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Articles 811 - 840 of 1049

Full-Text Articles in Medical Specialties

Neuroimaging Of Unusual Glioblastoma Using Diffusion Tensor Imaging, Akshita Mehta, Lucien M. Levy, M. Isabel Almira-Suarez, Raymond Tu Apr 2014

Neuroimaging Of Unusual Glioblastoma Using Diffusion Tensor Imaging, Akshita Mehta, Lucien M. Levy, M. Isabel Almira-Suarez, Raymond Tu

GW Research Days 2014

Background: Gliosarcoma refers to an uncommon astrocytic tumor of the central nervous system. These tumors include both glial and mesenchymal components by definition, and are extremely malignant. Gliosarcomas are particular to tumors with distinct gliomatous and sarcomatous constituents, and are distinguished from gliobastomas which have undergone mesenchymal metaplasia. Gliosarcomas encompass 2-8% of all glioblastoma cases and tend to occupy the supratentorial regions of the brain, especially the temporal lobe. Rare infratentorial lesions including the cerebellar hemisphere, intraventricular, and multi-focal tumors have also been reported. Accurate neuroimaging diagnosis is critical and diffusion tensor imaging (DTI) and spectroscopy can be useful to …


Bilateral Laparoscopic Partial Adrenalectomy: A Case Presentation And Review Of The Literature, Sean Satey, Nathan Richards, Frederick J. Brody Apr 2014

Bilateral Laparoscopic Partial Adrenalectomy: A Case Presentation And Review Of The Literature, Sean Satey, Nathan Richards, Frederick J. Brody

Surgery Faculty Publications

Laparoscopic adrenalectomy is the standard of care for benign adrenal diseases. Partial adrenalectomy has been performed for various tumors to preserve adrenal function while avoiding steroid supplements postoperatively. This article discusses a patient that underwent bilateral laparoscopic partial adrenalectomies for bilateral pheochromocytomas and reviews the literature on partial adrenalectomy. A 40-year-old woman with a recent diagnosis of hypertension and an emergent Caesarian section for pre-eclampsia presented to an outside clinic with labile blood pressure, diaphoresis, palpitations, and headache. Physical examination was unremarkable with no bruits appreciated in the neck or abdomen. Medical management of her hypertension was initiated with minimal …


Childhood Achalasia: A Comprehensive Review Of Disease, Diagnosis And Therapeutic Management, Ashanti L. Franklin, Mikael Petrosyan, Timothy D. Kane Apr 2014

Childhood Achalasia: A Comprehensive Review Of Disease, Diagnosis And Therapeutic Management, Ashanti L. Franklin, Mikael Petrosyan, Timothy D. Kane

Surgery Faculty Publications

Achalasia is an esophageal motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation and is rare in children. The most common symptoms are vomiting, dysphagia, regurgitation, and weight loss. Definitive diagnosis is made with barium swallow study and esophageal manometry. In adults, endoscopic biopsy is recommended to exclude malignancy however; it is not as often indicated in children. Medical management often fails resulting in recurrent symptoms and the ultimate definitive treatment is surgical. Laparoscopic Heller myotomy with or without an anti-reflux procedure is the treatment of choice and has become standard of care for children with achalasia. Peroral …


Transoral Incisionless Fundoplication Effective In Eliminating Gerd Symptoms In Partial Responders To Proton Pump Inhibitor Therapy At 6 Months: The Tempo Randomized Clinical Trial, Karim S. Trad, William E. Barnes, Gilbert Simoni, Ahmad B. Shughoury, Peter G. Mavrelis, Mamoon Raza, Jeffrey Heise, Daniel G. Turgeon, Mark A. Fox Apr 2014

Transoral Incisionless Fundoplication Effective In Eliminating Gerd Symptoms In Partial Responders To Proton Pump Inhibitor Therapy At 6 Months: The Tempo Randomized Clinical Trial, Karim S. Trad, William E. Barnes, Gilbert Simoni, Ahmad B. Shughoury, Peter G. Mavrelis, Mamoon Raza, Jeffrey Heise, Daniel G. Turgeon, Mark A. Fox

Surgery Faculty Publications

Background. Incomplete control of troublesome regurgitation and extraesophageal manifestations of chronic gastroesophageal reflux disease (GERD) is a known limitation of proton pump inhibitor (PPI) therapy. This multicenter randomized study compared the efficacy of transoral incisionless fundoplication (TIF) against PPIs in controlling these symptoms in patients with small hiatal hernias.

