L Kraus, Susan M Culican Dec 2018

New Advances In Amblyopia Therapy Ii: Refractive Therapies, Courtney L Kraus, Susan M Culican

Open Access Publications

The treatment of anisometropic or ametropic amblyopia has traditionally enjoyed a high treatment success rate. Early initiation and consistent use of spectacle correction can completely resolve amblyopia in a majority of patients. For those with anisometropic amblyopia that fail to improve with glasses wear alone, patching or atropine penalisation can lead to equalisation of visual acuity. However, successful treatment requires full-time compliance with refractive correction and this can be a challenge for a patient population that often has one eye with good acuity without correction. Other barriers for a select population with high anisometropic or ametropic amblyopia include rejection of …


The Jefferson Scale Of Empathy: A Nationwide Study Of Measurement Properties, Underlying Components, Latent Variable Structure, And National Norms In Medical Students., Mohammadreza Hojat, Jennifer Desantis, Stephen C. Shannon, Luke H. Mortensen, Mark R. Speicher, Lynn Bragan, Marianna Lanoue, Leonard H. Calabrese Dec 2018

The Jefferson Scale Of Empathy: A Nationwide Study Of Measurement Properties, Underlying Components, Latent Variable Structure, And National Norms In Medical Students., Mohammadreza Hojat, Jennifer Desantis, Stephen C. Shannon, Luke H. Mortensen, Mark R. Speicher, Lynn Bragan, Marianna Lanoue, Leonard H. Calabrese

College of Population Health Faculty Papers

The Jefferson Scale of Empathy (JSE) is a broadly used instrument developed to measure empathy in the context of health professions education and patient care. Evidence in support of psychometrics of the JSE has been reported in health professions students and practitioners with the exception of osteopathic medical students. This study was designed to examine measurement properties, underlying components, and latent variable structure of the JSE in a nationwide sample of first-year matriculants at U.S. colleges of osteopathic medicine, and to develop a national norm table for the assessment of JSE scores. A web-based survey was administered at the beginning …


The Dermatologist And Color, Andrzej Grzybowski, Lawrence Charles Parish Dec 2018

The Dermatologist And Color, Andrzej Grzybowski, Lawrence Charles Parish

Department of Dermatology and Cutaneous Biology Faculty Papers

The idea for an issue on color and the skin was initiated by the 2017 exhibit “Breathing Color” that had been created by the new Design Museum in London, England. While variations in color may play a significant role in diagnosing a skin disease, little attention has been directed towards the influence that color may be used to identify a dermatologic disease and even monitor a therapeutic agent. The reader need only to recall how early dermatology atlases were hand colored to provide a more realistic picture of a disease.


Drug-Coated Balloon Treatment Of Femoropopliteal Lesions Typically Excluded From Clinical Trials: 12-Month Findings From The In.Pact Global Study., Gary M Ansel, Marianne Brodmann, Koen Keirse, Antonio Micari, Michael R Jaff, Krishna Rocha-Singh, Eric J Fernandez, Hong Wang, Thomas Zeller, In.Pact Global Study Investigators Dec 2018

Drug-Coated Balloon Treatment Of Femoropopliteal Lesions Typically Excluded From Clinical Trials: 12-Month Findings From The In.Pact Global Study., Gary M Ansel, Marianne Brodmann, Koen Keirse, Antonio Micari, Michael R Jaff, Krishna Rocha-Singh, Eric J Fernandez, Hong Wang, Thomas Zeller, In.Pact Global Study Investigators

Heart and Vascular Articles

PURPOSE: To report a post hoc analysis comparing outcomes between subjects who would have been included in the IN.PACT SFA randomized controlled trial vs those who would have been excluded.

METHODS: The 1406 subjects enrolled in the IN.PACT Global Study ( ClinicalTrials.gov identifier NCT01609296) were retrospectively assigned to a standard-use group (n=281) based on the inclusion and exclusion criteria from the randomized IN.PACT SFA trial; the remaining 1125 patients were assigned to the broader-use group. Freedom from clinically-driven target lesion revascularization (CD-TLR) was evaluated at 12 months. The composite primary safety endpoint was freedom from 30-day device- and procedure-related death …


