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https://www.readbyqxmd.com/read/29777655/long-term-outcomes-in-systemic-sclerosis-associated-pulmonary-arterial-hypertension-from-the-pulmonary-hypertension-assessment-and-recognition-of-outcomes-in-scleroderma-registry-pharos
#1
Kathleen D Kolstad, Shufeng Li, Virginia Steen, Lorinda Chung
BACKGROUND: Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH. METHODS: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of SSc patients at high risk for or with incident pulmonary hypertension based on right heart catheterization (RHC). Incident World Health Organization Group I PAH patients were analyzed...
May 16, 2018: Chest
https://www.readbyqxmd.com/read/29776943/mycophenolate-mofetil-induced-colitis-in-a-patient-with-systemic-sclerosis
#2
Gianluca Moroncini, Devis Benfaremo, Alessandra Mandolesi, Armando Gabrielli
We present the case of a 44-year-old woman affected by systemic sclerosis (SSc) who was admitted to our department for abdominal pain, nausea, vomiting and fever. Imaging studies showed the presence of a thickened colon wall involving the descending colon and the sigma, while a subsequent endoscopy revealed multiple serpiginous ulcers covered with fibrin and exudates. Under the hypothesis of drug-induced colitis, mycophenolate mofetil (MMF), which she was taking for SSc-related interstitial lung disease (ILD), was readily suspended, with a rapid recovery without further treatment...
May 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29776440/radiological-pleuroparenchymal-fibroelastosis-associated-to-limited-cutaneous-systemic-sclerosis-a-case-report
#3
D Hassoun, S Dirou, P P Arrigoni, C Durant, M Hamidou, A Néel, C Agard
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease (ILD) characterized by progressive fibrotic lesions of the visceral pleura and the sub-pleural parenchyma, affecting predominantly the upper lobes. PPFE may occur in different contextes like bone marrow or lung transplantations, but also in the context of telomeropathy with mutations of telomerase reverse transcriptase (TERT), telomerase RNA component (TERC) or regulator of telomere elongation helicase 1 (RTEL1) genes...
May 18, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29773274/scleroderma-in-children
#4
REVIEW
Francesco Zulian
Juvenile scleroderma with its two varieties, juvenile localized scleroderma and systemic sclerosis (SSc), represents the third most frequent rheumatic disease in childhood. In juvenile SSc, new developments have been recently reported in the fields of classification and monitoring. The introduction of new classification criteria for adult SSc has stimulated new ideas on how to improve the performance of the provisional 2007 PRES/ACR/EULAR pediatric criteria. The introduction of a multidimensional severity score, named "J4S," which includes parameters on growth, skin, and internal organ involvement, has improved the approach to the patients in the daily practice to guide decision-making...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29771823/residual-force-enhancement-is-attenuated-in-a-shortening-magnitude-dependent-manner
#5
Atsuki Fukutani, Walter Herzog
INTRODUCTION: The isometric force attained after active stretch is greater than that attained in a purely isometric contraction. This property is referred to as residual force enhancement (RFE). Although RFE is thought to contribute to the enhanced force and power in stretch-shortening cycles (SSCs), it is unclear whether shortening that occurs after active stretch eliminates the RFE induced by active stretch. Therefore, we evaluated the influence of shortening on RFE. METHODS: Skinned rabbit soleus fibers (N = 43) were used for all tests...
May 16, 2018: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/29771006/the-anti-fibrotic-effect-of-adenosine-a2b-receptor-antagonism-in-a-mouse-model-of-dermal-fibrosis
#6
Harry Karmouty-Quintana, Jose G Molina, Kemly Philip, Chiara Bellocchi, Brent Gudenkauf, Minghua Wu, Ning-Yuan Chen, Scott D Collum, Junsuk Ko, Sandeep K Agarwal, Shervin Assassi, Hongyan Zhong, Michael R Blackburn, Tingting Weng
OBJECTIVE: Systemic sclerosis (SSc - scleroderma) is a chronic disease that affects the skin and various internal organs. Dermal fibrosis is a major component of this disease. The mechanisms that promote dermal fibrosis remain elusive. Elevations in tissue adenosine levels and the subsequent engagement of the pro-fibrotic A2B adenosine receptor (ADORA2B) have been shown to regulate fibrosis in multiple organs including the lung, kidney and penis; however, the role of the ADORA2B in dermal fibrosis has not been investigated...
