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https://www.readbyqxmd.com/read/28523047/alpha-thalassemia-deletions-found-in-suspected-cases-of-beta-thalassemia-major-in-pakistani-population
#1
Saba Shahid, Muhammad Nadeem, Danish Zahid, Jawad Hassan, Saqib Ansari, Tahir Shamsi
BACKGROUND & OBJECTIVE: Alpha (α) thalassemia is a hereditary disorder and is caused by deletions or mutations in globin genes. It is present in two clinically significant forms: hemoglobin Bart hydrops fetalis (Hb Bart) syndrome and hemoglobin H (HbH) disease. It is highly prevalent in South-East Asia or Mediterranean countries. The most common deletion reported in alpha thalassemia in Pakistani population was -α(3.7) with a frequency of 8.3%, and the rare forms were -α(4.2) (0.2%) and ααα(anti3...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28507920/passenger-lymphocyte-syndrome-pls-a-single-center-retrospective-analysis-of-minor-abo-incompatible-liver-transplants
#2
Sévérine de Bruijn, Ester Philipse, Marie Madeleine Couttenye, Bart Bracke, Dirk Ysebaert, Peter Michielsen, Sven Francque, Thomas Vanwolleghem, Anke Verlinden
Background and Aims: Due to the shortage of donor livers, minor ABO-incompatible liver transplantations are commonly performed. Together with the allograft, immunocompetent B-lymphocytes, called passenger lymphocytes, are transplanted. In case of minor ABO-incompatibility, these passenger lymphocytes produce antibodies directed towards the recipient's red blood cells, which causes immune-mediated hemolysis, also known as the passenger lymphocyte syndrome (PLS). Although this is a self-limiting disorder, serious complications can occur, including graft failure...
March 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/28505236/could-albumin-be-a-biomarker-to-monitor-the-effect-of-intravenous-immunoglobulin-on-guillain-barr%C3%A3-syndrome-reply
#3
Willem-Jan R Fokkink, Bart C Jacobs
No abstract text is available yet for this article.
May 15, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28502071/update-from-the-abdominal-compartment-society-wsacs-on-intra-abdominal-hypertension-and-abdominal-compartment-syndrome-past-present-and-future-beyond-banff-2017
#4
Andrew W Kirkpatrick, Michael Sugrue, Jessica L McKee, Bruno M Pereira, Derek J Roberts, Jan J De Waele, Ari Leppaniemi, Janeth C Ejike, Annika Reintam Blaser, Scott D'Amours, Bart De Keulenaer, Manu L N G Malbrain
No abstract text is available yet for this article.
May 14, 2017: Anaesthesiology Intensive Therapy
https://www.readbyqxmd.com/read/28501428/management-of-ovarian-stimulation-for-ivf-narrative-review-of-evidence-provided-for-world-health-organization-guidance
#5
REVIEW
Cindy Farquhar, Jane Marjoribanks, Julie Brown, Bart C J M Fauser, Anne Lethaby, Selma Mourad, Robert Rebar, Marian Showell, Sheryl van der Poel
In this paper, a review of evidence provided to the World Health Organization (WHO) guideline development, who prepare global guidance on the management of ovarian stimulation for women undergoing IVF, is presented. The purpose of ovarian stimulation is to facilitate retrieval of multiple oocytes during a single IVF cycle. Availability of multiple oocytes compensates for inefficiencies in subsequent stages of the cycle, which include oocyte maturation, IVF, embryo culture, embryo transfer, and implantation...
April 26, 2017: Reproductive Biomedicine Online
https://www.readbyqxmd.com/read/28486600/sclt1-deficiency-causes-cystic-kidney-by-activating-erk-and-stat3-signaling
#6
Jianshuang Li, Di Lu, Huadie Liu, Bart O Williams, Paul A Overbeek, Brendan Lee, Ling Zheng, Tao Yang
Ciliopathies form a group of inherited disorders sharing several clinical manifestations due to abnormal cilia formation or function, and few treatments have been successful against these disorders. Here, we report a mouse model with mutated Sclt1 gene, which encodes a centriole distal appendage protein important for ciliogenesis. SCLT1 mutations were associated with the oral-facial-digital syndrome (OFD), an autosomal recessive ciliopathy. The Sclt1-/- mice exhibit typical ciliopathy phenotypes, including cystic kidney, cleft palate, and polydactyly...
