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Articles 1 - 30 of 79
Full-Text Articles in Medical Specialties
The Ciliary Protein Cystin Forms A Regulatory Complex With Necdin To Modulate Myc Expression, Maoqing Wu, Chaozhe Yang, Binli Tao, Su Bu, Lisa Guay-Woodford
The Ciliary Protein Cystin Forms A Regulatory Complex With Necdin To Modulate Myc Expression, Maoqing Wu, Chaozhe Yang, Binli Tao, Su Bu, Lisa Guay-Woodford
Pediatrics Faculty Publications
Cystin is a novel cilia-associated protein that is disrupted in the cpk mouse, a well-characterized mouse model of autosomal recessive polycystic kidney disease (ARPKD). Interestingly, overexpression of the Myc gene is evident in animal models of ARPKD and is thought to contribute to the renal cystic phenotype. Using a yeast two-hybrid approach, the growth suppressor protein necdin, known to modulate Myc expression, was found as an interacting partner of cystin. Deletion mapping demonstrated that the C-terminus of cystin and both termini of necdin are required for their mutual interaction. Speculating that these two proteins may function to regulate gene expression, …
The Impact Of A Large-Scale Power Outage On Hemodialysis Center Operations, Mahshid Abir, Sophia Jan, Lindsay Jubelt, Raina M. Merchant, Nicole Lurie
The Impact Of A Large-Scale Power Outage On Hemodialysis Center Operations, Mahshid Abir, Sophia Jan, Lindsay Jubelt, Raina M. Merchant, Nicole Lurie
Emergency Medicine Faculty Publications
INTRODUCTION:
On June 29, 2012, mid-Atlantic storms resulted in a large-scale power outage affecting up to three million people across multiple (US) states. Hemodialysis centers are dependent on electricity to provide dialysis care to end-stage renal disease patients. The objective of thisstudy was to determine how the power outage impacted operations in a sample of hemodialysis centers in the impacted regions.
METHODS:
The sample consisted of all hemodialysis centers located in the District of Columbia and a total of five counties with the largest power losses in West Virginia, Virginia, and Maryland. A semi-structured interview guide was developed, and …
Clinical And Laboratory Features Distinguishing Juvenile Polymyositis And Muscular Dystrophy, Gulnara Mamyrova, James D. Katz, Robert V. Jones, Ira N. Targoff, Peter A. Lachenbruch, Olcay Y. Jones, Frederick W. Miller, Lisa G. Rider
Clinical And Laboratory Features Distinguishing Juvenile Polymyositis And Muscular Dystrophy, Gulnara Mamyrova, James D. Katz, Robert V. Jones, Ira N. Targoff, Peter A. Lachenbruch, Olcay Y. Jones, Frederick W. Miller, Lisa G. Rider
Pathology Faculty Publications
OBJECTIVE:
To differentiate juvenile polymyositis (PM) and muscular dystrophy, both of which may present with chronic muscle weakness and inflammation.
METHODS:
We studied 39 patients with probable or definite juvenile PM and 9 patients with muscular dystrophies who were initially misdiagnosed as having juvenile PM. Differences in demographic, clinical, and laboratory results; outcomes; and treatment responses were evaluated by Fisher's exact and rank sum tests. Random forests classification analysis and logistic regression were performed to examine significant differences in multivariable models.
RESULTS:
Clinical features and serum muscle enzyme levels were similar between juvenile PM and dystrophy patients, except 89% of …
Targeting Inflammation Using Salsalate In Patients With Type 2 Diabetes: Effects On Flow-Mediated Dilation (Tinsal-Fmd)., Allison B Goldfine, J Stewart Buck, Cyrus Desouza, Vivian Fonseca, Yii-Der Ida Chen, Steven E Shoelson, Kathleen A. Jablonski, Mark A Creager, The Tinsal-Fmd Team
Targeting Inflammation Using Salsalate In Patients With Type 2 Diabetes: Effects On Flow-Mediated Dilation (Tinsal-Fmd)., Allison B Goldfine, J Stewart Buck, Cyrus Desouza, Vivian Fonseca, Yii-Der Ida Chen, Steven E Shoelson, Kathleen A. Jablonski, Mark A Creager, The Tinsal-Fmd Team
GW Biostatistics Center
OBJECTIVE: To test whether inhibiting inflammation with salsalate improves endothelial function in patients with type 2 diabetes (T2D).
