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https://www.readbyqxmd.com/read/28938315/proteus-syndrome-with-a-cranial-intraosseous-lipoma
#1
Erik M Wolfswinkel, Thomas A Imahiyerobo, J Gordon McComb, Pedro A Sanchez-Lara, Mark M Urata
Intraosseous lipomas are almost exclusively seen in the long bones. Presence in the craniofacial skeleton is extremely rare. A 7-year-old male is presented with a marked craniofacial deformation from a bony tumor containing an intraosseous lipoma. This finding established a clinical diagnosis of Proteus syndrome. Given the size of the tumor, producing an extensive deformity, three-dimensional modeling was used to generate a three-dimensional printed implant. The process to achieve a successful outcome is herein described...
September 21, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28931508/corticosteroids-for-treatment-of-sore-throat-systematic-review-and-meta-analysis-of-randomised-trials
#2
Behnam Sadeghirad, Reed A C Siemieniuk, Romina Brignardello-Petersen, Davide Papola, Lyubov Lytvyn, Per Olav Vandvik, Arnaud Merglen, Gordon H Guyatt, Thomas Agoritsas
Objective To estimate the benefits and harms of using corticosteroids as an adjunct treatment for sore throat.Design Systematic review and meta-analysis of randomised control trials.Data sources Medline, Embase, Cochrane Central Register of Controlled Trials (CENTRAL), trial registries up to May 2017, reference lists of eligible trials, related reviews.Study selection Randomised controlled trials of the addition of corticosteroids to standard clinical care for patients aged 5 or older in emergency department and primary care settings with clinical signs of acute tonsillitis, pharyngitis, or the clinical syndrome of sore throat...
September 20, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28910146/management-of-ards-and-refractory-hypoxemia-a-multicenter-observational-study
#3
Erick H Duan, Neill Kj Adhikari, Frederick D'Aragon, Deborah J Cook, Sangeeta Mehta, Waleed Alhazzani, Ewan Goligher, Emmanuel Charbonney, Yaseen M Arabi, Tim Karachi, Alexis F Turgeon, Lori Hand, Qi Zhou, Peggy Austin, Jan Friedrich, Francois Lamontagne, François Lauzier, Rakesh Patel, John Muscedere, Richard Hall, Pierre Aslanian, Thomas Piraino, Martin Albert, Sean M Bagshaw, Mike Jacka, Gordon Wood, William Henderson, Delbert Dorscheid, Niall D Ferguson, Maureen O Meade
RATIONALE: Clinicians' current practice patterns in the management of acute respiratory distress syndrome (ARDS) and refractory hypoxemia are not well described. OBJECTIVES: To describe mechanical ventilation strategies and treatment adjuncts for adults with ARDS including refractory hypoxemia. METHODS: Prospective cohort study (March 2014-February 2015) of mechanically ventilated adults with moderate-to-severe ARDS requiring FiO2 ≥0.50 in 24 ICUs...
September 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28882065/annals-express-serum-high-sensitive-c-reactive-protein-is-associated-with-dietary-intakes-in-diabetic-patients-with-and-without-hypertension-a-cross-sectional-study
#4
Mohammad Bagherniya, Sayyed Saeid Khayyatzadeh, Ali Reza Heidari-Bakavoli, Gordon Ferns, Mahmoud Ebrahimi, Mohammad Safarian, Mohsen Nematy, Majid Ghayour-Mobarhan
BACKGROUND: Serum high sensitive C-reactive protein (hs-CRP) concentrations independently predict the development of diabetes, metabolic syndrome and cardiovascular disease. However, the impact of dietary factors on serum hs-CRP concentrations in diabetic patients has received limited attention. We aimed to investigate the association between dietary factors and hs-CRP, among diabetic patients with and without hypertension and healthy subjects. METHODS: In this cross-sectional study, diabetics with (n=325) and without hypertension (n=599) and healthy individuals (n=1220) were recruited in Mashhad, Iran...