May 9, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28457019/dynamics-of-von-willebrand-factor-reactivity-in-sickle-cell-disease-during-vaso-occlusive-crisis-and-steady-state
#7
Joep W R Sins, Marein Schimmel, Brenda M Luken, Erfan Nur, Sacha S Zeerleder, Charlotte F J van Tuijn, Dees P M Brandjes, Wil F Kopatz, Rolf T Urbanus, Joost C M Meijers, Bart J Biemond, Karin Fijnvandraat
BACKGROUND: Endothelial activation plays a central role in the pathophysiology of vaso-occlusion in sickle cell disease (SCD), facilitating adhesive interactions with circulating blood cells. Upon activation various adhesive molecules are expressed, including von Willebrand factor (VWF). Increased VWF levels have been observed in patients with SCD during steady state. However, the role of VWF in the pathogenesis of SCD vaso-occlusion is unclear. OBJECTIVES: To longitudinally assess quantity and reactivity of VWF and its regulating protease ADAMTS-13 during vaso-occlusive crisis (VOC)...
April 29, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28447405/high-mortality-from-guillain-barr%C3%A3-syndrome-in-bangladesh
#8
Tanveen Ishaque, Mohammad B Islam, Gulshan Ara, Hubert P Endtz, Quazi D Mohammad, Bart C Jacobs, Zhahirul Islam
Although Guillain-Barré syndrome (GBS) has higher incidence and poor outcome in Bangladesh, mortality from GBS in Bangladesh has never been explored before. We sought to explore the frequency, timing and risk factors for deaths from GBS in Bangladesh. We conducted a prospective study on 407 GBS patients who were admitted to Dhaka Medical College Hospital, Dhaka, Bangladesh from 2010 to 2013. We compared deceased and alive patients to identify risk factors. Cox regression model was used to adjust for confounders...
April 26, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28446791/tissue-distribution-of-the-mers-coronavirus-receptor-in-bats
#9
W Widagdo, Lineke Begeman, Debby Schipper, Peter R van Run, Andrew A Cunningham, Nils Kley, Chantal B Reusken, Bart L Haagmans, Judith M A van den Brand
Middle East respiratory syndrome coronavirus (MERS-CoV) has been shown to infect both humans and dromedary camels using dipeptidyl peptidase-4 (DPP4) as its receptor. The distribution of DPP4 in the respiratory tract tissues of humans and camels reflects MERS-CoV tropism. Apart from dromedary camels, insectivorous bats are suggested as another natural reservoir for MERS-like-CoVs. In order to gain insight on the tropism of these viruses in bats, we studied the DPP4 distribution in the respiratory and extra-respiratory tissues of two frugivorous bat species (Epomophorus gambianus and Rousettus aegyptiacus) and two insectivorous bat species (Pipistrellus pipistrellus and Eptesicus serotinus)...
April 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28441232/atriobronchial-fistula-complicated-by-septic-cerebral-air-emboli-after-pulmonary-vein-ablation
#10
David Velghe, Thomas Apers, Stefanie Devriendt, Ivo Deblier, Bart Hendriks, Rogier Nieuwendijk, Peter Rogiers
OBJECTIVE: To describe a case of an infected atriobronchial fistula as a late complication after pulmonary vein ablation, leading to septic air emboli and requiring urgent cardiac surgery. DATA SOURCES: Clinical observation. STUDY SELECTION: Case report. DATA EXTRACTION: Relevant clinical information. PubMed was searched for relevant literature. DATA SYNTHESIS: Given its high success and low complication rate, pulmonary vein isolation is expected to be increasingly performed worldwide...
April 22, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28432749/motor-nerve-excitability-after-childhood-guillain-barr%C3%A3-syndrome
#11
Judith Drenthen, Joyce Roodbol, Ellen M Maathuis, Coriene E Catsman-Berrevoets, Joleen H Blok, Marie-Claire Y de Wit, Bart C Jacobs
OBJECTIVE: To determine the residual motor nerve dysfunction after pediatric Guillain-Barré syndrome (GBS). METHODS: Observational cross-sectional cohort study in patients who previously developed GBS during childhood (<18 years). Ulnar motor nerve dysfunction was defined by compound motor action potential (CMAP) scan in patients after a follow-up of at least one year compared with age-matched healthy controls, in relation to clinical course and outcome. RESULTS: Thirty-seven persons previously diagnosed with GBS in childhood were included with a mean age at current examination of 20...