Development And Validation Of A Simple Convenience Store Shelf Audit., Tanya M. Horacek, Elif Dede Yildirim, Erin Kelly, Adrienne A. White, Karla P. Shelnutt, Kristin Riggsbee, Melissa D. Olfert, Jesse Morrell, Anne E. Matthews, Terezie T Mosby, Tandalayo Kidd, Kendra Kattelmann, Geoffrey Greene, Lisa Franzen-Castle, Sarah E. Colby, Carol Byrd-Bredbenner, Onikia Brown Nov 2018

Development And Validation Of A Simple Convenience Store Shelf Audit., Tanya M. Horacek, Elif Dede Yildirim, Erin Kelly, Adrienne A. White, Karla P. Shelnutt, Kristin Riggsbee, Melissa D. Olfert, Jesse Morrell, Anne E. Matthews, Terezie T Mosby, Tandalayo Kidd, Kendra Kattelmann, Geoffrey Greene, Lisa Franzen-Castle, Sarah E. Colby, Carol Byrd-Bredbenner, Onikia Brown

Health and Nutritional Sciences Faculty Publications

ackground This paper describes the development, reliability, and convergent validity of a practical tool-the Convenience StoreSupportive Healthy Environment for Life-Promoting Food (SHELF) Audit. Methods Audit items included: a variety of fresh, processed, and frozen fruits and vegetables; low-fat dairy products; healthy staples and frozen meals; healthy food incentive programs; items sold in check-out areas; portion/cup sizes; and pricing. Each audit item was scored using a five-point semantic-differential scale (1 = provides little or no support for healthful foods to 5 = provides high support for healthful foods). Convergent validity was examined by comparing the SHELF audit to Ghirardelli et al. …


Rare Presentation Of An Atrial Myxoma In An Adolescent Patient: A Case Report And Literature Review, Eduardo Macias, Elizabeth Nieman, Kentaro Yomogida, Orlando Petrucci, Cylen Javidan, Kevin Baszis, Shafkat Anwar Nov 2018

Rare Presentation Of An Atrial Myxoma In An Adolescent Patient: A Case Report And Literature Review, Eduardo Macias, Elizabeth Nieman, Kentaro Yomogida, Orlando Petrucci, Cylen Javidan, Kevin Baszis, Shafkat Anwar

Open Access Publications

BACKGROUND: Cardiac tumors are uncommon in the pediatric population. When present, cardiac manifestations stem from the tumor causing inflow or outflow obstruction. While common in adults, cardiac myxomas presenting with generalized systemic illness or peripheral emboli especially with no cardiac or neurological symptoms are rare in children.

CASE PRESENTATION: We report a case of a previously healthy adolescent girl who presented with a 6-month history of constitutional symptoms and a purpuric rash with no cardiac or neurologic symptoms, found to have a cardiac myxoma.

CONCLUSIONS: A vasculopathic rash in the setting of atrial myxomas has been shown be a precursor …


Macrophage-Derived Netrin-1 Promotes Abdominal Aortic Aneurysm Formation By Activating Mmp3 In Vascular Smooth Muscle Cells, Tarik Hadi, Ludovic Boytard, Michele Silvestro, Dornazsadat Alebrahim, Samson Jacob, Jordyn Feinstein, Krista Barone, Wes Spiro, Susan Hutchison, Russell Simon, Debra L. Rateri, Florence Pinet, David Fenyo, Mark Adelman, Kathryn J. Moore, Holger K. Eltzschig, Alan Daugherty, Bhama Ramkhelawon Nov 2018

Macrophage-Derived Netrin-1 Promotes Abdominal Aortic Aneurysm Formation By Activating Mmp3 In Vascular Smooth Muscle Cells, Tarik Hadi, Ludovic Boytard, Michele Silvestro, Dornazsadat Alebrahim, Samson Jacob, Jordyn Feinstein, Krista Barone, Wes Spiro, Susan Hutchison, Russell Simon, Debra L. Rateri, Florence Pinet, David Fenyo, Mark Adelman, Kathryn J. Moore, Holger K. Eltzschig, Alan Daugherty, Bhama Ramkhelawon

Physiology Faculty Publications

Abdominal aortic aneurysms (AAA) are characterized by extensive extracellular matrix (ECM) fragmentation and inflammation. However, the mechanisms by which these events are coupled thereby fueling focal vascular damage are undefined. Here we report through single-cell RNA-sequencing of diseased aorta that the neuronal guidance cue netrin-1 can act at the interface of macrophage-driven injury and ECM degradation. Netrin-1 expression peaks in human and murine aneurysmal macrophages. Targeted deletion of netrin-1 in macrophages protects mice from developing AAA. Through its receptor neogenin-1, netrin-1 induces a robust intracellular calcium flux necessary for the transcriptional regulation and persistent catalytic activation of matrix metalloproteinase-3 (MMP3) …