January 1, 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/28861031/visual-dysfunction-in-posterior-cortical-atrophy
#5
REVIEW
Mari N Maia da Silva, Rebecca S Millington, Holly Bridge, Merle James-Galton, Gordon T Plant
Posterior cortical atrophy (PCA) is a syndromic diagnosis. It is characterized by progressive impairment of higher (cortical) visual function with imaging evidence of degeneration affecting the occipital, parietal, and posterior temporal lobes bilaterally. Most cases will prove to have Alzheimer pathology. The aim of this review is to summarize the development of the concept of this disorder since it was first introduced. A critical discussion of the evolving diagnostic criteria is presented and the differential diagnosis with regard to the underlying pathology is reviewed...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28840615/the-effects-of-curcumin-and-curcumin-phospholipid-complex-on-the-serum-pro-oxidant-antioxidant-balance-in-subjects-with-metabolic-syndrome
#6
Maryam Ghazimoradi, Maryam Saberi-Karimian, Farzane Mohammadi, Amirhossein Sahebkar, Shima Tavallaie, Hamideh Safarian, Gordon A Ferns, Majid Ghayour-Mobarhan, Mohsen Moohebati, Habibollah Esmaeili, Malihe Ahmadinejad
Metabolic syndrome (MetS) is defined by a clustering of metabolic and anthropometric abnormalities and is associated by an increased risk of cardiovascular disease. We have investigated the effect of curcumin supplementation on the serum pro-oxidant-antioxidant balance (PAB) in patients with MetS. This double-blind, randomized, placebo-controlled trial was conducted over 6 weeks. Subjects (n = 120) were randomly allocated to one of three groups (curcumin, phospholipidated curcumin, and placebo). The curcumin group received 1 g/day of simple curcumin, the phospholipidated curcumin group received 1 g/day of phospholipidated curcumin (containing 200 mg of pure curcumin), and the control group received 1 g/day of placebo...
August 25, 2017: Phytotherapy Research: PTR
https://www.readbyqxmd.com/read/28816424/cover-image-volume-173a-number-9-september-2017
#7
Paul Kruszka, Antonio R Porras, Yonit A Addissie, Angélica Moresco, Sofia Medrano, Gary T K Mok, Gordon K C Leung, Cedrik Tekendo-Ngongang, Annette Uwineza, Meow-Keong Thong, Premala Muthukumarasamy, Engela Honey, Ekanem N Ekure, Ogochukwu J Sokunbi, Nnenna Kalu, Kelly L Jones, Julie D Kaplan, Omar A Abdul-Rahman, Lisa M Vincent, Amber Love, Khadija Belhassan, Karim Ouldim, Ihssane El Bouchikhi, Anju Shukla, Katta M Girisha, Siddaramappa J Patil, Nirmala D Sirisena, Vajira H W Dissanayake, C Sampath Paththinige, Rupesh Mishra, Eva Klein-Zighelboim, Bertha E Gallardo Jugo, Miguel Chávez Pastor, Hugo H Abarca-Barriga, Steven A Skinner, Eloise J Prijoles, Eben Badoe, Ashleigh D Gill, Vorasuk Shotelersuk, Patroula Smpokou, Monisha S Kisling, Carlos R Ferreira, Leon Mutesa, Andre Megarbane, Antonie D Kline, Amy Kimball, Emmy Okello, Peter Lwabi, Twalib Aliku, Emmanuel Tenywa, Nonglak Boonchooduang, Pranoot Tanpaiboon, Antonio Richieri-Costa, Ambroise Wonkam, Brian H Y Chung, Roger E Stevenson, Marshall Summar, Kausik Mandal, Shubha R Phadke, María G Obregon, Marius G Linguraru, Maximilian Muenke
The cover image, by Paul Kruszka et al., is based on the Original Article Noonan Syndrome in Diverse Populations, DOI: 10.1002/ajmg.a.38362. Design Credit: Darryl Leja.
