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Hunter syndrome

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https://www.readbyqxmd.com/read/28178428/effect-of-histophilus-somni-on-heart-and-brain-microvascular-endothelial-cells
#1
D O'Toole, R Hunter, T Allen, B Zekarias, J Lehmann, K S Kim, D Grab, L B Corbeil
Histophilus somni is a pathogenic gram-negative bacterium responsible for pneumonia and septicemia in cattle. Sequelae include infectious thrombotic meningoencephalitis (ITME), myocarditis, arthritis, and abortion. These syndromes are associated with widespread vasculitis and thrombosis, implicating a role for endothelium in pathogenesis. Histopathologic and immunohistochemical investigation of 10 natural cases of bovine H. somni myocarditis and 1 case of ITME revealed intravascular H. somni in large biofilm-like aggregates adherent to the luminal surface of microvascular endothelium...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28176204/universal-screening-for-lynch-syndrome-among-patients-with-colorectal-cancer-patient-perspectives-on-screening-and-sharing-results-with-at-risk-relatives
#2
Jessica Ezzell Hunter, Kathleen A Arnold, Jennifer E Cook, Jamilyn Zepp, Marian J Gilmore, Alan F Rope, James V Davis, Kellene M Bergen, Elizabeth Esterberg, Kristin R Muessig, Susan K Peterson, Sapna Syngal, Louise Acheson, Georgia Wiesner, Jacob Reiss, Katrina A B Goddard
Universal screening for Lynch syndrome (LS) among all cases of colorectal cancer (CRC) could increase the diagnosis of LS and reduce morbidity and mortality of LS-associated cancers. Given universal screening includes all patients, irrespective of high risk factors such early age at onset or family history of CRC, it is important to understand perspectives of all patients and not just those at high risk. As part of a study to assess the feasibility and implementation of universal screening, 189 patients newly diagnosed with CRC were surveyed about their interest in screening for LS and communication of results with at-risk family members...
February 7, 2017: Familial Cancer
https://www.readbyqxmd.com/read/28171221/the-significance-of-electron-microscopic-examination-of-gingiva-in-cases-of-hunter-syndrome-and-hereditary-gingival-fibromatosis
#3
(no author information available yet)
INTRODUCTION: Electron microscopy has been for decades a basic morphological method still used in diagnostic protocols of some pathological conditions affecting the ultrastructure of cells and extracellular matrix. The aim of this study was an ultrastructural description of gingiva of patients with Hunter syndrome and hereditary gingival fibromatosis. PATIENTS AND METHODS: Gingival biopsies were obtained during surgical periodontal treatment from a 9-year-old boy with Hunter disease (with enzyme replacement therapy with recombinant human idursulphase) and a 15-year-old girl with hereditary gingival fibromatosis...
October 8, 2016: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28159115/william-hunter-harridge-lecture-the-changing-face-of-short-gut-syndrome-management
#4
REVIEW
John J Fung
The evolution of managing short gut syndrome optimizes management and decision making of intestinal failure by way of a multidisciplinary team utilizing the latest advances in therapeutic options. Only the minority of patients referred for small bowel transplantation will actually need a transplant. Many of these patients can be rehabilitated without the need for transplant, by way of early referral, and thus the likelihood of medical therapy increases. On the other hand, in those patients with little likelihood of success with medical therapy, early referral decreases the morbidity and mortality associated with long-term total parenteral nutrition and associated complications and will improve their overall survival outcomes...
January 25, 2017: American Journal of Surgery
https://www.readbyqxmd.com/read/28154186/structural-analysis-reveals-the-deleterious-effects-of-telomerase-mutations-in-telomerase-associated-bone-marrow-failure-syndromes
#5
Hunter Hoffman, Cory Rice, Emmanuel Skordalakes
Naturally occurring mutations in the ribonucleoprotein reverse transcriptase, telomerase, are associated with the bone marrow failure syndromes dyskeratosis congenita (DKC), aplastic anemia (AA), and idiopathic pulmonary fibrosis (IPF). However, the mechanism by which these mutations impact telomerase function remains unknown. Here we present the structure of the human telomerase c-terminal extension (CTE or thumb domain) determined by the method of single-wavelength anomalous diffraction (SAD) to 2.31 A resolution...