Methods. Between June and August 2012, 63 patients were randomized at 7 US community hospitals. Patients in the PPI group were placed on maximum standard dose (MSD). Patients in the TIF group underwent esophagogastric fundoplication using the EsophyX2 device. Primary outcome was elimination of daily troublesome regurgitation or …


Redefining Frequent Emergency Department Users, Amy Waldner, Maria Raven, Danielle Lazar, Jesse Pines Apr 2014

Redefining Frequent Emergency Department Users, Amy Waldner, Maria Raven, Danielle Lazar, Jesse Pines

Center for Health Care Quality

Frequent ED users are perceived to be a costly population that often abuse or misuse ED services due to a combination of unmet social needs and medical conditions that, in theory, could be treated outside of the ED at a lower cost. The reality is that factors contributing to frequent ED use are more varied and complex than originally believed.


The Impact Of Salsalate Treatment On Serum Levels Of Advanced Glycation End Products In Type 2 Diabetes., Joshua I Barzilay, Kathleen A. Jablonski, Vivian Fonseca, Steven E Shoelson, Allison B Goldfine, Christopher Strauch, Vincent M Monnier, Tinsal-T2d Research Consortium. Apr 2014

The Impact Of Salsalate Treatment On Serum Levels Of Advanced Glycation End Products In Type 2 Diabetes., Joshua I Barzilay, Kathleen A. Jablonski, Vivian Fonseca, Steven E Shoelson, Allison B Goldfine, Christopher Strauch, Vincent M Monnier, Tinsal-T2d Research Consortium.

GW Biostatistics Center

OBJECTIVE Salsalate is a nonacetylated salicylate that lowers glucose levels in people with type 2 diabetes (T2D). Here we examined whether salsalate also lowered serum-protein-bound levels of early and advanced glycation end products (AGEs) that have been implicated in diabetic vascular complications. RESEARCH DESIGN AND METHODS Participants were from the Targeting Inflammation Using Salsalate for Type 2 Diabetes (TINSAL-T2D) study, which examined the impact of salsalate treatment on hemoglobin A1c (HbA1c) and a wide variety of other parameters. One hundred eighteen participants received salsalate, 3.5 g/day for 48 weeks, and 109 received placebo. Early glycation product levels (HbA1c and fructoselysine …


Genetic Risk Of Progression To Type 2 Diabetes And Response To Intensive Lifestyle Or Metformin In Prediabetic Women With And Without A History Of Gestational Diabetes Mellitus., Shannon D Sullivan, Kathleen A. Jablonski, Jose C Florez, Dana Dabelea, Paul W Franks, Sam Dagogo-Jack, Catherine Kim, William C Knowler, Costas A Christophi, Robert Ratner, Diabetes Prevention Program Research Group. Apr 2014

Genetic Risk Of Progression To Type 2 Diabetes And Response To Intensive Lifestyle Or Metformin In Prediabetic Women With And Without A History Of Gestational Diabetes Mellitus., Shannon D Sullivan, Kathleen A. Jablonski, Jose C Florez, Dana Dabelea, Paul W Franks, Sam Dagogo-Jack, Catherine Kim, William C Knowler, Costas A Christophi, Robert Ratner, Diabetes Prevention Program Research Group.

GW Biostatistics Center

OBJECTIVE The Diabetes Prevention Program (DPP) trial investigated rates of progression to diabetes among adults with prediabetes randomized to treatment with placebo, metformin, or intensive lifestyle intervention. Among women in the DPP, diabetes risk reduction with metformin was greater in women with prior gestational diabetes mellitus (GDM) compared with women without GDM but with one or more previous live births.

RESEARCH DESIGN AND METHODS We asked if genetic variability could account for these differences by comparing β-cell function and genetic risk scores (GRS), calculated from 34 diabetes-associated loci, between women with and without histories of GDM.

RESULTS β-Cell function was …


Ultrasound-Enhanced Ocular Delivery Of Dexamethasone Sodium Phosphate: An In Vivo Study, Marjan Nabili, Aditi Shenoy, Shawn Chawla, Sankaranarayana P. Mahesh, Craig Geist, Vesna Zderic Mar 2014

Ultrasound-Enhanced Ocular Delivery Of Dexamethasone Sodium Phosphate: An In Vivo Study, Marjan Nabili, Aditi Shenoy, Shawn Chawla, Sankaranarayana P. Mahesh, Craig Geist, Vesna Zderic

Ophthalmology Faculty Publications

Background

The eye's unique anatomy and its physiological and anatomical barriers can limit effective drug delivery into the eye.