September 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28790973/malignant-cerebellar-edema-subsequent-to-accidental-prescription-opioid-intoxication-in-children
#8
Daniel Duran, Robert D Messina, Lauren A Beslow, Julio D Montejo, Jason K Karimy, Charuta Gavankar Furey, Alison D Sheridan, Gordon Sze, Yanki Yarman, Michael L DiLuna, Kristopher T Kahle
We present two recent cases of toddlers who developed malignant cerebellar edema subsequent to accidental ingestion of prescription opioids. Both children presented acute neurological decline, hydrocephalus, and tonsillar herniation requiring emergent ventricular drain placement, suboccipital craniectomy, and partial cerebellectomy. Together with several other reports, these cases suggest the existence of an uncommon yet severe syndrome of acute opioid-induced malignant cerebellar edema. We hypothesize that the condition results from a combination of primary opioid receptor-mediated changes in neuronal metabolism that are exacerbated by secondary hypoxic insult...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28783043/nbeal2-is-required-for-neutrophil-and-nk-cell-function-and-pathogen-defense
#9
John M Sowerby, David C Thomas, Simon Clare, Marion Espéli, Jose A Guerrero, Kim Hoenderdos, Katherine Harcourt, Morgan Marsden, Juneid Abdul-Karim, Mathew Clement, Robin Antrobus, Yagnesh Umrania, Philippa R Barton, Shaun M Flint, Jatinder K Juss, Alison M Condliffe, Paul A Lyons, Ian R Humphreys, Edwin R Chilvers, Willem H Ouwehand, Gordon Dougan, Kenneth Gc Smith
Mutations in the human NBEAL2 gene cause gray platelet syndrome (GPS), a bleeding diathesis characterized by a lack of α granules in platelets. The functions of the NBEAL2 protein have not been explored outside platelet biology, but there are reports of increased frequency of infection and abnormal neutrophil morphology in patients with GPS. We therefore investigated the role of NBEAL2 in immunity by analyzing the phenotype of Nbeal2-deficient mice. We found profound abnormalities in the Nbeal2-deficient immune system, particularly in the function of neutrophils and NK cells...
August 7, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28782660/evaluation-and-management-of-primary-ovarian-insufficiency-in-adolescents-and-young-adults
#10
Rula V Kanj, Nana Ama Ofei-Tenkorang, Mekibib Altaye, Catherine M Gordon
STUDY OBJECTIVE: To identify clinical features associated with primary ovarian insufficiency (POI) and collect data on the evaluation and treatment received. DESIGN: Retrospective chart review. Data abstracted on etiology of POI, history, laboratory evaluation, imaging results, return for clinical care, and treatment plans. SETTING: Urban children's hospital in Cincinnati, Ohio. PARTICIPANT: s: 50 females, age 11-26 years, with initial presentation of POI between January 1, 2006-December 31, 2015...
August 3, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28777195/prevalence-and-prognostic-association-of-circulating-troponin-in-the-acute-respiratory-distress-syndrome
#11
Thomas S Metkus, Eliseo Guallar, Lori Sokoll, David Morrow, Gordon Tomaselli, Roy Brower, Steven Schulman, Frederick K Korley
OBJECTIVE: Circulating cardiac troponin has been associated with adverse prognosis in the acute respiratory distress syndrome in small and single-center studies; however, comprehensive studies of myocardial injury in acute respiratory distress syndrome using modern high-sensitivity troponin assays, which can detect troponin at much lower circulating concentrations, have not been performed. DESIGN: We performed a prospective cohort study. SETTING: We included patients enrolled in previously completed trials of acute respiratory distress syndrome...
October 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28770579/dietary-behaviors-in-relation-to-prevalence-of-irritable-bowel-syndrome-in-adolescent-girls
#12
Sayyed Saeid Khayyatzadeh, Seyyed Mohammad Reza Kazemi-Bajestani, Seyed Jamal Mirmousavi, Masoud Heshmati, Somaieh Khoshmohabbat, Gordon A Ferns, Majid Ghayour-Mobarhan
BACKGROUNDS AND AIMS: There is limited evidence regarding the relationship between dietary behaviors and irritable bowel syndrome (IBS). This study aimed to explore the association between diet-related practices and prevalence of IBS. METHODS: The study was conducted among 988 adolescent girls living in Iran. Dietary behaviors were pre-defined and assessed in nine domains using a pre-tested questionnaire. To investigate the association between diet-related practices and the presence of IBS, we used logistic regression analysis in crude and adjusted models...