RESEARCH DESIGN AND METHODS: We conducted an ancillary study to the National Institutes of Health-sponsored, multicenter, randomized, double-masked, placebo-controlled trial evaluating the safety and efficacy of salsalate in targeting inflammation to improve glycemia in patients with T2D. Flow-mediated, endothelium-dependent dilation (FMD) and endothelium-independent, nitroglycerin-mediated dilation (NMD) of the brachial artery were assessed at baseline and 3 and 6 months following randomization to either salsalate 3.5 g/day or placebo. The primary end point was change in FMD at 6 months.
RESULTS: A total …
Global Mortality Estimates For The 2009 Influenza Pandemic From The Glamor Project: A Modeling Study, Lone Simonsen, Peter Spreeuwenberg, Roger Lustig, Robert J. Taylor, Douglas M. Fleming, Madelon Kroneman, Maria D. Van Kerkhove, Anthony D. Mounts, W. John Paget
Global Mortality Estimates For The 2009 Influenza Pandemic From The Glamor Project: A Modeling Study, Lone Simonsen, Peter Spreeuwenberg, Roger Lustig, Robert J. Taylor, Douglas M. Fleming, Madelon Kroneman, Maria D. Van Kerkhove, Anthony D. Mounts, W. John Paget
Global Health Faculty Publications
Background
Assessing the mortality impact of the 2009 influenza A H1N1 virus (H1N1pdm09) is essential for optimizing public health responses to future pandemics. The World Health Organization reported 18,631 laboratory-confirmed pandemic deaths, but the total pandemic mortality burden was substantially higher. We estimated the 2009 pandemic mortality burden through statistical modeling of mortality data from multiple countries.
Methods and Findings
We obtained weekly virology and underlying cause-of-death mortality time series for 2005–2009 for 20 countries covering ~35% of the world population. We applied a multivariate linear regression model to estimate pandemic respiratory mortality in each collaborating country. We then used …
Depression Is Associated With Repeat Emergency Department Visits In Patients With Non-Specific Abdominal Pain, Andrew C. Meltzer, Benjamin Bregman, Janice Blanchard
Depression Is Associated With Repeat Emergency Department Visits In Patients With Non-Specific Abdominal Pain, Andrew C. Meltzer, Benjamin Bregman, Janice Blanchard
Emergency Medicine Faculty Publications
Introduction: Patients with abdominal pain often return multiple times despite no definitive diagnosis. Our objective was to determine if repeat emergency department (ED) use among patients with non-specific abdominal pain might be associated with a diagnosis of moderate to severe depressive disorder.
Methods: We screened 987 ED patients for major depression during weekday daytime hours from June 2011 through November 2011 using a validated depression screening tool, the PHQ-9. Each subject was classified as either no depression, mild depression or moderate/ severe depression based on the screening tool. Within this group, we identified 83 patients with non-specific abdominal pain by …
Severe Painful Vaso-Occlusive Crises And Mortality In A Contemporary Adult Sickle Cell Anemia Cohort Study., Deepika S. Darbari, Zhengyuan Wang, Minjung Kwak, Mariana Hildesheim, James Nichols, Darlene Allen, Catherine Seamon, Marlene Peters-Lawrence, Anna Conrey, Mary K. Hall, Gregory J. Kato, James G. Taylor Vi
Severe Painful Vaso-Occlusive Crises And Mortality In A Contemporary Adult Sickle Cell Anemia Cohort Study., Deepika S. Darbari, Zhengyuan Wang, Minjung Kwak, Mariana Hildesheim, James Nichols, Darlene Allen, Catherine Seamon, Marlene Peters-Lawrence, Anna Conrey, Mary K. Hall, Gregory J. Kato, James G. Taylor Vi
Pediatrics Faculty Publications
BACKGROUND: Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort.