May 17, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29770326/characterization-and-comparative-analysis-of-the-complete-chloroplast-genome-of-the-critically-endangered-species-streptocarpus-teitensis-gesneriaceae
#7
Cornelius M Kyalo, Andrew W Gichira, Zhi-Zhong Li, Josphat K Saina, Itambo Malombe, Guang-Wan Hu, Qing-Feng Wang
Streptocarpus teitensis (Gesneriaceae) is an endemic species listed as critically endangered in the International Union for Conservation of Nature (IUCN) red list of threatened species. However, the sequence and genome information of this species remains to be limited. In this article, we present the complete chloroplast genome structure of Streptocarpus teitensis and its evolution inferred through comparative studies with other related species. S. teitensis displayed a chloroplast genome size of 153,207 bp, sheltering a pair of inverted repeats (IR) of 25,402 bp each split by small and large single-copy (SSC and LSC) regions of 18,300 and 84,103 bp, respectively...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29769578/metabolomic-profile-of-systemic-sclerosis-patients
#8
Federica Murgia, Silvia Svegliati, Simone Poddighe, Milena Lussu, Aldo Manzin, Tatiana Spadoni, Colomba Fischetti, Armando Gabrielli, Luigi Atzori
Systemic sclerosis (SSc) is an autoimmune disease of unknown aetiology characterized by vascular lesions, immunological alterations and diffuse fibrosis of the skin and internal organs. Since recent evidence suggests that there is a link between metabolomics and immune mediated disease, serum metabolic profile of SSc patients and healthy controls was investigated by 1 H-NMR and GC-MS techniques. The results indicated a lower level of aspartate, alanine, choline, glutamate, and glutarate in SSc patients compared with healthy controls...
May 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29769294/pulmonary-function-tests-as-outcomes-for-systemic-sclerosis-interstitial-lung-disease
#9
REVIEW
Melissa Caron, Sabrina Hoa, Marie Hudson, Kevin Schwartzman, Russell Steele
Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression ( i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD ( i...
June 30, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29768337/variation-in-origin-of-the-long-head-of-the-biceps-brachii-tendon-in-a-cadaver-a-case-report
#10
Shuji Katsuki, Hayato Terayama, Ryuta Tanaka, Ning Qu, Osamu Tanaka, Kanae Umemoto, Kaori Suyama, Kou Sakabe
INTRODUCTION: In general, the long head of the biceps brachii (LHB) tendon arises from the supraglenoid tubercle in the shoulder joint, and it has an important stabilizing mechanism for the humeral head in the shoulder joint. This case demonstrates that even if the LHB tendon can be palpated outside of the shoulder joint, it may disappear in the intertubercular sulcus (IS) and in the glenohumeral (GH) joint because of abnormal articulation. PATIENT CONCERNS: This case involved the cadaver of an 82-year-old Japanese man (number 1936, cause of death: hepatocellular tumor), who was selected from the bodies used for gross anatomy practice at the Tokai University School of Medicine in 2017...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29766518/pls-ipls-ga-pls-models-for-soluble-solids-content-ph-and-acidity-determination-in-intact-dovyalis-fruit-using-near-infrared-spectroscopy
#11
Mateus Wagner Dantas de Assis, Deborah Oliveira De Fusco, Rosangela Câmara Costa, Kássio Michell Gomes de Lima, Luis Carlos Cunha Júnior, Gustavo Henrique de Almeida Teixeira
BACKGROUND: Dovyalis species Dovyalis abyssinica Warb. and Dovyalis hebecarpa Warb. were introduced in Brazil, but the fruit quality of these species are not appropriate for fresh consumption due to the high titratable acidity (TA) and low soluble solids content (SSC). With the selection of new D. abyssinica clones with lower acidity and the hybridization of these two dovyalis specie (D. abyssinica and D. hebecarpa) the fruit quality improved and the better physical-chemical characteristics make them more suitable for fresh consumption...