Methods

An in vivo study was designed to determine the effectiveness and safety of ultrasound application in enhancing drug delivery in a rabbit model. Permeability of a steroid ophthalmic drug, dexamethasone sodium phosphate, was investigated in ultrasound- and sham-treated cases. For this study, an eye cup filled with dexamethasone sodium phosphate was placed on the cornea. Ultrasound was applied at intensity of 0.8 W/cm2 and frequency of 400 or 600 kHz for 5 min. The drug concentration in aqueous humor …


International Telemedicine Consultations For Neurodevelopmental Disabilities, Phillip L. Pearl, Craig A. Sable, Sarah Helen Evans, Joseph Knight, Parker Cunningham, Gaetano R. Lotrecchiano, Andrea L. Gropman, Sheela Stuart, Penny J. Glass, Anne Conway, Issam Ramadan, Tania Paiva, Mark L. Batshaw, Roger J. Packer Mar 2014

International Telemedicine Consultations For Neurodevelopmental Disabilities, Phillip L. Pearl, Craig A. Sable, Sarah Helen Evans, Joseph Knight, Parker Cunningham, Gaetano R. Lotrecchiano, Andrea L. Gropman, Sheela Stuart, Penny J. Glass, Anne Conway, Issam Ramadan, Tania Paiva, Mark L. Batshaw, Roger J. Packer

Neurology Faculty Publications

Background: A telemedicine program was developed between the Children's National Medical Center (CNMC) in Washington, DC, and the Sheikh Khalifa Bin Zayed Foundation in the United Arab Emirates (UAE). A needs assessment and a curriculum of on-site training conferences were devised preparatory to an ongoing telemedicine consultation program for children with neurodevelopmental disabilities in the underserved eastern region of the UAE.

Materials and Methods: Weekly telemedicine consultations are provided by a multidisciplinary faculty. Patients are presented in the UAE with their therapists and families. Real-time (video over Internet protocol; average connection, 768 kilobits/s) telemedicine conferences are held weekly following previews …


Indirect Cyclopexy For Treatment Of A Chronic Traumatic Cyclodialysis Cleft With Hypotony, Nisha Chadha, Tania Lamba, David A. Belyea, Kunal Y. Merchant Mar 2014

Indirect Cyclopexy For Treatment Of A Chronic Traumatic Cyclodialysis Cleft With Hypotony, Nisha Chadha, Tania Lamba, David A. Belyea, Kunal Y. Merchant

Ophthalmology Faculty Publications

Cyclodialysis cleft is a rare clinical finding and therefore, reports on surgical repair techniques in the literature are limited. Additionally, hypotony can make repair technically challenging. We share a novel, simple surgical approach to management of a case of chronic traumatic cyclodialysis cleft with a successful outcome.


Microfluidic-Enabled Liposomes Elucidate Size-Dependent Transdermal Transport, Renee R. Hood, Eric L. Kendall, Mariana Junqueira, Wyatt N. Vreeland, Zenaide M.N. Quezado, Julia C. Finkel, Don L. Devoe Mar 2014

Microfluidic-Enabled Liposomes Elucidate Size-Dependent Transdermal Transport, Renee R. Hood, Eric L. Kendall, Mariana Junqueira, Wyatt N. Vreeland, Zenaide M.N. Quezado, Julia C. Finkel, Don L. Devoe

Pediatrics Faculty Publications

Microfluidic synthesis of small and nearly-monodisperse liposomes is used to investigate the size-dependent passive transdermal transport of nanoscale lipid vesicles. While large liposomes with diameters above 105 nm are found to be excluded from deeper skin layers past the stratum corneum, the primary barrier to nanoparticle transport, liposomes with mean diameters between 31–41 nm exhibit significantly enhanced penetration. Furthermore, multicolor fluorescence imaging reveals that the smaller liposomes pass rapidly through the stratum corneum without vesicle rupture. These findings reveal that nanoscale liposomes with well-controlled size and minimal size variance are excellent vehicles for transdermal delivery of functional nanoparticle drugs.


