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https://www.readbyqxmd.com/read/29761123/zika-virus-and-guillain-barr%C3%A3-syndrome-in-bangladesh
#1
Corine H GeurtsvanKessel, Zhahirul Islam, Md Badrul Islam, Sandra Kamga, Nowshin Papri, David A M C van de Vijver, Chantal Reusken, Ramona Mogling, Astrid P Heikema, Israt Jahan, Florence K Pradel, Rebecca L Pavlicek, Quazi D Mohammad, Marion P G Koopmans, Bart C Jacobs, Hubert P Endtz
Objective: Previous studies have associated Guillain-Barré syndrome (GBS) with Zika virus (ZIKV) outbreaks in South America and Oceania. In Asia, ZIKV is known to circulate widely, but the association with Guillain-Barré syndrome is unclear. We investigated whether endemic ZIKV infection is associated with the development of GBS. Methods: A prospective study was conducted from 2011 to 2015 in Bangladesh. A total of 418 patients and 418 healthy family controls were included in the study...
May 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29751799/the-aging-lung-tissue-telomere-shortening-in-health-and-disease
#2
Stephanie Everaerts, Elise J Lammertyn, Dries S Martens, Laurens J De Sadeleer, Karen Maes, Aernoud A van Batenburg, Roel Goldschmeding, Coline H M van Moorsel, Lieven J Dupont, Wim A Wuyts, Robin Vos, Ghislaine Gayan-Ramirez, Naftali Kaminski, James C Hogg, Wim Janssens, Geert M Verleden, Tim S Nawrot, Stijn E Verleden, John E McDonough, Bart M Vanaudenaerde
BACKGROUND: Telomere shortening has been associated with several lung diseases. However, telomere length is generally measured in peripheral blood leucocytes rather than in lung tissue, where disease occurs. Consequently, telomere dynamics have not been established for the normal human lung nor for diseased lung tissue. We hypothesized an age- and disease-dependent shortening of lung tissue telomeres. METHODS: At time of (re-)transplantation or autopsy, 70 explant lungs were collected: from unused donors (normal, n = 13) and patients with cystic fibrosis (CF, n = 12), chronic obstructive pulmonary disease (COPD, n = 11), chronic hypersensitivity pneumonitis (cHP, n = 9), bronchiolitis obliterans syndrome (BOS) after prior transplantation (n = 11) and restrictive allograft syndrome (RAS) after prior transplantation (n = 14)...
May 11, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29732924/identification-and-characterization-of-a-novel-fbn1-gene-variant-in-an-extended-family-with-variable-clinical-phenotype-of-marfan-syndrome
#3
Mahmut Cerkez Ergoren, Burcu Turkgenc, Kerem Teralı, Orhan Rodopluu, Aline Verstraeten, Lut Van Laer, Gamze Mocan, Bart Loeys, Omer Tetik, Sehime G Temel
No abstract text is available yet for this article.
May 6, 2018: Connective Tissue Research
https://www.readbyqxmd.com/read/29722429/bart-s-syndrome-in-a-family-affected-three-consecutive-generations-with-mutation-c-6007g-a-in-col7a1
#4
Zhiming Chen, Wenbo Bu, Suying Feng, Hongsheng Wang
Bart's syndrome (BS), characterized by aplasia cutis congenita (ACC, also called congenital localized absence of skin) and epidermolysis bullosa (EB), is diagnosed clinically based on the disorder's unique signs and symptoms. We report the case of a family, three members of which presented with ACC at birth and one had blisters on the mucous membranes. The patient was treated conservatively with topical antibacterial ointment and wet gauze dressing. Periodic follow up showed complete healing with minimal scarring...