METHODS: Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations …
Two-Category Place Representations Persist Over Body Rotations, Hyoun Kyoung Pyoun, Jesse Sargent, Stephen Dopkins, John W. Philbeck
Two-Category Place Representations Persist Over Body Rotations, Hyoun Kyoung Pyoun, Jesse Sargent, Stephen Dopkins, John W. Philbeck
Neurological Surgery Faculty Publications
We explored a system that constructs environment-centered frames of reference and coordinates memory for the azimuth of an object in an enclosed space. For one group, we provided two environmental cues (doors): one in the front, and one in the rear. For a second group, we provided two object cues: a front and a rear cue.For a third group, we provided no external cues; we assumed that for this group, their reference frames would be determined by the orthogonal geometry of the floor-and-wall junction that divides a space in half or into multiple territories along the horizontal …
Fixed Drug Eruption Associated With Sulfonamides Sold In Latino Grocery Stores — Greater Washington, Dc, Area, 2012–2013, Christine Yang, Audrey Green, Scott A. Norton
Fixed Drug Eruption Associated With Sulfonamides Sold In Latino Grocery Stores — Greater Washington, Dc, Area, 2012–2013, Christine Yang, Audrey Green, Scott A. Norton
Dermatology Faculty Publications
No abstract provided.
A Profile Of Children With Complex Chronic Conditions At End Of Life Among Medicaid Beneficiaries: Implications For Health Care Reform, Lisa C. Lindley, Maureen E. Lyon
A Profile Of Children With Complex Chronic Conditions At End Of Life Among Medicaid Beneficiaries: Implications For Health Care Reform, Lisa C. Lindley, Maureen E. Lyon
Pediatrics Faculty Publications
Background: As the United States braces for full implementation of health care reform, the eyes of the nation are on Medicaid. The large number of newly eligible Medicaid beneficiaries may challenge health care resources and ultimately impact quality of care. This is a special concern among current Medicaid beneficiaries such as children with complex chronic conditions (CCCs) who have significant health care needs, especially at end of life (EOL). Yet, a comprehensive profile of these children is lacking.
Objective: To understand the demographic and health characteristics, health care utilization, and expenditures among Medicaid children with CCCs at EOL.
Methods: Our …
An Assessment Of Hepatitis E Virus (Hev) In Us Blood Donors And Recipients: No Detectable Hev Rna In 1939 Donors Tested And No Evidence For Hev Transmission To 362 Prospectively Followed Recipients., Chenyu Xu, Richard Y. Wang, Cathy A. Schechterly, Shengxiang Ge, James W. Shih, Ning-Shao Xia, Naomi L. Luban, Harvey J. Alter
An Assessment Of Hepatitis E Virus (Hev) In Us Blood Donors And Recipients: No Detectable Hev Rna In 1939 Donors Tested And No Evidence For Hev Transmission To 362 Prospectively Followed Recipients., Chenyu Xu, Richard Y. Wang, Cathy A. Schechterly, Shengxiang Ge, James W. Shih, Ning-Shao Xia, Naomi L. Luban, Harvey J. Alter
Pediatrics Faculty Publications
BACKGROUND:
Hepatitis E virus (HEV) infection has become relevant to blood transfusion practice because isolated cases of blood transmission have been reported and because HEV has been found to cause chronic infection and severe liver disease in immunocompromised patients.
STUDY DESIGN AND METHODS:
We tested for immunoglobulin (Ig)G and IgM antibodies to the HEV and for HEV RNA in 1939 unselected volunteer US blood donors. Subsequently, we tested the same variables in pre- and serial posttransfusion samples from 362 prospectively followed blood recipients to assess transfusion risk.