Distinct White Matter Changes Associated With Cerebrospinal Fluid Amyloid-Β1-42 And Hypertension, Omar M. Al-Janabi, Christopher A. Brown, Ahmed A. Bahrani, Erin L. Abner, Justin M. Barber, Brian T. Gold, Larry B. Goldstein, Richard R. Murphy, Peter T. Nelson, Nathan F. Johnson, Leslie M. Shaw, Charles D. Smith, John Q. Trojanowski, Donna M. Wilcock, Gregory A. Jicha Nov 2018

Distinct White Matter Changes Associated With Cerebrospinal Fluid Amyloid-Β1-42 And Hypertension, Omar M. Al-Janabi, Christopher A. Brown, Ahmed A. Bahrani, Erin L. Abner, Justin M. Barber, Brian T. Gold, Larry B. Goldstein, Richard R. Murphy, Peter T. Nelson, Nathan F. Johnson, Leslie M. Shaw, Charles D. Smith, John Q. Trojanowski, Donna M. Wilcock, Gregory A. Jicha

Sanders-Brown Center on Aging Faculty Publications

BACKGROUND: Alzheimer's disease (AD) pathology and hypertension (HTN) are risk factors for development of white matter (WM) alterations and might be independently associated with these alterations in older adults.

OBJECTIVE: To evaluate the independent and synergistic effects of HTN and AD pathology on WM alterations.

METHODS: Clinical measures of cerebrovascular disease risk were collected from 62 participants in University of Kentucky Alzheimer's Disease Center studies who also had cerebrospinal fluid (CSF) sampling and MRI brain scans. CSF Aβ1-42 levels were measured as a marker of AD, and fluid-attenuated inversion recovery imaging and diffusion tensor imaging were obtained to assess …


Multicenter Trial Of A Combination Probiotic For Children With Gastroenteritis., Stephen B Freedman, Sarah Williamson-Urquhart, Ken J Farion, Serge Gouin, Andrew R Willan, Naveen Poonai, Katrina Hurley, Philip M Sherman, Yaron Finkelstein, Bonita E Lee, Xiao-Li Pang, Linda Chui, David Schnadower, Jianling Xie, Marc Gorelick, Suzanne Schuh Nov 2018

Multicenter Trial Of A Combination Probiotic For Children With Gastroenteritis., Stephen B Freedman, Sarah Williamson-Urquhart, Ken J Farion, Serge Gouin, Andrew R Willan, Naveen Poonai, Katrina Hurley, Philip M Sherman, Yaron Finkelstein, Bonita E Lee, Xiao-Li Pang, Linda Chui, David Schnadower, Jianling Xie, Marc Gorelick, Suzanne Schuh

Paediatrics Publications

Background

Gastroenteritis accounts for approximately 1.7 million visits to the emergency department (ED) by children in the United States every year. Data to determine whether the use of probiotics improves outcomes in these children are lacking.

Methods

We conducted a randomized, double-blind trial involving 886 children 3 to 48 months of age with gastroenteritis who presented to six pediatric EDs in Canada. Participants received a 5-day course of a combination probiotic product containing Lactobacillus rhamnosus R0011 and L. helveticus R0052, at a dose of 4.0×10

Results

Moderate-to-severe gastroenteritis within 14 days after enrollment occurred in 108 of 414 participants (26.1%) …


Financial Incentives For Smoking Treatment: Protocol Of The Fiesta Trial And Fiesta Oral Microbiome Substudy, Katherine M French, Sasha Z Gonzalez, Scott E Sherman, Alissa R Link, Sadozai Zoe Malik, Chi-Hong Tseng, Saahil A Jumkhawala, Briesny Tejada, Andrew White, Joseph A Ladapo Nov 2018

Financial Incentives For Smoking Treatment: Protocol Of The Fiesta Trial And Fiesta Oral Microbiome Substudy, Katherine M French, Sasha Z Gonzalez, Scott E Sherman, Alissa R Link, Sadozai Zoe Malik, Chi-Hong Tseng, Saahil A Jumkhawala, Briesny Tejada, Andrew White, Joseph A Ladapo

