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Hughe's syndrome

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https://www.readbyqxmd.com/read/29455227/the-clinical-features-of-patients-concurrent-with-guillain-barre-syndrome-and-myasthenia-gravis
#1
Junliang Yuan, Juan Zhang, Bingwei Zhang, Wenli Hu
OBJECTIVE: To evaluate all the coincidence cases of Guillain-Barre syndrome (GBS) and myasthenia gravis (MG). METHODS: We performed web-based research of the overlapping incidence of GBS and MG in studies occurring from 1982 to 2016 and restricted to the English language. RESULTS: Among 15 cases, an elevated CSF protein level without pleocytosis was found in 10 cases (66.7%); reduced nerve conduction was found in 13 cases (86.6%); a positive repetitive nerve stimulation test occurred in 11 cases (73...
January 2018: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/29445110/promising-biomarkers-of-environmental-enteric-dysfunction-a-prospective-cohort-study-in-pakistani-children
#2
Najeeha Talat Iqbal, Kamran Sadiq, Sana Syed, Tauseefullah Akhund, Fayyaz Umrani, Sheraz Ahmed, Mohammad Yawar Yakoob, Najeeb Rahman, Shahida Qureshi, Wenjun Xin, Jennie Z Ma, Molly Hughes, Syed Asad Ali
Environmental Enteric Dysfunction (EED), a syndrome characterized by chronic gut inflammation, contributes towards stunting and poor response to enteric vaccines in children in developing countries. In this study, we evaluated major putative biomarkers of EED using growth faltering as its clinical proxy. Newborns (n = 380) were enrolled and followed till 18 months with monthly anthropometry. Biomarkers associated with gut and systemic inflammation were assessed at 6 and 9 months. Linear mixed effects model was used to determine the associations of these biomarkers with growth faltering between birth and 18 months...
February 14, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29438790/cervical-spinal-fusion-16-year-trends-in-epidemiology-indications-and-in-hospital-outcomes-by-surgical-approach
#3
Stephan N Salzmann, Peter B Derman, Lukas P Lampe, Janina Kueper, Ting Jung Pan, Jingyan Yang, Jennifer Shue, Federico P Girardi, Stephen Lyman, Alexander P Hughes
BACKGROUND: The rate of cervical spinal fusion has been increasing significantly. However, there is paucity of literature describing trends based on surgical approach using complete population databases. We investigated the approach-based trends in epidemiology, indications and in-hospital outcomes of cervical spinal fusion. METHODS: New York's Statewide Planning and Research Cooperative System database was queried to identify patients who underwent primary subaxial cervical fusion from 1997-2012...
February 10, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29430432/hughes-stovin-syndrome-as-an-outcome-of-beh%C3%A3-et-disease-or-as-a-different-entity
#4
Serkan Demirkan, Yıldırım Gültekin
Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym "Hughes-Stovin syndrome" was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease...
February 2018: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29419916/consensus-opinion-on-diagnosis-and-management-of-thrombotic-microangiopathy-in-australia-and-new-zealand
#5
REVIEW
Lucy Fox, Solomon J Cohney, Joshua Y Kausman, Jake Shortt, Peter D Hughes, Erica M Wood, Nicole M Isbel, Theo de Malmanche, Anne Durkan, Pravin Hissaria, Piers Blombery, Thomas D Barbour
Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. Whilst TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal...
February 8, 2018: Nephrology
https://www.readbyqxmd.com/read/29417387/fitz-hugh-curtis-syndrome-in-a-man-positive-for-chlamydia-trachomatis
#6
Kazuhide Takata, Hiromi Fukuda, Kaoru Umeda, Ryo Yamauchi, Sho Fukuda, Hideo Kunimoto, Takashi Tanaka, Keiji Yokoyama, Daisuke Morihara, Yasuaki Takeyama, Makoto Irie, Satoshi Shakado, Shotaro Sakisaka
Fitz-Hugh-Curtis syndrome (FHCS) is characterized by perihepatic and pelvic inflammation and occurs mostly in women of childbearing age. Here, we report a case of FHCS caused by Chlamydia trachomatis in a 50-year-old man. The patient presented to our hospital with right upper quadrant abdominal pain, and enhanced computed tomography revealed perihepatic and pelvic free fluid and early-phase hepatic capsular enhancement. A urine specimen was positive for Chlamydia trachomatis. The patient was diagnosed with FHCS due to Chlamydia trachomatis infection...