RESULTS:
IgG anti-HEV seroprevalence in the total 1939 donations was 18.8%: 916 of …
Second Messenger/Signal Transduction Pathways In Major Mood Disorders: Moving From Membrane To Mechanism Of Action, Part Ii: Bipolar Disorder, Mark Niciu, Dawn F. Ionescu, Daniel C. Matthews, Erica M. Richards, Carlos A. Zarate
Second Messenger/Signal Transduction Pathways In Major Mood Disorders: Moving From Membrane To Mechanism Of Action, Part Ii: Bipolar Disorder, Mark Niciu, Dawn F. Ionescu, Daniel C. Matthews, Erica M. Richards, Carlos A. Zarate
Psychiatry and Behavioral Sciences Faculty Publications
The etiopathogenesis and treatment of major mood disorders have historically focused on modulation of monoaminergic (serotonin, norepinephrine, dopamine) and amino acid [γ-aminobutyric acid (GABA), glutamate] receptors at the plasma membrane. Although the activation and inhibition of these receptors acutely alter local neurotransmitter levels, their neuropsychiatric effects are not immediately observed. This time lag implicates intracellular neuroplasticity as primary in the mechanism of action of antidepressants and mood stabilizers. The modulation of intracellular second messenger/signal transduction cascades affects neurotrophic pathways that are both necessary and sufficient for monoaminergic and amino acid–based treatments. In this review, we will discuss the evidence in …
Incidence Of Methicillin-Resistant Staphylococcus Aureus Infection In A Children's Hospital In The Washington Metropolitan Area Of The United States, 2003-2010, Xiaoyan Song, Joanthan Cogen, Nalini Singh
Incidence Of Methicillin-Resistant Staphylococcus Aureus Infection In A Children's Hospital In The Washington Metropolitan Area Of The United States, 2003-2010, Xiaoyan Song, Joanthan Cogen, Nalini Singh
Pediatrics Faculty Publications
Methicillin-resistant Staphylococcus aureus (MRSA) has emerged as a major public health threat. In this retrospective cohort study, we included patients with laboratory-confirmed MRSA infections treated at Children’s National Medical Center in Washington, District of Columbia between July 2003 and December 2010. The secular trends in the incidence rates of skin/soft tissue and invasive MRSA infections were assessed. Molecular analyses were performed on a subset of patients with invasive infections whose MRSA isolates were available for genotyping. The study identified 3750 patients with MRSA infections. The incidence of MRSA infections peaked in 2007 (incidence rate: 5.34 per 1000 patient-visits) and subsequently …
Palliative Care For The Medically Complex Child, Melanie Anspacher, Neha H. Shah
Palliative Care For The Medically Complex Child, Melanie Anspacher, Neha H. Shah
E-Learning Modules
**From the AAMC website: Contains time-sensitive information that will likely be inaccurate, obsolete, or irrelevant by August 12, 2016**
Children with special health care needs are increasingly prevalent in US hospitals. The pediatric hospitalist is often the primary provider of inpatient care for these patients. The Complex Care Curriculum was developed to provide a multi-modal educational curriculum for providers with the overall goal of improving inpatient care for this population. The curriculum is composed primarily of a series of topic-specific learning modules. This particular resource was created to provide pediatricians with educational materials related to palliative care for the medically …
Neonatal Neurobehavioral Abnormalities And Mri Brain Injury In Encephalopathic Newborns Treated With Hypothermia, Maya B. Coleman, Penny Glass, Judy Brown, Nadja Kadom, Tammy Tsuchida, Joseph Scafidi, Taeun Chang, Gilbert Vezina, An Nguyen Massaro
Neonatal Neurobehavioral Abnormalities And Mri Brain Injury In Encephalopathic Newborns Treated With Hypothermia, Maya B. Coleman, Penny Glass, Judy Brown, Nadja Kadom, Tammy Tsuchida, Joseph Scafidi, Taeun Chang, Gilbert Vezina, An Nguyen Massaro
Pediatrics Faculty Publications
Background
Neonatal Encephalopathy (NE) is a prominent cause of infant mortality and neurodevelopmental disability. Hypothermia is an effective neuroprotective therapy for newborns with encephalopathy. Post-hypothermia functional–anatomical correlation between neonatal neurobehavioral abnormalities and brain injury findings on MRI in encephalopathic newborns has not been previously described.
Aim
To evaluate the relationship between neonatal neurobehavioral abnormalities and brain injury on magnetic resonance imaging (MRI) in encephalopathic newborns treated with therapeutic hypothermia.