NYMC Student Publications

BACKGROUND: Smoking is the leading preventable cause of death in the United States, but evidence-based smoking cessation therapy is underutilized. Financial incentive strategies represent an innovative approach for increasing the use of counseling and pharmacotherapy. If effective, they could supplement or supplant resource-intensive policy options, particularly in populations for whom smoking has substantial societal costs. FIESTA (Financial IncEntives for Smoking TreAtment) will randomize hospitalized smokers to receive usual smoking cessation care alone or usual smoking care augmented with financial incentives. We aim to compare the impact of these two strategies on 1) smoking abstinence, 2) use of counseling and nicotine …


Unilateral, 3d Arm Movement Kinematics Are Encoded In Ipsilateral Human Cortex, David T. Bundy, Nicholas Szrama, Mrinal Pahwa, Eric C. Leuthardt Nov 2018

Unilateral, 3d Arm Movement Kinematics Are Encoded In Ipsilateral Human Cortex, David T. Bundy, Nicholas Szrama, Mrinal Pahwa, Eric C. Leuthardt

Open Access Publications

There is increasing evidence that the hemisphere ipsilateral to a moving limb plays a role in planning and executing movements. However, the exact relationship between cortical activity and ipsilateral limb movements is uncertain. We sought to determine whether 3D arm movement kinematics (speed, velocity, and position) could be decoded from cortical signals recorded from the hemisphere ipsilateral to the moving limb. By having invasively monitored patients perform unilateral reaches with each arm, we also compared the encoding of contralateral and ipsilateral limb kinematics from a single cortical hemisphere. In four motor-intact human patients (three male, one female) implanted with electrocorticography …


Bafopathies' Dna Methylation Epi-Signatures Demonstrate Diagnostic Utility And Functional Continuum Of Coffin-Siris And Nicolaides-Baraitser Syndromes., Erfan Aref-Eshghi, Eric G Bend, Rebecca L Hood, Laila C Schenkel, Deanna Alexis Carere, Rana Chakrabarti, Sandesh C S Nagamani, Sau Wai Cheung, Philippe M Campeau, Chitra Prasad, Victoria Mok Siu, Lauren Brady, Mark A Tarnopolsky, David J Callen, A Micheil Innes, Susan M White, Wendy S Meschino, Andrew Y Shuen, Guillaume Paré, Dennis E Bulman, Peter J Ainsworth, Hanxin Lin, David I Rodenhiser, Raoul C Hennekam, Kym M Boycott, Charles E Schwartz, Bekim Sadikovic Nov 2018

Bafopathies' Dna Methylation Epi-Signatures Demonstrate Diagnostic Utility And Functional Continuum Of Coffin-Siris And Nicolaides-Baraitser Syndromes., Erfan Aref-Eshghi, Eric G Bend, Rebecca L Hood, Laila C Schenkel, Deanna Alexis Carere, Rana Chakrabarti, Sandesh C S Nagamani, Sau Wai Cheung, Philippe M Campeau, Chitra Prasad, Victoria Mok Siu, Lauren Brady, Mark A Tarnopolsky, David J Callen, A Micheil Innes, Susan M White, Wendy S Meschino, Andrew Y Shuen, Guillaume Paré, Dennis E Bulman, Peter J Ainsworth, Hanxin Lin, David I Rodenhiser, Raoul C Hennekam, Kym M Boycott, Charles E Schwartz, Bekim Sadikovic

Paediatrics Publications

Coffin-Siris and Nicolaides-Baraitser syndromes (CSS and NCBRS) are Mendelian disorders caused by mutations in subunits of the BAF chromatin remodeling complex. We report overlapping peripheral blood DNA methylation epi-signatures in individuals with various subtypes of CSS (ARID1B, SMARCB1, and SMARCA4) and NCBRS (SMARCA2). We demonstrate that the degree of similarity in the epi-signatures of some CSS subtypes and NCBRS can be greater than that within CSS, indicating a link in the functional basis of the two syndromes. We show that chromosome 6q25 microdeletion syndrome, harboring ARID1B deletions, exhibits a similar CSS/NCBRS methylation profile. Specificity of this epi-signature was confirmed across …


A Rare Regulatory Variant In The Mef2d Gene Affects Gene Regulation And Splicing And Is Associated With A Sle Sub-Phenotype In Swedish Cohorts, Fabiana H G Farias, Et Al Nov 2018