The Temporal Version Of The Pediatric Sepsis Biomarker Risk Model, Hector R. Wong, Scott L. Weiss, John S. Giuliano Jr., Mark S. Wainwright, Natalie Z. Cvijanovich, Robert J. Freishtat, +19 Additional Authors Mar 2014

The Temporal Version Of The Pediatric Sepsis Biomarker Risk Model, Hector R. Wong, Scott L. Weiss, John S. Giuliano Jr., Mark S. Wainwright, Natalie Z. Cvijanovich, Robert J. Freishtat, +19 Additional Authors

Pediatrics Faculty Publications

Background

PERSEVERE is a risk model for estimating mortality probability in pediatric septic shock, using five biomarkers measured within 24 hours of clinical presentation.

Objective

Here, we derive and test a temporal version of PERSEVERE (tPERSEVERE) that considers biomarker values at the first and third day following presentation to estimate the probability of a “complicated course”, defined as persistence of ≥2 organ failures at seven days after meeting criteria for septic shock, or death within 28 days.

Methods

Biomarkers were measured in the derivation cohort (n = 225) using serum samples obtained during days 1 and 3 of septic shock. …


Challenging Situations In Family Centered Rounds: Making The Best Out Of Worst Case Scenarios, Priti Bhansali, Kerry Moss, Christiane Corriveau, Geeta Singhal, Robert Kavanagh, Jeffrey Simmons Mar 2014

Challenging Situations In Family Centered Rounds: Making The Best Out Of Worst Case Scenarios, Priti Bhansali, Kerry Moss, Christiane Corriveau, Geeta Singhal, Robert Kavanagh, Jeffrey Simmons

E-Learning Modules

This resource is a workshop intended for both seasoned and novice health care providers who are interested in becoming prepared for challenging scenarios that may be encountered during FCR. Case scenarios of challenging situations relating to discussing sensitive topics, correcting errors, and teaching learners in front of both parents and patients are presented. Tangible opportunities to role model communication skills, efficiency, and teaching skills for trainees are highlighted. The session will conclude with a question and answer session with a multi-specialty panel to share experiences and strategies for addressing the unexpected during FCR. By the end of the workshop, participants …


Genomics In Premature Infants: A Non-Invasive Strategy To Obtain High-Quality Dna, Mariam M. Said, Clint D. Cappiello, Joseph M. Devaney, Daniele S. Podini, Alana L. Beres, Stanislav Vukmanovic, Khodayar Rais-Bahrami, Naomi L. Luban, Anthony D. Sandler, Zohreh Tatari-Calderone Mar 2014

Genomics In Premature Infants: A Non-Invasive Strategy To Obtain High-Quality Dna, Mariam M. Said, Clint D. Cappiello, Joseph M. Devaney, Daniele S. Podini, Alana L. Beres, Stanislav Vukmanovic, Khodayar Rais-Bahrami, Naomi L. Luban, Anthony D. Sandler, Zohreh Tatari-Calderone

Pediatrics Faculty Publications

We used a cost-effective, non-invasive method to obtain high-quality DNA from buccal epithelial-cells (BEC) of premature infants for genomic analysis. DNAs from BEC were obtained from premature infants with gestational age ≤ 36 weeks. Short terminal repeats (STRs) were performed simultaneously on DNA obtained from the buccal swabs and blood from the same patient. The STR profiles demonstrated that the samples originated from the same individual and exclude any contamination by external DNAs. Whole exome sequencing was performed on DNAs obtained from BEC on premature infants with and without necrotizing enterocolitis, and successfully provided a total number of reads and …


Do Not Judge A Kidney By Its Pelvis: A Rare Case Of An Unobstructed Dilated Renal Pelvis, Aaron D. Martin, Craig A. Peters Mar 2014

Do Not Judge A Kidney By Its Pelvis: A Rare Case Of An Unobstructed Dilated Renal Pelvis, Aaron D. Martin, Craig A. Peters

Urology Faculty Publications

We present a rare case of an unobstructed dilated renal pelvis in a newborn female. Prenatal imaging documented a large abdominal cyst in a stable fetus. Postnatal imaging confirmed persistence of this large cyst but with an unclear etiology. The child was taken to surgery by the pediatric surgeons with concern for a possible harmful nonurologic diagnosis. Intraoperative findings were that of a severely dilated renal pelvis; however, in the absence of an expected ureteropelvic junction obstruction. Reduction pyeloplasty without interference of the ureteropelvic junction proved successful.