February 1, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28126926/molecular-disease-monitoring-using-circulating-tumor-dna-in-myelodysplastic-syndromes
#6
Paul Yeh, Michael Dickinson, Sarah Ftouni, Tane Hunter, Devbarna Sinha, Stephen Q Wong, Rishu Agarwal, Ravikiran Vedururu, Kenneth Doig, Chun Yew Fong, Piers Blombery, David Westerman, Mark A Dawson, Sarah-Jane Dawson
The diagnosis and monitoring of myelodysplastic syndromes (MDS) are highly reliant on bone marrow morphology, which is associated with substantial inter-observer variability. Azacitidine is the mainstay of treatment in MDS, however only half of all patients respond. Therefore, there is an urgent need for improved modalities for the diagnosis and monitoring of MDS. The majority of MDS patients have either clonal somatic karyotypic abnormalities and/or gene mutations that aid in the diagnosis and can be used to monitor treatment response...
January 26, 2017: Blood
https://www.readbyqxmd.com/read/28103342/failed-tracheal-intubation-in-a-child-with-unrecognized-hunter-s-syndrome-having-emergency-surgery
#7
F Meng, Y Li, G Wang, Q Wang
No abstract text is available yet for this article.
July 7, 2016: West Indian Medical Journal
https://www.readbyqxmd.com/read/28087518/cardiovascular-function-in-male-and-female-jcr-la-cp-rats-effect-of-high-fat-high-sucrose-diet
#8
Ian Hunter, Amanda Soler, Gregory Joseph, Brenda Hutcheson, Chastity Bradford, Frank Zhang, Barry J Potter, Spencer D Proctor, Petra Rocic
30% of the world population is diagnosed with metabolic syndrome. High fat/high sucrose diet (HF/HS, Western diet) correlates with metabolic syndrome prevalence. We characterized effects of the HF/HS diet on vascular (arterial stiffness, vasoreactivity, coronary collateral development) and cardiac (echocardiography) function, oxidative stress and inflammation in a rat model of metabolic syndrome (JCR). Furthermore, we determined whether male vs. female animals were affected differentially by the Western diet...
January 13, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28077157/clinical-biochemical-and-molecular-characteristics-of-filipino-patients-with-mucopolysaccharidosis-type-ii-hunter-syndrome
#9
Mary Anne D Chiong, Daffodil M Canson, Mary Ann R Abacan, Melissa Mae P Baluyot, Cynthia P Cordero, Catherine Lynn T Silao
BACKGROUND: Mucopolysaccharidosis type II, an X-linked recessive disorder is the most common lysosomal storage disease detected among Filipinos. This is a case series involving 23 male Filipino patients confirmed to have Hunter syndrome. The clinical and biochemical characteristics were obtained and mutation testing of the IDS gene was done on the probands and their female relatives. RESULTS: The mean age of the patients was 11.28 (SD 4.10) years with an average symptom onset at 1...
January 11, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28062802/moving-target-transient-rotational-stenosis-precipitating-jugular-bow-hunter-s-syndrome
#10
Waleed Brinjikji, Christopher S Graffeo, Avital Perry, Terence Zimmerman, Jeffrey R Janus, Pearce P Morris, Gregory D Cascino, Giuseppe Lanzino
A 60-year-old man presented with a 10-month history of of stereotypical spells characterized by vertigo, tinnitus, blurred vision, left hemibody numbness, and occasional syncope, precipitated by turning his head leftwards. Cerebral angiography and CT angiography of the head and neck with provocative maneuvers did not demonstrate vertebral artery narrowing. However, there was narrowing of the left internal jugular vein due to extrinsic compression from the sternocleidomastoid with leftward head rotation in the setting of hypoplasia of the right internal jugular vein...