Study design
Neonates with hypoxic ischemic encephalopathy (HIE) referred for therapeutic hypothermia were prospectively enrolled in this observational study. Neurobehavioral functioning was assessed with the NICU network neurobehavioral scale (NNNS) performed …
Peripheral Laser Iridoplasty Opens Angle In Plateau Iris By Thinning The Cross-Sectional Tissues, Ji Liu, Tania Lamba, David A. Belyea
Peripheral Laser Iridoplasty Opens Angle In Plateau Iris By Thinning The Cross-Sectional Tissues, Ji Liu, Tania Lamba, David A. Belyea
Ophthalmology Faculty Publications
Plateau iris syndrome has been described as persistent angle narrowing or occlusion with intraocular pressure elevation after peripheral iridotomy due to the abnormal plateau iris configuration. Argon laser peripheral iridoplasty (ALPI) is an effective adjunct procedure to treat plateau iris syndrome. Classic theory suggests that the laser causes the contraction of the far peripheral iris stroma, "pulls" the iris away from the angle, and relieves the iris-angle apposition. We report a case of plateau iris syndrome that was successfully treated with ALPI. Spectral domain optical coherence tomography confirmed the angle was open at areas with laser treatment but remained appositionally …
Increased Reticulocytosis During Infancy Is Associated With Increased Hospitalizations In Sickle Cell Anemia Patients During The First Three Years Of Life, Emily Riehm Meier, Colleen Byrnes, Y. Terry Lee, Elizabeth C. Wright, Alan N. Schechter, Naomi L. Luban, Jeffrey L. Miller
Increased Reticulocytosis During Infancy Is Associated With Increased Hospitalizations In Sickle Cell Anemia Patients During The First Three Years Of Life, Emily Riehm Meier, Colleen Byrnes, Y. Terry Lee, Elizabeth C. Wright, Alan N. Schechter, Naomi L. Luban, Jeffrey L. Miller
Pediatrics Faculty Publications
Objective
Among older children with sickle cell anemia, leukocyte counts, hemoglobin, and reticulocytosis have previously been suggested as disease severity markers. Here we explored whether these blood parameters may be useful to predict early childhood disease severity when tested in early infancy, defined as postnatal ages 60–180 days.
Study Design
Data from fifty-nine subjects who were followed at Children’s National Medical Center’s Sickle Cell Program for at least three years was retrospectively analyzed. Comparisons were made between white blood cell counts, hemoglobin and reticulocyte levels measured at ages 60–180 days and the clinical course of sickle cell anemia during infancy …
Analysis Of Lmnb1 Duplications In Autosomal Dominant Leukodystrophy Provides Insights Into Duplication Mechanisms And Allele-Specific Expression, Elisa Giorgio, Harshvardhan Rolyan, Laura Kropp, Anish Baswanth Chakka, Svetlana Yatsenko, Adeline Vanderver, +31 Additional Authors
Analysis Of Lmnb1 Duplications In Autosomal Dominant Leukodystrophy Provides Insights Into Duplication Mechanisms And Allele-Specific Expression, Elisa Giorgio, Harshvardhan Rolyan, Laura Kropp, Anish Baswanth Chakka, Svetlana Yatsenko, Adeline Vanderver, +31 Additional Authors
Neurology Faculty Publications
Autosomal dominant leukodystrophy (ADLD) is an adult onset demyelinating disorder that is caused by duplications of the lamin B1 (LMNB1) gene. However, as only a few cases have been analyzed in detail, the mechanisms underlying LMNB1 duplications are unclear. We report the detailed molecular analysis of the largest collection of ADLD families studied, to date. We have identified the minimal duplicated region necessary for the disease, defined all the duplication junctions at the nucleotide level and identified the first inverted LMNB1 duplication. We have demonstrated that the duplications are not recurrent; patients with identical duplications …
Interests In High-Functioning Autism Are More Intense, Interfering, And Idiosyncratic Than Those In Neurotypical Development, Laura G. Anthony, Lauren Kenworthy, Benjamin E. Yerys, Kathryn F. Jankowski, Joette James, Madeline B. Harms, Alex Martin, Gregory L. Wallace
Interests In High-Functioning Autism Are More Intense, Interfering, And Idiosyncratic Than Those In Neurotypical Development, Laura G. Anthony, Lauren Kenworthy, Benjamin E. Yerys, Kathryn F. Jankowski, Joette James, Madeline B. Harms, Alex Martin, Gregory L. Wallace
Psychiatry and Behavioral Sciences Faculty Publications
Although circumscribed interests are pathognomonic with autism, much about these interests remains unknown. Using the Interests Scale (IS), this study compares interests between 76 neurotypical (NT) individuals and 109 individuals with high-functioning autism spectrum disorder (HF-ASD) matched groupwise on age, IQ, and gender ratio. Participants and their parents/caregivers completed diagnostic measures (the Autism Diagnostic Interview—Revised and the Autism Diagnostic Observation Schedule; HF-ASD only), cognitive tests (Wechsler IQ Scales), and questionnaires (the Repetitive Behavior Scale—Revised, the Behavior Rating Inventory of Executive Function, and the Social Responsiveness Scale), in addition to the IS. Consistent with previous research, HF-ASD and NT individuals did …
Ereply. Re: Balancing The Pulmonary Circulation In Tricuspid Atresia With An Aortopulmonary Window, Pranava Sinha, Syed Murfad Peer, Mary T. Donofrio
Ereply. Re: Balancing The Pulmonary Circulation In Tricuspid Atresia With An Aortopulmonary Window, Pranava Sinha, Syed Murfad Peer, Mary T. Donofrio
Pediatrics Faculty Publications
No abstract provided.
