keyword
MENU ▼
Read by QxMD icon Read
search

Progressive systemic sclerosis

keyword
https://www.readbyqxmd.com/read/28340090/predictive-value-of-european-scleroderma-group-activity-index-in-an-early-scleroderma-cohort
#1
Tatiana Nevskaya, Murray Baron, Janet E Pope
Objective.: To estimate the effect of disease activity, as measured by the European Scleroderma Research Group Activity Index (EScSG-AI), on the risk of subsequent organ damage in a large systemic sclerosis (SSc) cohort. Methods.: Of 421 SSc patients from the Canadian Scleroderma Research Group database with disease duration of ⩽ 3 years, 197 who had no evidence of end-stage organ damage initially and available 3 year follow-up were included. Disease activity was assessed by the EScSG-AI with two variability measures: the adjusted mean EScSG-AI (the area under the curve of the EScSG-AI over the observation period) and persistently active disease/flare...
March 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/28334875/white-matter-changes-in-paediatric-multiple-sclerosis-and-monophasic-demyelinating-disorders
#2
Giulia Longoni, Robert A Brown, Parya MomayyezSiahkal, Colm Elliott, Sridar Narayanan, Amit Bar-Or, Ruth Ann Marrie, E Ann Yeh, Massimo Filippi, Brenda Banwell, Douglas L Arnold
Most children who experience an acquired demyelinating syndrome of the central nervous system will have a monophasic disease course, with no further clinical or radiological symptoms. A subset will be diagnosed with multiple sclerosis, a life-long disorder. Using linear mixed effects models we examined longitudinal diffusion properties of normal-appearing white matter in 505 serial scans of 132 paediatric participants with acquired demyelinating syndromes followed for a median of 4.4 years, many from first clinical presentation, and 106 scans of 80 healthy paediatric participants...
March 14, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28331767/radiation-fibrosis-syndrome-imitating-breast-cancer-recurrence-a-case-report
#3
Dauren Sarsenov, Fatma Aktepe, Vahit Özmen
Nowadays, surgery, radiotherapy and chemotherapy are the most frequently used modalities in the treatment of breast cancer. It is very well-known that some severe complications may result from after these treatments. Early and late complications of radiotherapy are well known. The complications of radiation therapy may be seen in (early) or after three months (late-delayed) of periods. These complications may be related with direct or indirect effect of radiation. The radiation fibrosis syndrome is a progressive fibrotic tissue sclerosis together with various clinical symptoms in the irradiation field...
January 2017: J Breast Health (2013)
https://www.readbyqxmd.com/read/28323224/progressive-multifocal-leukoencephalopathy-and-rheumatoid-arthritis-treatments
#4
Gaëlle Clavel, Antoine Moulignier, Luca Semerano
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system due to reactivation of the JC virus (JCV). PML is extremely uncommon despite the high prevalence of the virus in the general population. No specific treatment is available, and the prognosis is bleak. The diagnosis is based on brain imaging findings, detection of the JCV genome in cerebrospinal fluid samples and, in some cases, histological studies of the brain lesions. The pathophysiological mechanisms that drive the development of PML are incompletely understood...
March 16, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28321351/bilateral-birdshot-retinochoroiditis-and-retinal-astrocytoma
#5
Sunil Mamtora, Yun Wong, Dugald Bell, Teresa Sandinha
Background. This case highlights the importance of recognising multiple pathologies within the eye which may not necessarily be linked. Both birdshot retinochoroiditis and astrocytoma are rare conditions. The case underlines the need for early identification and treatment of birdshot retinochoroiditis with steroids and disease modifying drugs. Astrocytoma in the absence of tuberous sclerosis is also uncommon. Case Presentation. A 36-year-old male presented with 3-month history of bilateral progressive flashing lights and floaters...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/28321017/a-non-invasive-technique-for-the-evaluation-of-peripheral-circulatory-functions-in-female-subjects-with-raynaud-s-phenomenon
#6
Seyed Mohammad Mirbod, Haruo Sugiura
Japanese women now account for 43 percent of the labor force. A number of them are involved in construction, agricultural and forestry jobs. The aim of this study was to establish a non-invasive technique for the evaluation of peripheral circulatory functions in women with Raynaud's phenomenon (RP); and introduce a specific method for the assessment of vascular disturbances in females exposed to hand-transmitted vibration. The subjects of this study were 10 women with primary RP, 7 women with progressive systemic sclerosis (PSS) secondary to RP, and 17 females who were included as the control group...
March 17, 2017: Industrial Health
https://www.readbyqxmd.com/read/28320766/an-adverse-lipid-profile-and-increased-levels-of-adiposity-significantly-predict-clinical-course-after-a-first-demyelinating-event
#7
Prudence Tettey, Steve Simpson, Bruce Taylor, Anne-Louise Ponsonby, Robyn M Lucas, Terence Dwyer, Karam Kostner, Ingrid Af van der Mei
OBJECTIVE: To investigate the prospective associations between adiposity and lipid-related variables and conversion to multiple sclerosis (MS), time to subsequent relapse and progression in disability. METHODS: A cohort of 279 participants with a first clinical diagnosis of central nervous system demyelination was prospectively followed to 5-year review. Height, weight, waist and hip circumference were measured, and serum samples taken for measurement of lipids and apolipoproteins...