January 6, 2017: Journal of Neurointerventional Surgery
https://www.readbyqxmd.com/read/28018694/anterior-hypopituitarism-and-treatment-response-in-hunter-syndrome-a-comparison-of-two-patients
#11
Munier A Nour, Paola Luca, David Stephure, Xing-Chang Wei, Aneal Khan
Hypopituitarism is a clinically important diagnosis and has not previously been reported in Hunter syndrome. We contrast two cases with anatomic pituitary anomalies: one with anterior panhypopituitarism and the other with intact pituitary function. Patient 1, a 10-year-old boy with Hunter syndrome, was evaluated for poor growth and an ectopic posterior pituitary gland. Endocrine testing revealed growth hormone (GH) deficiency, secondary adrenal insufficiency, and tertiary hypothyroidism. An improvement in growth velocity with hormone replacement (GH, thyroxine, and corticosteroid) was seen; however, final adult height remained compromised...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28013357/physical-activity-but-not-sedentary-activity-is-reduced-in-primary-sj%C3%A3-gren-s-syndrome
#12
Wan-Fai Ng, Ariana Miller, Simon J Bowman, Elizabeth J Price, George D Kitas, Colin Pease, Paul Emery, Peter Lanyon, John Hunter, Monica Gupta, Ian Giles, David Isenberg, John McLaren, Marian Regan, Annie Cooper, Steven A Young-Min, Neil McHugh, Saravanan Vadivelu, Robert J Moots, David Coady, Kirsten MacKay, Bhaskar Dasgupta, Nurhan Sutcliffe, Michele Bombardieri, Costantino Pitzalis, Bridget Griffiths, Sheryl Mitchell, Samira Tatiyama Miyamoto, Michael Trenell
The aim of the study was to evaluate the levels of physical activity in individuals with primary Sjögren's syndrome (PSS) and its relationship to the clinical features of PSS. To this cross-sectional study, self-reported levels of physical activity from 273 PSS patients were measured using the International Physical Activity Questionnaire-short form (IPAQ-SF) and were compared with healthy controls matched for age, sex and body mass index. Fatigue and other clinical aspects of PSS including disease status, dryness, daytime sleepiness, dysautonomia, anxiety and depression were assessed using validated tools...
December 24, 2016: Rheumatology International
https://www.readbyqxmd.com/read/28011587/elevated-20-hete-impairs-coronary-collateral-growth-in-metabolic-syndrome-via-endothelial-dysfunction
#13
Gregory Joseph, Amanda Soler, Rebecca Hutcheson, Ian Hunter, Chastity Bradford, Brenda Hutcheson, Katherine H Gotlinger, Houli Jiang, John R Falck, Spencer Proctor, Michal Laniado Schwartzman, Petra Rocic
Coronary collateral growth (CCG) is impaired in metabolic syndrome (MetS). microRNA-145 (miR-145-Adv) delivery to our rat model of metabolic syndrome (JCR) completely restored and neutrophil depletion significantly improved CCG. We determined whether low endogenous levels of miR-145 in MetS allowed for elevated production of 20-hydroxyeicosatetraenoic acid (20-HETE), which in turn, resulted in excessive neutrophil accumulation and endothelial dysfunction leading to impaired CCG. Rats underwent 0-9 days of repetitive ischemia (RI)...
December 23, 2016: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28001909/neudose-a-cubesat-mission-for-dosimetry-of-charged-particles-and-neutrons-in-low-earth-orbit
#14
A R Hanu, J Barberiz, D Bonneville, S H Byun, L Chen, C Ciambella, E Dao, V Deshpande, R Garnett, S D Hunter, A Jhirad, E M Johnston, M Kordic, M Kurnell, L Lopera, M McFadden, A Melnichuk, J Nguyen, A Otto, R Scott, D L Wagner, M Wiendels
During space missions, astronauts are exposed to a stream of energetic and highly ionizing radiation particles that can suppress immune system function, increase cancer risks and even induce acute radiation syndrome if the exposure is large enough. As human exploration goals shift from missions in low-Earth orbit (LEO) to long-duration interplanetary missions, radiation protection remains one of the key technological issues that must be resolved. In this work, we introduce the NEUtron DOSimetry & Exploration (NEUDOSE) CubeSat mission, which will provide new measurements of dose and space radiation quality factors to improve the accuracy of cancer risk projections for current and future space missions...
January 2017: Radiation Research
https://www.readbyqxmd.com/read/27999126/moving-target-transient-rotational-stenosis-precipitating-jugular-bow-hunter-s-syndrome
#15
Waleed Brinjikji, Christopher S Graffeo, Avital Perry, Terence Zimmerman, Jeffrey R Janus, Pearce P Morris, Gregory D Cascino, Giuseppe Lanzino
A 60-year-old man presented with a 10-month history of of stereotypical spells characterized by vertigo, tinnitus, blurred vision, left hemibody numbness, and occasional syncope, precipitated by turning his head leftwards. Cerebral angiography and CT angiography of the head and neck with provocative maneuvers did not demonstrate vertebral artery narrowing. However, there was narrowing of the left internal jugular vein due to extrinsic compression from the sternocleidomastoid with leftward head rotation in the setting of hypoplasia of the right internal jugular vein...