February 7, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29412158/allogeneic-hematopoietic-stem-cell-transplantation-for-gata2-deficiency-using-a-busulfan-based-regimen
#7
Mark Parta, Nirali N Shah, Kristin Baird, Hind Rafei, Katherine R Calvo, Thomas Hughes, Kristen Cole, Meg Kenyon, Bazetta Blacklock Schuver, Jennifer Cuellar-Rodriguez, Christa S Zerbe, Steven M Holland, Dennis D Hickstein
Allogeneic hematopoietic stem cell transplantation (HSCT) reverses the bone marrow failure syndrome due to GATA2 deficiency. The intensity of conditioning required to achieve reliable engraftment and prevent relapse remains unclear. Here, we describe the results of a prospective study of HSCT in 22 patients with GATA2 deficiency using a busulfan-based conditioning regimen. The study includes 2 matched related donor (MRD) recipients, 13 matched unrelated donor (URD) recipients, and 7 haploidentical related donor (HRD) recipients...
February 2, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29365426/the-approach-study-a-randomized-double-blind-placebo-controlled-phase-3-study-of-volanesorsen-administered-subcutaneously-to-patients-with-familial-chylomicronemia-syndrome-fcs
#8
Daniel Gaudet, Andres Digenio, Vickie Alexander, Marcello Arca, Alan Jones, Erik Stroes, Jean Bergeron, Ferdando Civeira, Linda Hemphill, Dirk Blom, Joann Flaim, Steven Hughes, Richard Geary, Sotirios Tsimikas, Joseph Witztum, Eric Bruckert
No abstract text is available yet for this article.
August 2017: Atherosclerosis
https://www.readbyqxmd.com/read/29340774/-exercise-to-me-is-a-scary-word-perceptions-of-fatigue-sleep-dysfunction-and-exercise-in-people-with-fibromyalgia-syndrome-a-focus-group-study
#9
D Russell, I C Álvarez Gallardo, I Wilson, C M Hughes, G W Davison, B Sañudo, J G McVeigh
Fibromyalgia syndrome (FMS) is a common and complex chronic pain condition. Exercise is recommended in the management of the FMS; however, people with FMS often find exercise exacerbates their condition and causes overwhelming fatigue. The objective of this study was to explore the perceptions of fatigue and sleep dysfunction, and exercise in people with FMS. Three, 60-90 min focus groups were conducted with people with FMS (n = 14). Participants were recruited from patient support groups who had experienced therapeutic exercise in the management of their condition...
January 16, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29339515/mers-cov-and-h5n1-influenza-virus-antagonize-antigen-presentation-by-altering-the-epigenetic-landscape
#10
Vineet D Menachery, Alexandra Schäfer, Kristin E Burnum-Johnson, Hugh D Mitchell, Amie J Eisfeld, Kevin B Walters, Carrie D Nicora, Samuel O Purvine, Cameron P Casey, Matthew E Monroe, Karl K Weitz, Kelly G Stratton, Bobbie-Jo M Webb-Robertson, Lisa E Gralinski, Thomas O Metz, Richard D Smith, Katrina M Waters, Amy C Sims, Yoshihiro Kawaoka, Ralph S Baric
Convergent evolution dictates that diverse groups of viruses will target both similar and distinct host pathways to manipulate the immune response and improve infection. In this study, we sought to leverage this uneven viral antagonism to identify critical host factors that govern disease outcome. Utilizing a systems-based approach, we examined differential regulation of IFN-γ-dependent genes following infection with robust respiratory viruses including influenza viruses [A/influenza/Vietnam/1203/2004 (H5N1-VN1203) and A/influenza/California/04/2009 (H1N1-CA04)] and coronaviruses [severe acute respiratory syndrome coronavirus (SARS-CoV) and Middle East respiratory syndrome CoV (MERS-CoV)]...