May 3, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29701807/evidence-that-environmental-and-familial-risks-for-psychosis-additively-impact-a-multidimensional-subthreshold-psychosis-syndrome
#5
Lotta-Katrin Pries, Sinan Guloksuz, Margreet Ten Have, Ron de Graaf, Saskia van Dorsselaer, Nicole Gunther, Christian Rauschenberg, Ulrich Reininghaus, Rajiv Radhakrishnan, Maarten Bak, Bart P F Rutten, Jim van Os
Background: The observed link between positive psychotic experiences (PE) and psychosis spectrum disorder (PSD) may be stronger depending on concomitant presence of PE with other dimensions of psychopathology. We examined whether the effect of common risk factors for PSD on PE is additive and whether the impact of risk factors on the occurrence of PE depends on the co-occurrence of other symptom dimensions (affective dysregulation, negative symptoms, and cognitive alteration). Method: Data from the Netherlands Mental Health Survey and Incidence Study 2 were used...
April 25, 2018: Schizophrenia Bulletin
https://www.readbyqxmd.com/read/29699897/development-of-regional-extracorporeal-life-support-system-the-importance-of-innovative-simulation-training
#6
Mateusz Puślecki, Marcin Ligowski, Marek Dąbrowski, Sebastian Stefaniak, Małgorzata Ładzińska, Aleksander Pawlak, Marcin Zieliński, Łukasz Szarpak, Bartłomiej Perek, Marek Jemielity
BACKGROUND: Despite advances in mechanical ventilation, severe acute respiratory distress syndrome (ARDS) is associated with high morbidity and mortality rates ranging from 30% to 60%. Extracorporeal Membrane Oxygenation (ECMO) can be used as a "bridge to recovery". ECMO is a complex network that provides oxygenation and ventilation and allows the lungs to rest and recover from respiratory failure, while minimizing iatrogenic ventilator-induced lung injury. In the critical care settings, ECMO is shown to improve survival rates and outcomes in patients with severe ARDS...
April 18, 2018: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29681958/personal-resources-and-satisfaction-with-life-in-marfan-syndrome-patients-with-aortic-pathology-and-in-abdominal-aortic-aneurysm-patients
#7
Michał-Goran Stanišić, Teresa Rzepa, Alicja Gawrońska, Przemysław Kubaszewski, Maciej Putowski, Sebastian Stefaniak, Bartłomiej Perek
Introduction: Whether or not the source of aortic pathology is Marfan syndrome (MFS) or other processes leading to development of abdominal aorta aneurysms (AAA), the awareness of pathology may lead to an emotional upset and low assessment of satisfaction with life. Aim: To assess, in regard to MFS patients with aortic pathology and to abdominal aortic aneurysm patients: 1) whether or not self-efficacy (SE) and health locus of control (HLoC) affect the patients' satisfaction with life; 2) whether the two groups of patients differ in terms of mental dispositions...
March 2018: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/29678852/whole-blood-pcr-for-rapid-screening-of-%C3%AE-0-thalassemia
#8
Phongsathorn Wichian, Supawadee Yamsri, Kanokwan Sanchaisuriya, Goonnapa Fucharoen, Supan Fucharoen
Hemoglobin Bart's hydrops fetalis (homozygous α0 -thalassemia) is the most severe form of thalassemia in the Southeast Asian population. Fetuses with this disorder almost always die in utero or shortly after birth. Screening of α0 -thalassemia carrier is therefore crucial. Currently, diagnosis of α0 -thalassemia genes is done by DNA-based analysis which relies on DNA extraction. We have developed a simple screening format based on whole blood PCR assay. The method was validated on 198 specimens and the results show 100% concordance with a conventional gap-PCR on DNA specimens...
March 2018: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/29671633/behavioral-problems-and-psychosocial-strengths-unique-factors-contributing-to-the-behavioral-profile-of-youth-with-down-syndrome
#9
Lisa M Dieleman, Sarah S W De Pauw, Bart Soenens, Geert Van Hove, Peter Prinzie
This study aimed to describe problem behaviors and psychosocial strengths, examine the problem-strength interrelations, and evaluate profiles of problems and strengths in youth with Down syndrome (DS). The community-based sample consisted of 67 parents of children with DS aged between 4 and 19 years. Parents reported about the developmental age (Vineland screener), behavioral problems (Child Behavior Checklist), and psychosocial strengths (Behavioral and Emotional Rating Scale) of their child. Results indicate that attention, social, and thought problems were most prevalent, whereas family involvement and receiving/expressing affection were identified as strengths...