April 21, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28414465/topical-intestinal-aminoimidazole-agonists-of-g-protein-coupled-bile-acid-receptor-1-promote-glucagon-like-peptide-1-secretion-and-improve-glucose-tolerance
#12
Manuel Lasalle, Vanessa Hoguet, Nathalie Hennuyer, Florence Leroux, Catherine Piveteau, Loïc Belloy, Sophie Lestavel, Emmanuelle Vallez, Emilie Dorchies, Isabelle Duplan, Emmanuel Sevin, Maxime Culot, Fabien Gosselet, Rajaa Boulahjar, Adrien Herledan, Bart Staels, Benoit Deprez, Anne Tailleux, Julie Charton
The role of the G-protein-coupled bile acid receptor TGR5 in various organs, tissues, and cell types, specifically in intestinal endocrine L-cells and brown adipose tissue, has made it a promising therapeutical target in several diseases, especially type-2 diabetes and metabolic syndrome. However, recent studies have shown deleterious on-target effects of systemic TGR5 agonists. To avoid these systemic effects while stimulating glucagon-like peptide-1 (GLP-1) secreting enteroendocrine L-cells, we have designed TGR5 agonists with low intestinal permeability...
May 5, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28412285/middle-east-respiratory-syndrome-coronavirus-vaccines-current-status-and-novel-approaches
#13
REVIEW
Nisreen Ma Okba, V Stalin Raj, Bart L Haagmans
Middle East respiratory syndrome coronavirus (MERS-CoV) is a cause of severe respiratory infection in humans, specifically the elderly and people with comorbidities. The re-emergence of lethal coronaviruses calls for international collaboration to produce coronavirus vaccines, which are still lacking to date. Ongoing efforts to develop MERS-CoV vaccines should consider the different target populations (dromedary camels and humans) and the correlates of protection. Extending on our current knowledge of MERS, vaccination of dromedary camels to induce mucosal immunity could be a promising approach to diminish MERS-CoV transmission to humans...
April 13, 2017: Current Opinion in Virology
https://www.readbyqxmd.com/read/28406555/international-guillain-barr%C3%A3-syndrome-outcome-study-igos-protocol-of-a-prospective-observational-cohort-study-on-clinical-and-biological-predictors-of-disease-course-and-outcome-in-guillain-barr%C3%A3-syndrome
#14
Bart C Jacobs, Bianca van den Berg, Christine Verboon, Govindsinh Chavada, David R Cornblath, Kenneth C Gorson, Thomas Harbo, Hans-Peter Hartung, Richard A C Hughes, Susumu Kusunoki, Pieter A van Doorn, Hugh J Willison
Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy with a highly variable clinical presentation, course, and outcome. The factors that determine the clinical variation of GBS are poorly understood which complicates the care and treatment of individual patients. The protocol of the ongoing International GBS Outcome Study (IGOS), a prospective, observational, multi-centre cohort study that aims to identify the clinical and biological determinants and predictors of disease onset, subtype, course and outcome of GBS is presented here...
April 12, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28388693/a-role-for-glycolipid-biosynthesis-in-severe-fever-with-thrombocytopenia-syndrome-virus-entry
#15
Mary Jane Drake, Benjamin Brennan, Kenneth Briley, Stephen M Bart, Eric Sherman, Agnieszka M Szemiel, Madeleine Minutillo, Frederic D Bushman, Paul Bates
A novel bunyavirus was recently found to cause severe febrile illness with high mortality in agricultural regions of China, Japan, and South Korea. This virus, named severe fever with thrombocytopenia syndrome virus (SFTSV), represents a new group within the Phlebovirus genus of the Bunyaviridae. Little is known about the viral entry requirements beyond showing dependence on dynamin and endosomal acidification. A haploid forward genetic screen was performed to identify host cell requirements for SFTSV entry...
April 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28384077/american-telemedicine-association-telestroke-guidelines
#16
Bart M Demaerschalk, Jill Berg, Brian W Chong, Hartmut Gross, Karin Nystrom, Opeolu Adeoye, Lee Schwamm, Lawrence Wechsler, Sallie Whitchurch
The following telestroke guidelines were developed to assist practitioners in providing assessment, diagnosis, management, and/or remote consultative support to patients exhibiting symptoms and signs consistent with an acute stroke syndrome, using telemedicine communication technologies. Although telestroke practices may include the more broad utilization of telemedicine across the entire continuum of stroke care, with some even consulting on all neurologic emergencies, this document focuses on the acute phase of stroke, including both pre- and in-hospital encounters for cerebrovascular neurological emergencies...