Trends In Antibiotic Resistance In Coagulase-Negative Staphylococci In The United States, 1999 To 2012, Larissa S. May, Eili Y. Klein, Richard E. Rothman, Ramanan Laxminarayan Mar 2014

Trends In Antibiotic Resistance In Coagulase-Negative Staphylococci In The United States, 1999 To 2012, Larissa S. May, Eili Y. Klein, Richard E. Rothman, Ramanan Laxminarayan

Emergency Medicine Faculty Publications

Coagulase-negative staphylococci (CoNS) are important bloodstream pathogens that are typically resistant to multiple antibiotics. Despite the concern about increasing resistance, there have been no recent studies describing the national prevalence of CoNS pathogens. We used national resistance data over a period of 13 years (1999 to 2012) from The Surveillance Network (TSN) to determine the prevalence of and assess the trends in resistance for Staphylococcus epidermidis, the most common CoNS pathogen, and all other CoNS pathogens. Over the course of the study period, S. epidermidis resistance to ciprofloxacin and clindamycin increased steadily from 58.3% to 68.4% and from 43.4% to …


Long-Term Safety Of Ospemifene (52-Week Extension) In The Treatment Of Vulvar And Vaginal Atrophy In Hysterectomized Postmenopausal Women, James A. Simon, David J. Portman, R. Garn Mabey, The Ospemifene Study Group Mar 2014

Long-Term Safety Of Ospemifene (52-Week Extension) In The Treatment Of Vulvar And Vaginal Atrophy In Hysterectomized Postmenopausal Women, James A. Simon, David J. Portman, R. Garn Mabey, The Ospemifene Study Group

Obstetrics and Gynecology Faculty Publications

Objective

To examine the long-term safety of oral ospemifene, a non-estrogen tissue-selective estrogen agonist/antagonist, for the treatment of moderate to severe dyspareunia, a symptom of vulvar and vaginal atrophy (VVA) due to menopause.

Study design

This multicenter, long-term, open-label, safety extension study was conducted in women without a uterus aged 40–80 years (N = 301) who received oral ospemifene 60 mg/day for 52 weeks. Participants either continued their 60-mg/day ospemifene dose from the initial 12-week pivotal efficacy study or switched from blinded placebo or ospemifene 30 mg/day to open-label ospemifene 60 mg/day. The 52-week open-label extension period plus initial …


Child And Adolescent Psychiatrists' Attitudes And Practices Prescribing Second Generation Antipsychotics, Angie Mae Rodday, Susan K. Parsons, Christoph U. Correll, Adelaide S. Robb, Bonnie T. Zima, Tully S. Saunders, Laurel K. Leslie Mar 2014

Child And Adolescent Psychiatrists' Attitudes And Practices Prescribing Second Generation Antipsychotics, Angie Mae Rodday, Susan K. Parsons, Christoph U. Correll, Adelaide S. Robb, Bonnie T. Zima, Tully S. Saunders, Laurel K. Leslie

Psychiatry and Behavioral Sciences Faculty Publications

Objective: The purpose of this study was to examine psychiatrists' attitudes and practices in prescribing second-generation antipsychotics (SGA) to children and adolescents (referred to here as “children”) and identify factors associated with off-label SGA use.

Methods: A survey was mailed to a national, randomly selected sample of 1600 child and adolescent psychiatrists identified by the American Medical Association. Multivariable logistic regression was used to identify factors, including psychiatrists' characteristics, practice characteristics, and psychiatrists' attitudes, that are associated with off-label SGA use (i.e., SGAs used in children with attention-deficit/hyperactivity disorder (ADHD), oppositional defiant disorder, conduct disorder, or nonbipolar mood disorders).

Results: …


A Melanin-Independent Interaction Between Mc1r And Met Signalling Pathways Is Required For Hgf-Dependent Melanoma, Agnieszka Wolnicka-Głubisz, Faith M. Strickland, Albert Wielgus, Miriam Anver, Glenn Merlino, Edward C. De Fabo, Frances P. Noonan Feb 2014

A Melanin-Independent Interaction Between Mc1r And Met Signalling Pathways Is Required For Hgf-Dependent Melanoma, Agnieszka Wolnicka-Głubisz, Faith M. Strickland, Albert Wielgus, Miriam Anver, Glenn Merlino, Edward C. De Fabo, Frances P. Noonan