May 2018: American Journal on Intellectual and Developmental Disabilities
https://www.readbyqxmd.com/read/29627773/clinical-outcome-of-guillain-barr%C3%A3-syndrome-after-prolonged-mechanical-ventilation
#10
Bianca van den Berg, Eline F Storm, Marcel J P Garssen, Patricia H Blomkwist-Markens, Bart C Jacobs
BACKGROUND: Patients with Guillain-Barré syndrome (GBS) may suffer from respiratory failure for months or longer. The aim of this study was to determine the frequency, clinical course and outcome of patients with GBS requiring prolonged mechanical ventilation (MV). METHODS: Prospectively collected data from 526 patients with GBS participating in previous trials were analysed to determine the frequency and duration of prolonged MV (longer than 2 months). In addition, a cross-sectional study was conducted in patients with GBS requiring MV to determine the clinical course and long-term outcome with the ability to walk unaided as primary endpoint...
April 7, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29626428/the-effectiveness-of-oral-pain-medication-and-corticosteroid-injections-for-carpal-tunnel-syndrome-a-systematic-review
#11
REVIEW
Bionka Ma Huisstede, Manon S Randsdorp, Janneke van den Brink, Thierry Pc Franke, Bart W Koes, Peter Hoogvliet
OBJECTIVE: To present an evidence-based overview of the effectiveness of oral pain medication and corticosteroid injections to treat carpal tunnel syndrome (CTS). DATA SOURCES: The Cochrane Library, PubMed, EMBASE, CINAHL, and PEDro were searched for relevant systematic reviews and randomized controlled trials (RCTs). DATA SELECTION AND EXTRACTION: Two reviewers independently selected the studies, extracted the data on pain (VAS), function or recovery and assessed the methodological quality...
April 4, 2018: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/29624575/montelukast-for-bronchiolitis-obliterans-syndrome-after-lung-transplantation-a-randomized-controlled-trial
#12
David Ruttens, Stijn E Verleden, Heleen Demeyer, Dirk E Van Raemdonck, Jonas Yserbyt, Lieven J Dupont, Bart M Vanaudenaerde, Robin Vos, Geert M Verleden
Bronchiolitis obliterans syndrome (BOS) remains the major problem which precludes long-term survival after lung transplantation. Previously, an open label pilot study from our group demonstrated a possible beneficial effect of montelukast in progressive BOS patients with low airway neutrophilia (<15%), and already on azithromycin treatment, in whom the further decline in pulmonary function was attenuated. This was, however, a non-randomized and non-placebo controlled trial. The study design is a single center, prospective, interventional, randomized, double blind, placebo-controlled trial, with a two arm parallel group design and an allocation ratio of 1:1...
2018: PloS One
https://www.readbyqxmd.com/read/29599668/selected-presentations-of-lip-enlargement-clinical-manifestation-and-differentiation
#13
REVIEW
Katarzyna J Błochowiak, Bartłomiej Kamiński, Henryk Witmanowski, Jerzy Sokalski
Lip enlargement may be an important symptom of either systemic or local diseases. On the basis of selected age-matched clinical cases we present the possible causes of lip swelling. We describe the most representative symptoms and recommend treatment of these pathologies. We differentiate lip swelling in Miescher syndrome, monosymptomatic form of Melkersson-Rosenthal syndrome, lip swelling in erythema multiforme and Stevens-Johnson syndrome and lip hemangioma and mucous extravasation cyst. We compare different causes of lip edema and indicate the most helpful diagnostic and treatment methods...