May 2017: Telemedicine Journal and E-health: the Official Journal of the American Telemedicine Association
https://www.readbyqxmd.com/read/28381876/genetic-origin-of-%C3%AE-0-thalassemia-sea-deletion-in-southeast-asian-populations-and-application-to-accurate-prenatal-diagnosis-of-hb-bart-s-hydrops-fetalis-syndrome
#17
Wittaya Jomoui, Goonnapa Fucharoen, Kanokwan Sanchaisuriya, Patnaree Charoenwijitkul, Jitpanu Maneesarn, Xiangmin Xu, Supan Fucharoen
α(0)-thalassemia of SEA deletion (-(SEA)) is common among Southeast Asian and Chinese. Using haplotype and phylogenetic analyses, we examined the origin of this defect in Southeast Asian populations. Study was done on both normal and α(0)-thalassemia alleles in 3 ethnic groups including 96 Thai, 52 Laotian and 21 Cambodian. Five SNPs encompassing the (-(SEA)) including (rs3760053 T>G), (rs1211375 A>C), (rs3918352 A>G), (rs1203974 A>G) and (rs11248914 C>T) were examined using high-resolution melting assays...
April 6, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28380315/myeloablative-versus-reduced-intensity-hematopoietic-cell-transplantation-for-acute-myeloid-leukemia-and-myelodysplastic-syndromes
#18
Bart L Scott, Marcelo C Pasquini, Brent R Logan, Juan Wu, Steven M Devine, David L Porter, Richard T Maziarz, Erica D Warlick, Hugo F Fernandez, Edwin P Alyea, Mehdi Hamadani, Asad Bashey, Sergio Giralt, Nancy L Geller, Eric Leifer, Jennifer Le-Rademacher, Adam M Mendizabal, Mary M Horowitz, H Joachim Deeg, Mitchell E Horwitz
Purpose The optimal regimen intensity before allogeneic hematopoietic cell transplantation (HCT) is unknown. We hypothesized that lower treatment-related mortality (TRM) with reduced-intensity conditioning (RIC) would result in improved overall survival (OS) compared with myeloablative conditioning (MAC). To test this hypothesis, we performed a phase III randomized trial comparing MAC with RIC in patients with acute myeloid leukemia or myelodysplastic syndromes. Patients and Methods Patients age 18 to 65 years with HCT comorbidity index ≤ 4 and < 5% marrow myeloblasts pre-HCT were randomly assigned to receive MAC (n = 135) or RIC (n = 137) followed by HCT from HLA-matched related or unrelated donors...
April 10, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28375874/immune-reconstitution-inflammatory-syndrome-associated-with-toxoplasmic-encephalitis-in-hiv-infected-patients-a-multicenter-cohort-study
#19
Ward P H van Bilsen, Charlotte H S B van den Berg, Bart J A Rijnders, Kees Brinkman, Jan W Mulder, Luc B S Gelinck, Andy I M Hoepelman, Ferdinand W N M Wit, Diederik van de Beek, Jan M Prins
OBJECTIVES: To investigate the incidence and risk factors of immune reconstitution inflammatory syndrome (IRIS) associated with toxoplasmic encephalitis (TE) in patients starting cART. DESIGN: A historical multicenter cohort study. METHODS: We included all HIV-infected patients diagnosed with TE in six Dutch hospitals between 1996 and 2016. Diagnosis of TE-IRIS was made using predefined IRIS criteria. We distinguished paradoxical TE-IRIS (worsening of underlying treated infection) from unmasking TE-IRIS (unmasking of subclinical infection after start of cART)...
April 3, 2017: AIDS
https://www.readbyqxmd.com/read/28367854/development-of-allograft-cancer-after-lung-transplantation-a-case-report
#20
Esther I Schwarz, Bart Vrugt, Lars C Huber, Christian Murer, Mace M Schuurmans, Ilhan Inci, Christian Benden
INTRODUCTION: In contrast to skin cancer and lymphoproliferative disorders, de-novo lung allograft cancer is seldom reported after lung transplantation. CASE REPORT: A 19-year-old patient with severe pulmonary hypertension listed urgently for lung transplantation underwent successful bilateral lung transplant procedure receiving lungs from a 55-year-old donor with a smoking history of 30 pack years. After 3.5 years of lung transplantation, a locally advanced squamous cell carcinoma in the left lung allograft was diagnosed...
March 29, 2017: Annals of Thoracic and Cardiovascular Surgery
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