August 2, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28765416/the-physician-s-role-in-perioperative-management-of-older-patients-undergoing-surgery
#13
Adam L Gordon, Barry J Evans, Jugdeep Dhesi
Life-sustaining and life-improving surgical interventions are increasingly available to older, frailer patients, many of whom have multimorbidity. Physicians can help support perioperative multidisciplinary teams with assessment and preoperative optimisation of physiological reserve, comorbidities and associated geriatric syndromes. Similar structured support can be useful in the postoperative period where older patients are at increased risk of delirium, medical complications, increased functional dependency and where discharge planning can prove more difficult than in younger cohorts...
July 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/28756152/ophthalmologic-features-of-progeria
#14
Iason S Mantagos, Monica E Kleinman, Mark W Kieran, Leslie B Gordon
PURPOSE: To establish the natural history of ophthalmic characteristics in Progeria patients and to determine incidence of ocular manifestations. DESIGN: Retrospective case series of patients with Progeria who were seen between 2007 and 2016. METHODS: Setting: Tertiary-care academic center. PATIENT POPULATION: Fourteen patients (28 eyes) with Hutchinson-Gilford Progeria syndrome were included for statistical analysis from a total of 84 patients who have been enrolled in clinical trials for Progeria at Boston Children's Hospital...
July 27, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28748642/noonan-syndrome-in-diverse-populations
#15
Paul Kruszka, Antonio R Porras, Yonit A Addissie, Angélica Moresco, Sofia Medrano, Gary T K Mok, Gordon K C Leung, Cedrik Tekendo-Ngongang, Annette Uwineza, Meow-Keong Thong, Premala Muthukumarasamy, Engela Honey, Ekanem N Ekure, Ogochukwu J Sokunbi, Nnenna Kalu, Kelly L Jones, Julie D Kaplan, Omar A Abdul-Rahman, Lisa M Vincent, Amber Love, Khadija Belhassan, Karim Ouldim, Ihssane El Bouchikhi, Anju Shukla, Katta M Girisha, Siddaramappa J Patil, Nirmala D Sirisena, Vajira H W Dissanayake, C Sampath Paththinige, Rupesh Mishra, Eva Klein-Zighelboim, Bertha E Gallardo Jugo, Miguel Chávez Pastor, Hugo H Abarca-Barriga, Steven A Skinner, Eloise J Prijoles, Eben Badoe, Ashleigh D Gill, Vorasuk Shotelersuk, Patroula Smpokou, Monisha S Kisling, Carlos R Ferreira, Leon Mutesa, Andre Megarbane, Antonie D Kline, Amy Kimball, Emmy Okello, Peter Lwabi, Twalib Aliku, Emmanuel Tenywa, Nonglak Boonchooduang, Pranoot Tanpaiboon, Antonio Richieri-Costa, Ambroise Wonkam, Brian H Y Chung, Roger E Stevenson, Marshall Summar, Kausik Mandal, Shubha R Phadke, María G Obregon, Marius G Linguraru, Maximilian Muenke
Noonan syndrome (NS) is a common genetic syndrome associated with gain of function variants in genes in the Ras/MAPK pathway. The phenotype of NS has been well characterized in populations of European descent with less attention given to other groups. In this study, individuals from diverse populations with NS were evaluated clinically and by facial analysis technology. Clinical data and images from 125 individuals with NS were obtained from 20 countries with an average age of 8 years and female composition of 46%...
July 27, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28738749/an-update-on-multiple-sclerosis-in-children-diagnosis-therapies-and-prospects-for-the-future
#16
Eliza Gordon-Lipkin, Brenda Banwell
Multiple sclerosis (MS), a chronic demyelinating disease of the central nervous system, is increasingly being recognized in children and adolescents. Pediatric MS follows a relapsing-remitting course at onset, with a risk for early cognitive impairment. Areas covered: In this review, we discuss the clinical features of acute demyelinating syndromes in children and risk factors that increase the likelihood of a diagnosis of MS. We also address the application of diagnostic criteria for MS in children, immunological features, therapeutic options and psychosocial considerations for children and adolescents with MS...