Microbiology, Immunology, and Tropical Medicine Faculty Publications

Melanocortin 1 receptor (MC1R) signaling stimulates black eumelanin production through a cAMP-dependent pathway. MC1R polymorphisms can impair this process, resulting in a predominance of red phaeomelanin. The red hair, fair skin and UV sensitive phenotype is a well-described melanoma risk factor. MC1R polymorphisms also confer melanoma risk independent of pigment. We investigated the effect of Mc1r deficiency in a mouse model of UV-induced melanoma. C57BL/6-Mc1r+/+-HGF transgenic mice have a characteristic hyperpigmented black phenotype with extra-follicular dermal melanocytes located at the dermal/epidermal junction. UVB induces melanoma, independent of melanin pigmentation, but UVA-induced and spontaneous melanomas are dependent on black eumelanin. We …


Gaps And Opportunities In Refractory Status Epilepticus Research In Children: A Multi-Center Approach By The Pediatric Status Epilepticus Research Group (Pserg), Ivan Sanchez Fernandez, Nicholas S. Abend, Satish Agadi, Sookee An, Ravindra Arya, Jessica L. Carpenter, Kevin E. Chapman, William D. Gaillard, Tracy A. Glauser, David B. Golstein, Joshua L. Goldstein, Howard P. Goodkin, Cecil D. Hahn, Erin L. Heinzen, Mohamad A. Mikati, Katrina Peariso, John P. Pestian, Margie Ream, James J. Riviello, Robert C. Tasker, Korwyn Williams, Tobias Loddenkemper Feb 2014

Gaps And Opportunities In Refractory Status Epilepticus Research In Children: A Multi-Center Approach By The Pediatric Status Epilepticus Research Group (Pserg), Ivan Sanchez Fernandez, Nicholas S. Abend, Satish Agadi, Sookee An, Ravindra Arya, Jessica L. Carpenter, Kevin E. Chapman, William D. Gaillard, Tracy A. Glauser, David B. Golstein, Joshua L. Goldstein, Howard P. Goodkin, Cecil D. Hahn, Erin L. Heinzen, Mohamad A. Mikati, Katrina Peariso, John P. Pestian, Margie Ream, James J. Riviello, Robert C. Tasker, Korwyn Williams, Tobias Loddenkemper

Neurology Faculty Publications

PURPOSE:

Status epilepticus (SE) is a life-threatening condition that can be refractory to initial treatment. Randomized controlled studies to guide treatment choices, especially beyond first-line drugs, are not available. This report summarizes the evidence that guides the management of refractory convulsive SE (RCSE) in children, defines gaps in our clinical knowledge and describes the development and works of the 'pediatric Status Epilepticus Research Group' (pSERG).

METHODS:

A literature review was performed to evaluate current gaps in the pediatric SE and RCSE literature. In person and online meetings helped to develop and expand the pSERG network.

RESULTS:

The care of pediatric …


Phase 2 Randomized, Flexible Crossover, Double-Blinded, Placebo-Controlled Trial Of The Farnesyltransferase Inhibitor Tipifarnib In Children And Young Adults With Neurofibromatosis Type 1 And Progressive Plexiform Neurofibromas, Brigitte C. Widemann, Eva Dombi, Andrea Gillespie, Pamela L. Wolters, Jean Belasco, Aerang Kim, +13 Additional Authors Feb 2014

Phase 2 Randomized, Flexible Crossover, Double-Blinded, Placebo-Controlled Trial Of The Farnesyltransferase Inhibitor Tipifarnib In Children And Young Adults With Neurofibromatosis Type 1 And Progressive Plexiform Neurofibromas, Brigitte C. Widemann, Eva Dombi, Andrea Gillespie, Pamela L. Wolters, Jean Belasco, Aerang Kim, +13 Additional Authors

Pediatrics Faculty Publications

Background

RAS is dysregulated in neurofibromatosis type 1 (NF1) related plexiform neurofibromas (PNs). The activity of tipifarnib, which blocks RAS signaling by inhibiting its farnesylation, was tested in children and young adults with NF1 and progressive PNs.