Appendicitis Is Like A Box Of Chocolates, Clint D. Cappiello, Phillip C. Guzzetta, Faisal G. Qureshi
Appendicitis Is Like A Box Of Chocolates, Clint D. Cappiello, Phillip C. Guzzetta, Faisal G. Qureshi
Pediatrics Faculty Publications
Appendicitis is the most commonly encountered abdominal emergency in pediatric surgery. However, the presentation of acute appendicitis is as diverse as the patient population. We present here a case of appendicitis presenting as an intra-abdominal mass consistent with lymphoma. Our patient is a 3 year old male with an atypical presentation of acute appendicitis. Additionally, he was found to have malrotation at the time of surgery. A delayed presentation, coupled with anomalous laboratory findings and aberrant anatomy made for a difficult diagnosis and overall interesting case of appendicitis.
Metastatic Castration-Resistant Prostate Cancer: Critical Review Of Enzalutamide, Joelle El-Amm, Nihar Patel, Ashley Freeman, Jeanny B. Aragon-Ching
Metastatic Castration-Resistant Prostate Cancer: Critical Review Of Enzalutamide, Joelle El-Amm, Nihar Patel, Ashley Freeman, Jeanny B. Aragon-Ching
Medicine Faculty Publications
Enzalutamide, previously known as MDV300, is an oral, second-generation androgen receptor (AR) signaling inhibitor or antagonist that was approved by the Food and Drug Administration in 2012 for the treatment of metastatic castrate-resistant prostate cancer (mCRPC) postdocetaxel. Preclinical studies have demonstrated impressive affinity to the AR compared to the first-generation AR inhibitors. The landmark Phase III AFFIRM trial demonstrated improved overall survival benefit compared to placebo in addition to improvement in all tested parameters. Enzalutamide is currently being studied in several trials prechemotherapy and in earlier settings of prostate cancer. This review will discuss the mechanism of action of enzalutamide, …
Pediatric Palliative Care For Youth With Hiv/Aids: Systematic Review Of The Literature, Megan L. Wilkins, Ronald H. Dallas, Kathleen E. Fanone, Maureen E. Lyon
Pediatric Palliative Care For Youth With Hiv/Aids: Systematic Review Of The Literature, Megan L. Wilkins, Ronald H. Dallas, Kathleen E. Fanone, Maureen E. Lyon
Pediatrics Faculty Publications
Improvement in treatment has led to decreased death in youth with human immunodeficiency virus (HIV) in developed countries. Despite this, youth with HIV are still at risk for increased mortality and morbidity compared with their uninfected counterparts. In developing countries, high numbers of youth die from acquired immune deficiency syndrome (AIDS)-related illnesses due to lack of access to consistent antiretroviral treatment. As a result, pediatric palliative care is a relevant topic for those providing care to youth with HIV. A systematic review was conducted to gather information regarding the status of the literature related to pediatric palliative care and medical …
Crystal Structure Of The N-Acetyltransferase Domain Of Human N-Acetyl-L-Glutamate Synthase In Complex With N-Acetyl-L-Glutamate Provides Insights Into Its Catalytic And Regulatory Mechanisms, Gengxiang Zhao, Zhongmin Jin, Norma M. Allewell, Mendel Tuchman, Dashuang Shi
Crystal Structure Of The N-Acetyltransferase Domain Of Human N-Acetyl-L-Glutamate Synthase In Complex With N-Acetyl-L-Glutamate Provides Insights Into Its Catalytic And Regulatory Mechanisms, Gengxiang Zhao, Zhongmin Jin, Norma M. Allewell, Mendel Tuchman, Dashuang Shi
Pediatrics Faculty Publications