February 2018: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29593057/multicenter-evaluation-of-the-biofire-filmarray-respiratory-panel-2-for-the-detection-of-viruses-and-bacteria-in-nasopharyngeal-swab-samples
#14
Amy L Leber, Kathy Everhart, Judy A Daly, Aubrey Hopper, Amanda Harrington, Paul Schreckenberger, Kathleen McKinley, Matthew Jones, Kristen Holmberg, Bart Kensinger
The FilmArray® Respiratory Panel 2 (RP2) is a multiplex in vitro diagnostic test for the simultaneous and rapid (∼45 minutes) detection of 22 pathogens directly from nasopharyngeal swab (NPS) samples. It contains updated (and in some instances redesigned) assays that improve upon the FilmArray® Respiratory Panel (RP; version 1.7), with a faster run time. The organisms identified are adenovirus, coronavirus 229E, coronavirus HKU1, coronavirus NL63, coronavirus OC43, human metapneumovirus, human rhinovirus/enterovirus, influenza A, influenza A H1, influenza A H1-2009, influenza A H3, influenza B, parainfluenza virus 1, parainfluenza virus 2, parainfluenza virus 3, parainfluenza virus 4, respiratory syncytial virus, Bordetella pertussis , Chlamydia pneumoniae , and Mycoplasma pneumoniae...
March 28, 2018: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/29551664/type-iii-collagen-affects-dermal-and-vascular-collagen-fibrillogenesis-and-tissue-integrity-in-a-mutant-col3a1-transgenic-mouse-model
#15
Sanne D'hondt, Brecht Guillemyn, Delfien Syx, Sofie Symoens, Riet De Rycke, Leen Vanhoutte, Wendy Toussaint, Bart N Lambrecht, Anne De Paepe, Douglas R Keene, Yoshihiro Ishikawa, Hans Peter Bächinger, Sophie Janssens, Mathieu J M Bertrand, Fransiska Malfait
Type III collagen is a major fibrillar collagen consisting of three identical α1 (III)-chains that is particularly present in tissues exhibiting elastic properties, such as the skin and the arterial wall. Heterozygous mutations in the COL3A1 gene result in vascular Ehlers-Danlos syndrome (vEDS), a severe, life-threatening disorder, characterized by thin, translucent skin and propensity to arterial, intestinal and uterine rupture. Most human vEDS cases result from a missense mutation substituting a crucial glycine residue in the triple helical domain of the α1 (III)-chains...
March 15, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29452988/expert-consensus-recommendations-on-the-cardiogenetic-care-for-patients-with-thoracic-aortic-disease-and-their-first-degree-relatives
#16
Judith M A Verhagen, Marlies Kempers, Luc Cozijnsen, Berto J Bouma, Anthonie L Duijnhouwer, Jan G Post, Yvonne Hilhorst-Hofstee, Sebastiaan C A M Bekkers, Wilhelmina S Kerstjens-Frederikse, Thomas J van Brakel, Eric Lambermon, Marja W Wessels, Bart L Loeys, Jolien W Roos-Hesselink, Ingrid M B H van de Laar
BACKGROUND: Thoracic aortic aneurysm (TAA) is a potentially life-threatening disorder with a strong genetic component. The number of genes implicated in TAA has increased exponentially over the last decade. Approximately 20% of patients with TAA have a positive family history. As most TAA remain asymptomatic for a long time, screening of at risk relatives is warranted to prevent complications. Existing international guidelines lack detailed instructions regarding genetic evaluation and family screening of TAA patients...
May 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29430970/pain-exposure-physical-therapy-versus-conventional-treatment-in-complex-regional-pain-syndrome-type-1-a-cost-effectiveness-analysis-alongside-a-randomized-controlled-trial
#17
Karlijn Barnhoorn, J Bart Staal, Robert Tm van Dongen, Jan Paul M Frölke, Frank P Klomp, Henk van de Meent, Eddy Adang, Maria Wg Nijhuis-van der Sanden
OBJECTIVE: To analyze cost-effectiveness of Pain Exposure Physical Therapy compared to conventional treatment alongside a randomized controlled trial (NCT00817128) in patients with complex regional pain syndrome type 1, where no clinical difference was shown between the two groups in an intention-to-treat analysis. DESIGN: Randomized controlled trial with 9 months follow-up. SETTING: Patients were recruited from hospitals and general practitioners in the region around a university hospital...