March 20, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28320130/spg2-mimicking-multiple-sclerosis-in-a-family-identified-using-next-generation-sequencing
#8
Anna Rubegni, Carla Battisti, Alessandra Tessa, Alfonso Cerase, Stefano Doccini, Alessandro Malandrini, Filippo M Santorelli, Antonio Federico
Several single gene disorders can potentially be overlooked in the differential diagnostic evaluation of patients with multiple sclerosis (MS). Pelizaeus-Merzbacher disease and spastic paraplegia type 2 are allelic X-linked disorders associated with defective myelination of the central nervous system and mutations in PLP1. Neurological symptoms are occasionally observed in female carriers of these mutations. Two women - the proposita (Pt1) and her mother (Pt2) - reported walking difficulties since adolescence and showed progressive cognitive decline...
April 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28320112/multiple-sclerosis-patients-understanding-and-preferences-for-risks-and-benefits-of-disease-modifying-drugs-a-systematic-review
#9
REVIEW
Gurpreet K Reen, Eli Silber, Dawn W Langdon
BACKGROUND: Multiple sclerosis (MS) patients are faced with complex risk-benefit profiles of disease-modifying drugs (DMDs) when making treatment decisions. For effective shared decision-making, MS patients should understand the risks and benefits of DMDs and make treatment decisions based on personal preferences. METHODS: This is an inclusive systematic review to primarily assess current understanding of MS patients for information about DMDs provided during the standard healthcare system...
April 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28317272/roles-of-ng2-glia-in-ischemic-stroke
#10
REVIEW
Fei-Er Song, Jia-Lv Huang, Si-Han Lin, Shuo Wang, Guo-Fen Ma, Xiao-Ping Tong
Recent studies have shown that a widely distributed class of glial cells, termed NG2-glia, engages in rapid signaling with surrounding neurons through direct synaptic contacts in the developing and mature central nervous system (CNS). This unique glial cell group has a typical function of proliferating and differentiating into oligodendrocytes during early development of the brain, which is crucial to axon myelin formation. Therefore, NG2-glia are also called oligodendrocyte precursor cells (OPCs). In vitro and in vivo studies reveal that NG2-glia expressing receptors and ion channels demonstrate functional significance for rapid signaling with neuronal synapses and modulation of neuronal activities in both physiological and pathological conditions...
March 19, 2017: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/28302159/a-data-driven-approach-links-microglia-to-pathology-and-prognosis-in-amyotrophic-lateral-sclerosis
#11
Johnathan Cooper-Knock, Claire Green, Gabriel Altschuler, Wenbin Wei, Joanna J Bury, Paul R Heath, Matthew Wyles, Catherine Gelsthorpe, J Robin Highley, Alejandro Lorente-Pons, Tim Beck, Kathryn Doyle, Karel Otero, Bryan Traynor, Janine Kirby, Pamela J Shaw, Winston Hide
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that lacks a predictive and broadly applicable biomarker. Continued focus on mutation-specific upstream mechanisms has yet to predict disease progression in the clinic. Utilising cellular pathology common to the majority of ALS patients, we implemented an objective transcriptome-driven approach to develop noninvasive prognostic biomarkers for disease progression. Genes expressed in laser captured motor neurons in direct correlation (Spearman rank correlation, p < 0...
March 16, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28302008/mitochondria-targeted-antioxidants-as-a-prospective-therapeutic-strategy-for-multiple-sclerosis
#12
Elena K Fetisova, Boris V Chernyak, Galina A Korshunova, Maria S Muntyan, Vladimir P Skulachev
BACKGROUND: Multiple sclerosis (MS) is one of the most widespread chronic neurological diseases that manifests itself by progressive demyelination in the central nervous system. The study of MS pathogenesis begins with the onset of the relapsing-remitting phase of the disease, which becomes apparent due to microglia activation, neuroinflammation and demyelination/remyelination in the white matter. The following progressive phase is accompanied by severe neurological symptoms when demyelination and neurodegeneration are spread to both gray and white matter...
March 16, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28298842/preoperative-parameters-and-their-prognostic-value-in-amyotrophic-lateral-sclerosis-patients-undergoing-implantation-of-a-diaphragm-pacing-stimulation-system
#13
Aydın Şanlı, Ihsan Şükrü Şengün, Volkan Karaçam, Aylin Özgen Alpaydın, Kemal Can Tertemiz, Sevgi Özalevli, Bahar Ağaoğlu Şanlı, Alper Kaya, Nezih Özdemir
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease with devastating and fatal respiratory complications. Diaphragm pacing stimulation (DPS) is a treatment option in diaphragm insufficient ALS patients. Ventilatory insufficiency depending on diaphragmatic failure is treated by the present study aimed to investigate prognostic value of preoperative clinical and functional characteristics of ALS patients undergoing implantation of a DPS system and to determine appropriate indications for the DPS system...