N-acetylglutamate synthase (NAGS) catalyzes the conversion of AcCoA and L-glutamate to CoA and N-acetyl-L-glutamate (NAG), an obligate cofactor for carbamyl phosphate synthetase I (CPSI) in the urea cycle. NAGS deficiency results in elevated levels of plasma ammonia which is neurotoxic. We report herein the first crystal structure of human NAGS, that of the catalyticN-acetyltransferase (hNAT) domain with N-acetyl-L-glutamate bound at 2.1 Å resolution. Functional studies indicate that the hNAT domain retains catalytic activity in the absence of the amino acid kinase (AAK) domain. Instead, the major functions of the AAK domain appear to be providing …
Creation And Implementation Of An Emergency Medicine Education And Training Program In Turkey: An Effective Educational Intervention To Address The Practitioner Gap, Jennifer Whitfield Bellows, Katherine A. Douglass, Atilla Ridvan, Jeffrey Smith, G. Bobby Kapur
Creation And Implementation Of An Emergency Medicine Education And Training Program In Turkey: An Effective Educational Intervention To Address The Practitioner Gap, Jennifer Whitfield Bellows, Katherine A. Douglass, Atilla Ridvan, Jeffrey Smith, G. Bobby Kapur
Emergency Medicine Faculty Publications
Background
The specialty of Emergency Medicine has enjoyed recognition for nearly 20 years in Turkey. However, the majority of underserved and rural Turkish emergency departments are staffed by general practitioners who lack formal training in the specialty and have few opportunities to increase emergency medicine-specific knowledge and skills.
Methods
To address this “practitioner gap,” the authors developed a four-phase comprehensive emergency medicine education and training program for general practitioners practicing in government hospitals in Turkey.
Results
From April 2006 until June 2009, 42 courses were taught by 62 trainers across seven regions in Turkey. A total of 2,262 physicians were …
A Synthetic Model Of Human Beta-Thalassemia Erythropoiesis Using Cd34+ Cells From Healthy Adult Donors, Y. Terry Lee, Ki-Soon Kim, Colleen Byrnes, Jaira F. De Vasconcellos, Seung-Jae Noh, Antoinette Rabel, Emily Riehm Meier, Jeffrey L. Meier
A Synthetic Model Of Human Beta-Thalassemia Erythropoiesis Using Cd34+ Cells From Healthy Adult Donors, Y. Terry Lee, Ki-Soon Kim, Colleen Byrnes, Jaira F. De Vasconcellos, Seung-Jae Noh, Antoinette Rabel, Emily Riehm Meier, Jeffrey L. Meier
Pediatrics Faculty Publications
Based upon the lack of clinical samples available for research in many laboratories worldwide, a significant gap exists between basic and clinical studies of beta-thalassemia major. To bridge this gap, we developed an artificially engineered model for human beta thalassemia by knocking down beta-globin gene and protein expression in cultured CD34+ cells obtained from healthy adults. Lentiviral-mediated transduction of beta-globin shRNA (beta-KD) caused imbalanced globin chain production. Beta-globin mRNA was reduced by 90% compared to controls, while alpha-globin mRNA levels were maintained. HPLC analyses revealed a 96% reduction in HbA with only a minor increase in HbF. During the terminal …
Racial Disparities In The Use Of Cardiac Revascularization: Does Local Hospital Capacity Matter?, Suhui Li, Arnold Chen, Katherine H. Mead
Racial Disparities In The Use Of Cardiac Revascularization: Does Local Hospital Capacity Matter?, Suhui Li, Arnold Chen, Katherine H. Mead
Health Policy and Management Faculty Publications
Objective: To assess the extent to which the observed racial disparities in cardiac revascularization use can be explained by the variation across counties where patients live, and how the within-county racial disparities is associated with the local hospital capacity.