October 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28730607/concurrent-chondrodysplasia-punctata-type-2-conradi-hunermann-happle-syndrome-and-ichthyosis-vulgaris-in-teenaged-twin-girls
#17
Haneol S Jeong, Tara Funari, Katherine Gordon, Gabriele Richard, Nnenna G Agim
We present concurrent X-linked chondrodysplasia punctata and ichthyosis vulgaris in adolescent fraternal twin girls, notable for initial presentation with dry skin in adolescence, characterized by dark-brown scale typical of ichthyosis vulgaris and blaschkolinear, atrophic, scaly plaques. This constellation of findings prompted further genetic investigation. Using a multigene approach to examine 39 genes associated with congenital ichthyosis, next-generation sequencing revealed a novel heterozygous missense mutation at a mutational hotspot in the EBP gene c...
July 21, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28708421/relationship-between-race-and-the-effect-of-fluids-on-long-term-mortality-after-acute-respiratory-distress-syndrome-secondary-analysis-of-the-nhlbi-fluid-and-catheter-treatment-trial
#18
Sarah E Jolley, Catherine L Hough, Gilles Clermont, Douglas Hayden, Suqin Hou, David Schoenfeld, Nicholas L Smith, Boyd Taylor Thompson, Gordon R Bernard, Derek C Angus
RATIONALE: Short-term follow up in the Fluid and Catheter Treatment Trial (FACTT) suggested differential mortality by race with conservative fluid management, but no significant interaction. OBJECTIVE: In a post-hoc analysis of FACTT including one-year follow up, we sought to estimate long-term mortality by race and test for an interaction between fluids and race. METHODS: Post-hoc analysis of FACTT and the Economic Analysis of Pulmonary Artery Catheters (EAPAC) study (included 655 of the 1000 FACTT patients with near complete one-year follow up)...
July 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28696129/gender-dysphoria-in-klinefelter-s-syndrome-three-cases
#19
Gordon W Davies, John Parkinson
BACKGROUND: Previous reports have found the incidence of gender dysphoria in Klinefelter's patients greater than in the general male population. METHODS: A cohort of patients with gender dysphoria was reviewed. RESULTS: Of the 220 patients with gender dysphoria, three had Klinefelter's syndrome. CONCLUSIONS: These three reports are further examples of gender dysphoria in Klinefelter's syndrome. The role of biological factors in gender identity is affirmed...
July 1, 2017: Australasian Psychiatry: Bulletin of Royal Australian and New Zealand College of Psychiatrists
https://www.readbyqxmd.com/read/28690029/high-levels-of-soluble-gpr56-adgrg1-are-associated-with-positive-rheumatoid-factor-and-elevated-tumor-necrosis-factor-in-patients-with-rheumatoid-arthritis
#20
Wen-Yi Tseng, Yeong-Jian Jan Wu, Tai-Yun Yang, Nien-Yi Chiang, Wen-Pin Tsai, Siamon Gordon, Gin-Wen Chang, Chang-Fu Kuo, Shue-Fen Luo, Hsi-Hsien Lin
BACKGROUND: GPR56/ADGRG1 is a member of the adhesion-class G protein-coupled receptor (aGPCR) family important in brain development, oncogenesis and tumor metastasis. Like other aGPCRs, GPR56 is cleaved at the GPCR proteolysis site (GPS) motif into an N-terminal fragment (NTF) and a C-terminal fragment (CTF). Existence of soluble GPR56 (sGPR56) has been shown in vitro, however the underlying mechanism and its pathophysiologic role remains undetermined. OBJECTIVE: To assess the presence of sGPR56 in human serum using ELISA assay and compare the serum sGPR56 levels among patients of various chronic inflammatory diseases and healthy subjects...
June 28, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
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