A Rare Regulatory Variant In The Mef2d Gene Affects Gene Regulation And Splicing And Is Associated With A Sle Sub-Phenotype In Swedish Cohorts, Fabiana H G Farias, Et Al

Open Access Publications

Systemic lupus erythematosus (SLE) is an autoimmune disorder with heterogeneous clinical presentation and complex etiology involving the interplay between genetic, epigenetic, environmental and hormonal factors. Many common SNPs identified by genome wide-association studies (GWAS) explain only a small part of the disease heritability suggesting the contribution from rare genetic variants, undetectable in GWAS, and complex epistatic interactions. Using targeted re-sequencing of coding and conserved regulatory regions within and around 215 candidate genes selected on the basis of their known role in autoimmunity and genes associated with canine immune-mediated diseases, we identified a rare regulatory variant rs200395694:G > T located in intron …


Micu1 Interacts With The D-Ring Of The Mcu Pore To Control Its Ca2+ Flux And Sensitivity To Ru360, Melanie Paillard, György Csordás, Kai-Ting Huang, Peter Várnai, Suresh K. Joseph, György Hajnóczky Nov 2018

Micu1 Interacts With The D-Ring Of The Mcu Pore To Control Its Ca2+ Flux And Sensitivity To Ru360, Melanie Paillard, György Csordás, Kai-Ting Huang, Peter Várnai, Suresh K. Joseph, György Hajnóczky

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Proper control of the mitochondrial Ca2+ uniporter’s pore (MCU) is required to allow Ca2+ dependent activation of oxidative metabolism and to avoid mitochondrial Ca2+ overload and cell death. The MCU’s gatekeeping and cooperative activation is mediated by the Ca2+ sensing MICU1 protein, which has been proposed to form dimeric complexes anchored to the EMRE scaffold of MCU. We unexpectedly find that MICU1 suppresses inhibition of MCU by ruthenium red/Ru360, which bind to MCU’s DIME motif, the selectivity filter. This led us to recognize in MICU1’s sequence, a putative DIME Interacting Domain (DID) which is required for …


The Fatal Failure Of The Regulatory State, W. Kip Viscusi Nov 2018

The Fatal Failure Of The Regulatory State, W. Kip Viscusi

William & Mary Law Review

Although regulatory agencies place high values on the benefits associated with the reduction in mortality risks due to regulations, these same agencies substantially undervalue lives in their enforcement efforts. The disparity between the valuation of prospective risks and fatalities that have occurred is often by several orders of magnitude, diminishing whatever safety incentives the regulations might have generated. A review of the practices by the major federal agencies with responsibility for product safety and occupational safety finds that the value placed on fatalities in agencies’ regulatory analyses can be a factor of 1000 times greater than the magnitude of the …


Dysregulated Nk Cell Plcγ2 Signaling And Activity In Juvenile Dermatomyositis, Allison A Throm, Joshua B Alinger, Jeanette T Pingel, Allyssa L Daugherty, Lauren M Pachman, Anthony R French Nov 2018

Dysregulated Nk Cell Plcγ2 Signaling And Activity In Juvenile Dermatomyositis, Allison A Throm, Joshua B Alinger, Jeanette T Pingel, Allyssa L Daugherty, Lauren M Pachman, Anthony R French

Open Access Publications

Juvenile dermatomyositis (JDM) is a debilitating pediatric autoimmune disease manifesting with characteristic rash and muscle weakness. To delineate signaling abnormalities in JDM, mass cytometry was performed with PBMCs from treatment-naive JDM patients and controls. NK cell percentages were lower while frequencies of naive B cells and naive CD4+ T cells were higher in JDM patients than in controls. These cell frequency differences were attenuated with cessation of active disease. A large number of signaling differences were identified in treatment-naive JDM patients compared with controls. Classification models incorporating feature selection demonstrated that differences in phospholipase Cγ2 (PLCγ2) phosphorylation comprised 10 of …


Functional Brain Activation Changes Associated With Practice In Delaying Smoking Among Moderate To Heavy Smokers: Study Protocol And Rationale Of A Randomized Trial (Cope)., Andrew T. Fox, Delwyn Catley, Kimber P. Richter, Edward F. Ellerbeck, Morgan G. Brucks, Vlad B. Papa, Laura E. Martin Nov 2018