Data Sources: Administrative data from Pennsylvania Health Care Cost Containment Council (PHC4) between 1995 and 2006.
Study Design: The study sample included 207,570 Medicare patients admitted to hospital for acute myocardial infarction (AMI). We identified the use of coronary artery bypass graft (CABG) and percutaneous coronary intervention (PCI) procedures within three months after the patient’s initial admission for AMI. Multi-level hierarchical …
Is Adrenalectomy Necessary During Unilateral Nephrectomy For Wilms Tumor? A Report From The Children's Oncology Group., Kathleen Kieran, James R Anderson, Jeffrey S Dome, Peter F Ehrlich, Michael L Ritchey, Robert C Shamberger, Elizabeth J Perlman, Daniel M Green, Andrew M Davidoff
Is Adrenalectomy Necessary During Unilateral Nephrectomy For Wilms Tumor? A Report From The Children's Oncology Group., Kathleen Kieran, James R Anderson, Jeffrey S Dome, Peter F Ehrlich, Michael L Ritchey, Robert C Shamberger, Elizabeth J Perlman, Daniel M Green, Andrew M Davidoff
Pediatrics Faculty Publications
PURPOSE: To determine whether performing adrenalectomy at the time of nephrectomy for unilateral Wilms tumor impacts clinical outcome.
METHODS: We reviewed information on all patients enrolled on National Wilms Tumor Study-4 and -5. Data were abstracted on patient demographics, tumor characteristics, surgical and pathologic status of the adrenal gland, and patient outcomes. The primary endpoints were intraoperative spill and five-year event-free survival (EFS) in patients who did or did not undergo adrenalectomy.
RESULTS: Of 3825 patients with complete evaluable data, the adrenal was left in situ in 2264 (57.9%) patients, and was removed completely in 1367 patients (36.7%) or partially …
Impact Of Vulvovaginal Health On Postmenopausal Women: A Review Of Surveys On Symptoms Of Vulvovaginal Atrophy, Sharon J. Parish, Rossella E. Nappi, Michael L. Krychman, Susan Kellogg-Spadt, James A. Simon, Jeffrey A. Goldstein, Sheryl A. Kingsberg
Impact Of Vulvovaginal Health On Postmenopausal Women: A Review Of Surveys On Symptoms Of Vulvovaginal Atrophy, Sharon J. Parish, Rossella E. Nappi, Michael L. Krychman, Susan Kellogg-Spadt, James A. Simon, Jeffrey A. Goldstein, Sheryl A. Kingsberg
Obstetrics and Gynecology Faculty Publications
Several recent, large-scale studies have provided valuable insights into patient perspectives on postmenopausal vulvovaginal health. Symptoms of vulvovaginal atrophy, which include dryness, irritation, itching, dysuria, and dyspareunia, can adversely affect interpersonal relationships, quality of life, and sexual function. While approximately half of postmenopausal women report these symptoms, far fewer seek treatment, often because they are uninformed about hypoestrogenic postmenopausal vulvovaginal changes and the availability of safe, effective, and well-tolerated treatments, particularly local vaginal estrogen therapy. Because women hesitate to seek help for symptoms, a proactive approach to conversations about vulvovaginal discomfort would improve diagnosis and treatment.
Smartphone Apps For Disasters: Workshop Lesson Plan, Paul Levett
Smartphone Apps For Disasters: Workshop Lesson Plan, Paul Levett
Himmelfarb Library Faculty Posters and Presentations
This instruction was designed to be delivered to a Disaster Medical Assistance Team (DMAT) or a Community Emergency Response Team (CERT).
Instructional Goal #1: Using a personal smartphone connected to the internet, learners will download medical information apps, before deployment to a disaster environment.
Instructional Goal #2: Recalling real and simulated patient care situations that required additional medical information, learners will identify and search smartphone apps for relevant, current, authoritative, medical information that could be applied to the care of individual patients in disasters.