January 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28292174/extrahepatic-manifestations-of-primary-biliary-cholangitis
#14
REVIEW
Sara L Chalifoux, Peter G Konyn, Gina Choi, Sammy Saab
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition...
March 16, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28291582/sequential-nailfold-videocapillaroscopy-examinations-have-responsiveness-to-detect-organ-progression-in-systemic-sclerosis
#15
Jérôme Avouac, Gemma Lepri, Vanessa Smith, Elide Toniolo, Charlotte Hurabielle, Anaïs Vallet, Fazia Amrouche, André Kahan, Maurizio Cutolo, Yannick Allanore
OBJECTIVE: To determine the merit of nailfold videocapillaroscopy (NVC) to detect meaningful microvascular changes over time in patients with systemic sclerosis (SSc) and whether these changes are associated with overall disease progression and organ involvements. METHODS: A prospective cohort of 140 SSc patients was recruited over a 12-month period and was followed up on an annual basis for 3 years. Detailed NVC analysis was performed at inclusion and repeated annually...
February 10, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28283486/systematic-literature-review-and-network-meta-analysis-in-highly-active-relapsing-remitting-multiple-sclerosis-and-rapidly-evolving-severe-multiple-sclerosis
#16
Eline Huisman, Katerina Papadimitropoulou, James Jarrett, Matthew Bending, Zoe Firth, Felicity Allen, Nick Adlard
OBJECTIVE: Multiple sclerosis (MS) is a chronic, neurodegenerative autoimmune disorder affecting the central nervous system. Relapsing-remitting MS (RRMS) is the most common clinical form of MS and affects ∼85% of cases at onset. Highly active (HA) and rapidly evolving severe (RES) RRMS are 2 forms of RRMS amenable to disease-modifying therapies (DMT). This study explored the efficacy of fingolimod relative to other DMTs for the treatment of HA and RES RRMS. METHODS: A systematic literature review (SLR) was conducted to identify published randomised controlled trials in HA and RES RRMS...
March 10, 2017: BMJ Open
https://www.readbyqxmd.com/read/28282924/autophagy-and-microglia-novel-partners-in-neurodegeneration-and-aging
#17
REVIEW
Ainhoa Plaza-Zabala, Virginia Sierra-Torre, Amanda Sierra
Autophagy is emerging as a core regulator of Central Nervous System (CNS) aging and neurodegeneration. In the brain, it has mostly been studied in neurons, where the delivery of toxic molecules and organelles to the lysosome by autophagy is crucial for neuronal health and survival. However, we propose that the (dys)regulation of autophagy in microglia also affects innate immune functions such as phagocytosis and inflammation, which in turn contribute to the pathophysiology of aging and neurodegenerative diseases...
March 9, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28282243/differences-in-right-ventricular-functional-changes-during-treatment-between-systemic-sclerosis-associated-pulmonary-arterial-hypertension-and-idiopathic-pulmonary-arterial-hypertension
#18
Rahul G Argula, Abhijit Karwa, Abigail Lauer, David Gregg, Richard M Silver, Carol Feghali-Bostwick, Lynn M Schnapp, Kim Egbert, Bruce W Usher, Viswanathan Ramakrishnan, Paul M Hassoun, Charlie Strange
RATIONALE: Patients with scleroderma associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality despite the progress achieved with pulmonary arterial vasodilator therapies. OBJECTIVES: We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared to IPAH, based on the progression of echocardiographic right ventricular (RV) dysfunction. METHODS: Retrospective analysis of echocardiographic data in 13 SSc-PAH and 11 IPAH patients was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy...
March 10, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28277827/progress-and-prospects-for-the-use-and-the-understanding-of-the-mode-of-action-of-autologous-hematopoietic-stem-cell-transplantation-in-the-treatment-of-multiple-sclerosis
#19
Fredrika Collins, Majid Kazmi, Paolo A Muraro
A substantial proportion of patients with multiple sclerosis (MS) do not respond to pharmacological treatments and no currently approved therapy has been convincingly demonstrated to prevent or stop disease progression. With MS widely believed to be an auto-immune disease, immunoablative therapy followed by autologous haematopoietic stem cell transplantation (I/AHSCT) is being investigated as an alternative therapeutic option. Areas covered: With the results of phase III comparative trials only a few years away, this article reviews animal and clinical trials of I/AHSCT in the treatment of MS and discusses possible immunological mechanisms behind its action...
March 20, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28275485/serum-kl-6-and-surfactant-protein-d-as-monitoring-and-predictive-markers-of-interstitial-lung-disease-in-patients-with-systemic-sclerosis-and-mixed-connective-tissue-disease
#20
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. METHODS: We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD...
February 2017: Journal of Thoracic Disease
keyword
keyword
113130
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"