Functional Brain Activation Changes Associated With Practice In Delaying Smoking Among Moderate To Heavy Smokers: Study Protocol And Rationale Of A Randomized Trial (Cope)., Andrew T. Fox, Delwyn Catley, Kimber P. Richter, Edward F. Ellerbeck, Morgan G. Brucks, Vlad B. Papa, Laura E. Martin

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Most smokers struggle to overcome tobacco addiction. Neuroscientific models of addiction emphasize the importance of brain regions associated with cognitive control and reward to understand the cycle of addiction and relapse. During an attempt at abstinence, the cognitive control system appears to be underpowered to override the heightened reward system of the addicted brain. Thus, one neural target for treatment is to strengthen the cognitive control system. It may be possible to improve the functioning of the cognitive control system via deliberate practice.

METHODS/DESIGN: This study will determine the effects of practicing delaying smoking on brain and behavioral measures …


One-Day Oral Polyethylene Glycol Based Cleanout Is Effective For Pre-Colonoscopy Preparation In Children., Ashwath S. Kumar, Brooke L. Beutler, Thomas M. Attard Nov 2018

One-Day Oral Polyethylene Glycol Based Cleanout Is Effective For Pre-Colonoscopy Preparation In Children., Ashwath S. Kumar, Brooke L. Beutler, Thomas M. Attard

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The adequacy of pre-procedure preparation is the principal determinant of the quality of colonoscopy in pediatric as in adult patients. There is a lack of consensus, among providers on a standard pre-procedure regimen. Professional society guidelines include the use of Polyethylene glycol (PEG). Herein we report on the provider-assessed adequacy of a one day, age-categorized dosing, PEG based cleanout regimen in children undergoing colonoscopy in a tertiary institution.

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METHODS: The standard bowel preparation regime at our institution includes an age dependent minimum PEG dosing regimen in addition to clear liquids the day prior to the procedure. We retrospectively …


Effects Of A Community Population Health Initiative On Blood Pressure Control In Latinos, James R Langabeer, Timothy D Henry, Carlos Perez Aldana, Larissa Deluna, Nora Silva, Tiffany Champagne-Langabeer Nov 2018

Effects Of A Community Population Health Initiative On Blood Pressure Control In Latinos, James R Langabeer, Timothy D Henry, Carlos Perez Aldana, Larissa Deluna, Nora Silva, Tiffany Champagne-Langabeer

Faculty, Staff and Student Publications

Background Hypertension remains one of the most important, modifiable cardiovascular risk factors. Yet, the largest minority ethnic group (Hispanics/Latinos) often have different health outcomes and behavior, making hypertension management more difficult. We explored the effects of an American Heart Association-sponsored population health intervention aimed at modifying behavior of Latinos living in Texas. Methods and Results We enrolled 8071 patients, and 5714 (65.7%) completed the 90-day program (58.5 years ±11.7; 59% female) from July 2016 to June 2018. Navigators identified patients with risk factors; initial and final blood pressure ( BP ) readings were performed in the physician's office; and interim …


Changes In The Us Burden Of Chronic Kidney Disease From 2002 To 2016: An Analysis Of The Global Burden Of Disease Study, Benjamin Bowe, Yan Xie, Tingting Li, Ali H. Mokdad, Hong Xian, Yan Yan, Geetha Maddukuri, Ziyad Al-Aly Nov 2018

Changes In The Us Burden Of Chronic Kidney Disease From 2002 To 2016: An Analysis Of The Global Burden Of Disease Study, Benjamin Bowe, Yan Xie, Tingting Li, Ali H. Mokdad, Hong Xian, Yan Yan, Geetha Maddukuri, Ziyad Al-Aly

Open Access Publications

Introduction: Over the past 15 years, changes in demographic, social, and epidemiologic trends occurred in the United States. These changes likely contributed to changes in chronic kidney disease (CKD) epidemiology.

Objective: To describe the change in burden of CKD at the US state level from 2002 to 2016.

Design, Setting, and Participants: This systematic analysis used data and methodologies from the 2016 Global Burden of Disease study in the United States. Data on CKD from 2002 to 2016 were examined at the state level.

Main Outcomes and Measures: Disability-adjusted life years (DALYs) and death due to CKD.