December 20, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27992710/subjective-and-objective-measures-of-dryness-symptoms-in-primary-sj%C3%A3-gren-s-syndrome-capturing-the-discrepancy
#16
Oriana M Bezzina, Peter Gallagher, Sheryl Mitchell, Simon J Bowman, Bridget Griffiths, Victoria Hindmarsh, Ben Hargreaves, Elizabeth J Price, Colin T Pease, Paul Emery, Peter Lanyon, Michele Bombardieri, Nurhan Sutcliffe, Costantino Pitzalis, John Hunter, Monica Gupta, John McLaren, Anne M Cooper, Marian Regan, Ian P Giles, David A Isenberg, Saravanan Vadivelu, David Coady, Bhaskar Dasgupta, Neil J McHugh, Steven A Young-Min, Robert J Moots, Nagui Gendi, Mohammed Akil, Kirsten MacKay, W Fai Ng, Lucy J Robinson
BACKGROUND: There is a weak relationship between subjective symptoms and objective markers of disease activity in individuals with Primary Sjögren's Syndrome (PSS). This presents a significant barrier to developing treatments if modifying disease markers does not translate into reduced perception of symptoms. Little is known about the reasons for this discrepancy. OBJECTIVES: To develop a novel method for capturing the discrepancy between objective tests and subjective dryness symptoms (a 'Sensitivity' scale) and to explore predictors of dryness Sensitivity...
December 19, 2016: Arthritis Care & Research
https://www.readbyqxmd.com/read/27971714/cost-analysis-of-therapy-with-idursulfase-for-hunter-syndrome-in-bulgaria
#17
M Kamusheva, M Doneva, M Dimitrova, G Petrova
No abstract text is available yet for this article.
November 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/27965142/duane-syndrome-with-prominent-oculo-auricular-phenomenon
#18
Aubrey L Gilbert, David G Hunter
Duane syndrome is a congenital cranial dysinnervation disorder involving absent or anomalous innervation of the lateral and medial rectus muscles that is sometimes associated with other manifestations of dysinnervation. We describe a patient with right esotropic Duane syndrome with a long-standing retroauricular tugging sensation in right gaze who was noted to have prominent ipsilateral oculo-auricular phenomenon, representing either abnormal enhancement of existing innervation or an uncommon dysinnervation...
December 10, 2016: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/27913904/association-between-brain-structural-anomalies-electroencephalogram-and-history-of-seizures-in-mucopolysaccharidosis-type-ii-hunter-syndrome
#19
Ramón Ernesto Jiménez-Arredondo, Aniel Jessica Leticia Brambila-Tapia, Francisco Miguel Mercado-Silva, Martha Ortiz-Aranda, Verónica Benites-Godinez, Graciela Olmos-García-de-Alba, Luis Eduardo Figuera
Mucopolysaccharidosis type II or Hunter syndrome (MPS II) is a genetic disease that can course with intellectual impairment and central nervous system (CNS) alterations. To date, no report has documented electroencephalogram (EEG) measures associated with CNS alterations, detected by imaging studies, and the history of seizures in patients with MPS II. Therefore, we decided to search this association. We included 9 patients with MPS II and performed imaging studies of the brain to detect the presence of cortico-subcortical atrophy, enlarged subarachnoid space and supratentorial ventricular size...
December 2, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27899021/does-preoperative-diplopia-determine-the-incidence-of-postoperative-diplopia-after-repair-of-orbital-floor-fracture-an-institutional-review
#20
Avisham Ramphul, Gary Hoffman
PURPOSE: The purpose of our study was to investigate whether the occurrence of preoperative diplopia determines the incidence of postoperative diplopia after orbital floor repair. MATERIALS AND METHODS: We undertook a retrospective cohort study with a review of the records of 126 consecutive patients who had undergone repair of an orbital floor fracture under the maxillofacial surgery service at John Hunter Hospital (Newcastle, NSW, Australia). The primary predictor variables were a number of demographic, etiologic, and operative factors that might influence the occurrence of diplopia...
November 9, 2016: Journal of Oral and Maxillofacial Surgery
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