January 16, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29326294/guillain-barr%C3%A3-syndrome-in-southern-china-retrospective-analysis-of-hospitalised-patients-from-14-provinces-in-the-area-south-of-the-huaihe-river
#11
Shuping Liu, Zheman Xiao, Min Lou, Fang Ji, Bei Shao, Hongyuan Dai, Can Luo, Bo Hu, Ruiling Zhou, Zhangyu Zou, Jing Li, Xiaoyi Li, Jun Xu, Fan Hu, Chao Qin, Lian Wang, Tao Liu, Runtao Bai, Yangmei Chen, Haiyan Lv, Ruxu Zhang, Xiaoming Wang, Yunfu Wang, Shanling Ren, Xiaoming He, Zhenwei Jiang, Huiwen Wu, Donghui Yu, Wenqiong Yang, Wenjing Luo, Daokai Gong, Bin Chen, Yin Liu, Jiajia Yao, Yujie Yang, Jingxia Guan, Mingzhen Zhu, Xiujuan Fu, Genshan Gao, Hong Zhang, Man Ding, Shanghua Fan, Qian Cao, Jingyi Lu, Zuneng Lu
OBJECTIVES: The clinical and epidemiological profiles of Guillain-Barré syndrome (GBS) in southern China have yet to be fully recognised. We aimed to investigate the subtypes of GBS in southern China, compare the clinical features of demyelinating form with that of axonal form and test whether preceding infections and age have influence on the clinical phenotype, disease course and severity of GBS. METHODS: Medical records of patients with a diagnosis of GBS admitted to 31 tertiary hospitals, located in 14 provinces in southern China, from 1 January 2013 to 30 September 2016, were collected and retrospectively reviewed...
January 11, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29315359/non-freezing-cold-injury-a-multi-faceted-syndrome
#12
Clare M Eglin, Hugh Montgomery, Michael J Tipton
No abstract text is available yet for this article.
January 5, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29301476/the-cutaneous-manifestations-are-significantly-related-to-cerebrovascular-in-a-serbian-cohort-of-patients-with-hughes-syndrome
#13
L Stojanovich, A Djokovic, N Stanisavljevic, M Zdravkovic
Objectives To investigate a possible relationship between cerebrovascular, such as stroke and transient ischaemic attack, and various cutaneous manifestations (livedo reticularis, skin ulcerations, pseudovasculitis lesions, superficial cutaneous necrosis and digital gangrene) in antiphospholipid syndrome (APS). This report is based on a Serbian cohort of APS patients. Methods A total of 508 antiphospholipid syndrome APS patients were assessed: 360 with primary (PAPS) and 148 with APS associated with SLE (SAPS)...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29284768/fitz-hugh-curtis-syndrome-caused-by-gonococcal-infection-in-a-patient-with-systemic-lupus-erythematous-a-case-report-and-literature-review
#14
Darío A Rueda, Luisina Aballay, Lisandro Orbea, Diego A Carrozza, Paola Finocchietto, Silvia B Hernandez, Mariano M Volpacchio, Horacio di Fonzo
BACKGROUND Fitz-Hugh-Curtis (FHC) syndrome is a perihepatitis linked to inflammatory pelvic disease. It can be caused by Neisseria gonorrhoeae or Chlamydia trachomatis infections. FHC syndrome usually presents with pain in the right hypochondrium and fever, associated with symptoms and signs of pelvic infection in women. CASE REPORT We present the case of a 22-year-old woman with systemic lupus erythematous (SLE) who presented with polyarthritis, cutaneous lesions, and abdominal pain. The diagnosis of FHC syndrome was based on the findings of abdominal computerized tomography (CT) and the isolation of Neisseria gonorrhoeae (NG) in blood cultures...
December 29, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29278532/risks-of-cardiovascular-or-central-nervous-system-adverse-events-and-immune-reconstitution-inflammatory-syndrome-for-dolutegravir-versus-other-antiretrovirals-meta-analysis-of-randomized-trials
#15
Andrew M Hill, Nikkita Mitchell, Sophie Hughes, Anton L Pozniak
PURPOSE OF REVIEW: Results from nonrandomized cohort studies suggest higher risks of CNS adverse events for dolutegravir, versus other ARVs. There have been two case reports of myocarditis on dolutegravir. Integrase inhibitors have been associated with IRIS in two cohort studies. Meta-analysis of randomized trials can be used to cross-check potential safety signals. This systematic review of drug safety used an EMBASE and MEDLINE search combined with serious adverse event (SAE) reports on the website www...