February 1, 2018: Clinical Rehabilitation
https://www.readbyqxmd.com/read/29411189/guillain-barr%C3%A3-syndrome-following-varicella-zoster-virus-infection
#18
Badrul Islam, Zhahirul Islam, Corine H GeurtsvanKessel, Israt Jahan, Hubert P Endtz, Quazi D Mohammad, Bart C Jacobs
We describe the frequency, clinical features, and electrophysiological and immunological phenotypes of Guillain-Barré Syndrome (GBS) patients treated at a single institution in Bangladesh who had preceding chicken pox (primary Varicella-zoster virus [VZV] infection) within 4 weeks of GBS onset. A literature review of GBS cases preceding VZV infection is also provided. Diagnosis of GBS was based on the National Institute of Neurological Disorders and Stroke criteria for GBS. Serum anti-VZV IgM and IgG antibodies were quantified by indirect chemiluminescence immunoassay (CLIA); anti-Campylobacter jejuni IgG, IgM, and IgA antibodies and anti-ganglioside GM1 IgM and IgG antibodies, by enzyme-linked immunosorbent assays...
March 2018: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/29408955/high-androgens-in-postmenopausal-women-and-the-risk-for-atherosclerosis-and-cardiovascular-disease-the-rotterdam-study
#19
Cindy Meun, Oscar H Franco, Klodian Dhana, Loes Jaspers, Taulant Muka, Yvonne Louwers, M Arfan Ikram, Bart C J M Fauser, Maryam Kavousi, Joop S E Laven
Context: Polycystic ovary syndrome (PCOS) is closely linked to hyperandrogenism. In PCOS, hyperandrogenism has been associated with metabolic disturbances which increase the risk for cardiovascular disease (CVD). Objective: To assess the association of high serum androgen levels, as a postmenopausal remnant of PCOS, with the prevalence of atherosclerosis and incidence of CVD in postmenopausal women. Design: The Rotterdam Study, a prospective population-based cohort study...
February 1, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29392890/a-mutation-update-on-the-lds-associated-genes-tgfb2-3-and-smad2-3
#20
Dorien Schepers, Giada Tortora, Hiroko Morisaki, Gretchen MacCarrick, Mark Lindsay, David Liang, Sarju G Mehta, Jennifer Hague, Judith Verhagen, Ingrid van de Laar, Marja Wessels, Yvonne Detisch, Mieke van Haelst, Annette Baas, Klaske Lichtenbelt, Kees Braun, Denise van der Linde, Jolien Roos-Hesselink, George McGillivray, Josephina Meester, Isabelle Maystadt, Paul Coucke, Elie El-Khoury, Sandhya Parkash, Birgitte Diness, Lotte Risom, Ingrid Scurr, Yvonne Hilhorst-Hofstee, Takayuki Morisaki, Julie Richer, Julie Désir, Marlies Kempers, Andrea L Rideout, Gabrielle Horne, Chris Bennett, Elisa Rahikkala, Geert Vandeweyer, Maaike Alaerts, Aline Verstraeten, Hal Dietz, Lut Van Laer, Bart Loeys
The Loeys-Dietz syndrome (LDS) is a connective tissue disorder affecting the cardiovascular, skeletal, and ocular system. Most typically, LDS patients present with aortic aneurysms and arterial tortuosity, hypertelorism, and bifid/broad uvula or cleft palate. Initially, mutations in transforming growth factor-β (TGF-β) receptors (TGFBR1 and TGFBR2) were described to cause LDS, hereby leading to impaired TGF-β signaling. More recently, TGF-β ligands, TGFB2 and TGFB3, as well as intracellular downstream effectors of the TGF-β pathway, SMAD2 and SMAD3, were shown to be involved in LDS...
May 2018: Human Mutation
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