Methods

Patients aged 3–25 years with NF1-related PNs and imaging evidence of tumor progression were randomized in a double-blinded fashion to receive tipifarnib (200 mg/m2 orally every 12 h) or placebo (phase A) and crossed over to the opposite treatment arm at the time of tumor progression (phase B). PN volumes were measured with MRI, and progression was defined as ≥20% volume …


Spirituality And Health, Arndt Bussing, Klaus Baumann, Niels Christian Hvidt, Harold G. Koenig, Christina M. Puchalski, John Swinton Jan 2014

Spirituality And Health, Arndt Bussing, Klaus Baumann, Niels Christian Hvidt, Harold G. Koenig, Christina M. Puchalski, John Swinton

Medicine Faculty Publications

No abstract provided.


Testing The Prognostic Accuracy Of The Updated Pediatric Sepsis Biomarker Risk Model, Hector R. Wong, Scott L. Weiss, John S. Giuliano Jr., Mark S. Wainwright, Robert J. Freishtat, +20 Additional Authors Jan 2014

Testing The Prognostic Accuracy Of The Updated Pediatric Sepsis Biomarker Risk Model, Hector R. Wong, Scott L. Weiss, John S. Giuliano Jr., Mark S. Wainwright, Robert J. Freishtat, +20 Additional Authors

Pediatrics Faculty Publications

Background

We previously derived and validated a risk model to estimate mortality probability in children with septic shock (PERSEVERE; PEdiatRic SEpsis biomarkEr Risk modEl). PERSEVERE uses five biomarkers and age to estimate mortality probability. After the initial derivation and validation of PERSEVERE, we combined the derivation and validation cohorts (n = 355) and updated PERSEVERE. An important step in the development of updated risk models is to test their accuracy using an independent test cohort.

Objective

To test the prognostic accuracy of the updated version PERSEVERE in an independent test cohort.

Methods

Study subjects were recruited from multiple pediatric intensive …


Zinc Deficiency-Associated Dermatitis In Infants During A Nationwide Shortage Of Injectable Zinc--Washington, Dc, And Houston, Texas 2012-2013, Duke Ruktanonchai, Michael Lowe, Scott A. Norton, Tiana Garrett, Lamia Soghier, Edward Weiss, June Hatfield, Jeffrey Lapinski, Steven Abrams, Wanda Barfield Jan 2014

Zinc Deficiency-Associated Dermatitis In Infants During A Nationwide Shortage Of Injectable Zinc--Washington, Dc, And Houston, Texas 2012-2013, Duke Ruktanonchai, Michael Lowe, Scott A. Norton, Tiana Garrett, Lamia Soghier, Edward Weiss, June Hatfield, Jeffrey Lapinski, Steven Abrams, Wanda Barfield

Pediatrics Faculty Publications

No abstract provided.


An Efficient, Free-Breathing Protocolf For Mr Right Heart Catheterization, Jonathan R. Mazal, Toby Rogers, Anthony Z. Faranesh, Peter Kellman, William H. Schenke, Annette M. Stine, Laurie P. Grant, Kanishka Ratnayaka, Robert J. Lederman Jan 2014

An Efficient, Free-Breathing Protocolf For Mr Right Heart Catheterization, Jonathan R. Mazal, Toby Rogers, Anthony Z. Faranesh, Peter Kellman, William H. Schenke, Annette M. Stine, Laurie P. Grant, Kanishka Ratnayaka, Robert J. Lederman

Pediatrics Posters and Presentations

No abstract provided.


Technologist Primer For Mri Right Heart Catheterization: The Nih And Cnmc Experience, Jonathan R. Mazal, Kendall O'Brien, William Schenke, Annette Stine, Laurie Grant, Anthony Z. Faranesh, Kanishka Ratnayaka, Robert J. Lederman Jan 2014

Technologist Primer For Mri Right Heart Catheterization: The Nih And Cnmc Experience, Jonathan R. Mazal, Kendall O'Brien, William Schenke, Annette Stine, Laurie Grant, Anthony Z. Faranesh, Kanishka Ratnayaka, Robert J. Lederman

Pediatrics Posters and Presentations

No abstract provided.