December 22, 2017: Current Opinion in HIV and AIDS
https://www.readbyqxmd.com/read/29248359/impact-of-long-term-elosulfase-alfa-on-activities-of-daily-living-in-patients-with-morquio-a-syndrome-in-an-open-label-multi-center-phase-3-extension-study
#16
Christian J Hendriksz, Rossella Parini, Moeenaldeen D AlSayed, Julian Raiman, Roberto Giugliani, John J Mitchell, Barbara K Burton, Norberto Guelbert, Fiona J Stewart, Derralynn A Hughes, Robert Matousek, Sara M Hawley, Celeste Decker, Paul R Harmatz
BACKGROUND: Long-term safety and efficacy of elosulfase alfa enzyme replacement therapy (ERT) were assessed in 173 patients with Morquio A syndrome (mucopolysaccharidosis IVA) in a 96-week, open-label, multi-center, phase 3 extension study (MOR-005) of the pivotal 24-week, placebo-controlled study (MOR-004). Changes in efficacy endpoints were evaluated over 120weeks, from MOR-004 baseline to MOR-005 week 96. We report the impact of ERT on activities of daily living (ADL) across three domains (mobility, self-care, and caregiver-assistance), as assessed by the Mucopolysaccharidosis Health Assessment Questionnaire (MPS-HAQ) after 72 and 120weeks or approximately 1 and 2years...
December 5, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29244787/loss-of-podocalyxin-causes-a-novel-syndromic-type-of-congenital-nephrotic-syndrome
#17
Hee Gyung Kang, Moses Lee, Kyoung Boon Lee, Michael Hughes, Bo Sang Kwon, Sangmoon Lee, Kelly M McNagny, Yo Han Ahn, Jung Min Ko, Il-Soo Ha, Murim Choi, Hae Il Cheong
Many cellular structures directly imply specific biological functions. For example, normal slit diaphragm structures that extend from podocyte foot processes ensure the filtering function of renal glomeruli. These slits are covered by a number of surface proteins, such as nephrin, podocin, podocalyxin and CD2AP. Here we report a human patient presenting with congenital nephrotic syndrome, omphalocele and microcoria due to two loss-of-function mutations in PODXL, which encodes podocalyxin, inherited from each parent...
December 15, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/29240651/acute-impact-of-different-exercise-modalities-on-arterial-and-platelet-function
#18
Andrew Haynes, Matthew D Linden, Elisa Robey, Gerald F Watts, P Hugh R Barrett, Louise H Naylor, Daniel J Green
PURPOSE: Acute coronary syndromes and ischemic stroke are associated with arterial events involving platelets, the endothelium and atherosclerosis. Whilst regular physical activity is associated with lower risk of cardiovascular events and mortality, risk is transiently increased during and immediately following participation in an acute bout of exercise. No previous study has investigated the acute impact of exercise on platelet activation and arterial function in the same participants; it is also unknown if responses are dependent on exercise modality...
December 13, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/29238995/test-dose-pharmacokinetics-in-pediatric-patients-receiving-once-daily-iv-busulfan-conditioning-for-hematopoietic-stem-cell-transplant-a-reliable-approach
#19
Kristina M Brooks, Paul Jarosinski, Thomas Hughes, Elizabeth Kang, Nirali N Shah, John B Le Gall, Dennis D Hickstein, Suk See De Ravin, Jomy M George, Parag Kumar
Intravenous (IV) busulfan test dose pharmacokinetics (PK) has been shown to accurately predict once-daily dose requirements and improve outcomes in adult transplant patients, but there are limited data to support this approach in children. Test doses of busulfan ∼0.8 mg/kg were infused over 2 to 3 hours, followed by serial sampling to 4-6 hours postinfusion in pediatric hematopoietic stem cell transplant recipients (n = 5). Once-daily busulfan doses were calculated based on a myelosuppressive area under the concentration-time curve (AUC) target of ∼3700 to 4000 μmol·min/L and assumed dose-proportionality to the test dose...
December 14, 2017: Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/29226518/acceptability-and-benefit-of-rectal-irrigation-in-patients-with-low-anterior-resection-syndrome-a-qualitative-study
#20
G M McCutchan, D Hughes, Z Davies, J Torkington, C Morris, J A Cornish
AIM: Low Anterior Resection Syndrome (LARS) following rectal cancer surgery impairs the patient's quality of life (QoL). Rectal Irrigation has been demonstrated to be effective for anterior resection syndrome but many surgeons do not suggest it as a treatment. This feasibility study aimed to explore treatment acceptability and the benefit of rectal irrigation in patients who developed LARS following an anterior resection for rectal cancer. METHODS: This was a qualitative study, involving semi-structured interviews...
December 11, 2017: Colorectal Disease: the Official Journal of the Association of Coloproctology of Great Britain and Ireland
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