Results: In this …


Association Between Health Insurance Literacy And Avoidance Of Health Care Services Owing To Cost, Renuka Tipirneni, Mary C. Politi, Jeffrey T. Kullgren, Edith C. Kieffer, Susan D. Goold, Aaron M. Scherer Nov 2018

Association Between Health Insurance Literacy And Avoidance Of Health Care Services Owing To Cost, Renuka Tipirneni, Mary C. Politi, Jeffrey T. Kullgren, Edith C. Kieffer, Susan D. Goold, Aaron M. Scherer

Open Access Publications

Importance: Navigating health insurance and health care choices requires considerable health insurance literacy. Although recommended preventive services are exempt from out-of-pocket costs under the Affordable Care Act, many people may remain unaware of this provision and its effect on their required payment. Little is known about the association between individuals' health insurance literacy and their use of preventive or nonpreventive health care services.

Objective: To assess the association between health insurance literacy and self-reported avoidance of health care services owing to cost.

Design, Setting, and Participants: In this survey study, a US national, geographically diverse, nonprobability sample of 506 US …


Dietary Protein Restriction Reduces Circulating Vldl Triglyceride Levels Via Crebh-Apoa5-Dependent And -Independent Mechanisms, J Humberto Treviño-Villarreal, Valeria Tosti, Nicola Veronese, Beatrice Bertozzi, Arnold D Bullock, Robert S Figenshau, Gerald L Andriole, Luigi Fontana, Et Al Nov 2018

Dietary Protein Restriction Reduces Circulating Vldl Triglyceride Levels Via Crebh-Apoa5-Dependent And -Independent Mechanisms, J Humberto Treviño-Villarreal, Valeria Tosti, Nicola Veronese, Beatrice Bertozzi, Arnold D Bullock, Robert S Figenshau, Gerald L Andriole, Luigi Fontana, Et Al

Open Access Publications

Hypertriglyceridemia is an independent risk factor for cardiovascular disease. Dietary interventions based on protein restriction (PR) reduce circulating triglycerides (TGs), but underlying mechanisms and clinical relevance remain unclear. Here, we show that 1 week of a protein-free diet without enforced calorie restriction significantly lowered circulating TGs in both lean and diet-induced obese mice. Mechanistically, the TG-lowering effect of PR was due, in part, to changes in very low-density lipoprotein (VLDL) metabolism both in liver and peripheral tissues. In the periphery, PR stimulated VLDL-TG consumption by increasing VLDL-bound APOA5 expression and promoting VLDL-TG hydrolysis and clearance from circulation. The PR-mediated increase …


Effects Of Pain Neuroscience Education On Physician Assistant Students Understanding Of Pain And Attitudes And Beliefs About Pain., Kory Zimney, Adriaan Louw, Julie Johnson, Suzanne Peppers, Kevin Farrell Nov 2018

Effects Of Pain Neuroscience Education On Physician Assistant Students Understanding Of Pain And Attitudes And Beliefs About Pain., Kory Zimney, Adriaan Louw, Julie Johnson, Suzanne Peppers, Kevin Farrell

Faculty Publications

PURPOSE: Evaluate the effectiveness and efficiency of two different pain neuroscience education (PNE) lectures provided to physician assistant (PA) students. Primary outcomes explored were knowledge of pain and shift in attitudes and beliefs about chronic pain after the lecture.

METHODS: A PNE lecture was provided at two separate university PA programs. One program received a two-hour PNE lecture with a case-based example. The other program received a one-hour PNE lecture without the casebased example. Measurement of change for pre and post-test pain knowledge and attitudes and beliefs about chronic pain were recorded.

RESULTS: Students at both universities showed medium effect …


Caregiver Perspectives On Body-Weight Supported Treadmill Training As An Intervention For Young Children With Cerebral Palsy, Megan Flores, Carolyn Da Silva Nov 2018

Caregiver Perspectives On Body-Weight Supported Treadmill Training As An Intervention For Young Children With Cerebral Palsy, Megan Flores, Carolyn Da Silva

Physical Therapy Collection

PURPOSE/HYPOTHESIS: Children with cerebral palsy (CP) in Gross Motor Function Classification System (GMFCS) levels IV and V present with challenges that impact their functional activities and participation in family activities. The purpose of this study was to examine caregivers’ perspectives on body-weight supported treadmill training (BWSTT) as an intervention for their young children with CP.

NUMBER OF SUBJECTS: Participants included 4 caregivers of children with CP in GMFCS levels IV (n = 2) and V (n = 2) who previously participated in 6 weeks of BWSTT.

MATERIALS/METHODS: Three children diagnosed with CP in GMFCS levels IV and V between the …