Sodium-Coupled Neutral Amino Acid Transporter 1 (Snat1) Modulates L-Citrulline Transport And Nitric Oxide (No) Signaling In Piglet Pulmonary Arterial Endothelial Cells, Anna Dikalova, Angela Fagiana, Judy L. Aschner, Michael Aschner, Marshall Summar, Candice D. Fike Jan 2014

Sodium-Coupled Neutral Amino Acid Transporter 1 (Snat1) Modulates L-Citrulline Transport And Nitric Oxide (No) Signaling In Piglet Pulmonary Arterial Endothelial Cells, Anna Dikalova, Angela Fagiana, Judy L. Aschner, Michael Aschner, Marshall Summar, Candice D. Fike

Pediatrics Faculty Publications

Rationale

There is evidence that impairments in nitric oxide (NO) signaling contribute to chronic hypoxia-induced pulmonary hypertension. The L-arginine-NO precursor, L-citrulline, has been shown to ameliorate pulmonary hypertension. Sodium-coupled neutral amino acid transporters (SNATs) are involved in the transport of L-citrulline into pulmonary arterial endothelial cells (PAECs). The functional link between the SNATs, L-citrulline, and NO signaling has not yet been explored.

Objective

We tested the hypothesis that changes in SNAT1 expression and transport function regulate NO production by modulating eNOS coupling in newborn piglet PAECs.

Methods and Results

A silencing RNA (siRNA) technique was used to assess the contribution …


Factitious Psychogenic Nonepileptic Paroxysmal Episodes, Alissa Romano, Saeed Alqahtani, James L. Griffith, Mohamad Z. Koubeissi Jan 2014

Factitious Psychogenic Nonepileptic Paroxysmal Episodes, Alissa Romano, Saeed Alqahtani, James L. Griffith, Mohamad Z. Koubeissi

Psychiatry and Behavioral Sciences Faculty Publications

Mistaking psychogenic nonepileptic paroxysmal episodes (PNEPEs) for epileptic seizures (ES) is potentially dangerous, and certain features should alert physicians to a possible PNEPE diagnosis. Psychogenic nonepileptic paroxysmal episodes due to factitious seizures carry particularly high risks of morbidity or mortality from nonindicated emergency treatment and, often, high costs in wasted medical treatment expenditures. We report a case of a 28-year-old man with PNEPEs that were misdiagnosed as ES. The patient had been on four antiseizure medications (ASMs) with therapeutic serum levels and had had multiple intubations in the past for uncontrolled episodes. He had no episodes for two days of …


Multiple Epidural Steroid Injections And Body Mass Index Linked With Occurrence Of Epidural Lipomatosis: A Case Series., Rafael Jaimes, Angelo G. Rocco Jan 2014

Multiple Epidural Steroid Injections And Body Mass Index Linked With Occurrence Of Epidural Lipomatosis: A Case Series., Rafael Jaimes, Angelo G. Rocco

Orthopaedic Surgery Faculty Publications

BACKGROUND:

Epidural lipomatosis (EL) is an increase of adipose tissue, normally occurring in the epidural space, sufficient to distort the thecal sac and compress neural elements. There is a lack of knowledge of risk factors, impact on patient's symptoms, and its possible association with epidural steroid injections.

METHODS:

History, physical examination, patient chart, and MRI were analyzed from 856 outpatients referred for epidural steroid injections. Seventy patients with signs of EL on MRI comprised the study group. Thirty-four randomly selected patients comprised the control group. The severity of EL was determined by the MRI assessment. The impact of EL was …


High Levels Of Dek Autoantibodies May Predict Early Flare Following Cessation Of Anti-Tnf Therapy In Juvenile Idiopathic Arthritis, Nirit Mor-Vaknin, Miguel Rivas, Maureen Legendre, Y. Yuanfang, Anne Johnson, Lawrence K. Jung, +12 Additional Authors Jan 2014

High Levels Of Dek Autoantibodies May Predict Early Flare Following Cessation Of Anti-Tnf Therapy In Juvenile Idiopathic Arthritis, Nirit Mor-Vaknin, Miguel Rivas, Maureen Legendre, Y. Yuanfang, Anne Johnson, Lawrence K. Jung, +12 Additional Authors

Pediatrics Faculty Publications

Introduction The nuclear oncoprotein DEK is a biochemically distinct, pro-inflammatory protein that is a chemoattractant for neutrophils and T-cells. High levels of DEK autoantibodies have been found in several autoimmune diseases including juvenile idiopathic arthritis (JIA), but their role in disease pathogenesis is unclear.

Objectives Since DEK and DEK autoantibodies can contribute to the development of immune complexes and joint inflammation, we suggest that DEK antibody levels can predict disease flare with discontinuation of anti-TNF therapy. Methods In 16 pediatric rheumatology centers, sera samples were collected from 137 children with polyarticular JIA on anti-TNF therapy. Therapy was stopped after 6 …