May 16, 2018: Journal of the Science of Food and Agriculture
https://www.readbyqxmd.com/read/29766241/cellulase-production-by-white-rot-basidiomycetous-fungi-solid-state-versus-submerged-cultivation
#12
REVIEW
Joseph A Bentil, Anders Thygesen, Moses Mensah, Lene Lange, Anne S Meyer
White-rot basidiomycetous (WRB) fungi are a group of wood-decaying fungi that are known to be endowed with the ability to secrete enzymes that can catalyze decomposition of a range of plant cell wall polysaccharides, including cellulose and lignin. Expression of these enzymes is induced by the substrate and the enzyme yields obtained depend on the growth of the fungi and thus the mode of cultivation. In order to exploit WRB fungi for local enzyme production for converting lignocellulosic materials in biorefinery processes, the fungi can principally be cultivated in either solid-state (SSC) or submerged cultivation (SmC) systems...
May 15, 2018: Applied Microbiology and Biotechnology
https://www.readbyqxmd.com/read/29763902/prognostic-factors-of-renal-involvement-in-systemic-sclerosis
#13
Edoardo Rosato, Antonietta Gigante, Biagio Barbano, Maria Ludovica Gasperini, Rosario Cianci, Maurizio Muscaritoli
BACKGROUND/AIMS: Renal involvement is common in systemic sclerosis (SSc), including asymptomatic reduction of glomerular filtration rate (GFR), increased renal resistance indices, scleroderma renal crisis (SRC) and ANCA-associated vasculitis. The aim was to evaluate type and evolution of renal involvement for a period of five years. METHODS: 121 SSc patients (100 F, 21 M) with mean age of 54.9 ± 13.8, disease duration of 9 ± 6 years, of which 62 had a diffused form and 59 limited form were enrolled...
May 10, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29763468/clinical-phenotypes-and-survival-of-pre-capillary-pulmonary-hypertension-in-systemic-sclerosis
#14
David Launay, David Montani, Paul M Hassoun, Vincent Cottin, Jérôme Le Pavec, Pierre Clerson, Olivier Sitbon, Xavier Jaïs, Laurent Savale, Jason Weatherald, Vincent Sobanski, Stephen C Mathai, Majid Shafiq, Jean-François Cordier, Eric Hachulla, Gérald Simonneau, Marc Humbert
Pre-capillary pulmonary hypertension (PH) in systemic sclerosis (SSc) is a heterogeneous condition with an overall bad prognosis. The objective of this study was to identify and characterize homogeneous phenotypes by a cluster analysis in SSc patients with PH. Patients were identified from two prospective cohorts from the US and France. Clinical, pulmonary function, high-resolution chest tomography, hemodynamic and survival data were extracted. We performed cluster analysis using the k-means method and compared survival between clusters using Cox regression analysis...
2018: PloS One
https://www.readbyqxmd.com/read/29762905/pharmacokinetics-and-the-transition-to-extended-half-life-factor-concentrates-communication-from-the-ssc-of-the-isth
#15
Margaret V Ragni, Stacy E Croteau, Massimo Morfini, Marjon H Cnossen, Alfonso Iorio
Extended half-life proteins (EHL) are increasingly used in clinical practice, but there is no standardized approach to sampling, interpretation, and implementation of pharmacokinetics (PK) data to maximize treatment benefit. The goal of EHL treatment is to attain a trough level sufficient to protect against spontaneous bleeds and reduce infusion frequency and limitations on individual activity and lifestyle. Performing classical PK assessments requires multiple blood samples which is burdensome for patients and providers...
May 15, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29761614/diagnostic-value-of-nailfold-videocapillaroscopy-in-systemic-sclerosis-secondary-pulmonary-arterial-hypertension-a-meta-analysis
#16
Zhongbin Xia, Guiping Wang, Huashi Xiao, Shanshan Guo, Ying Liu, Fanru Meng, Dan Liu, Guoqing Li, Liang Zong
BACKGROUND: Microvascular changes play a decisive role in systemic sclerosis (SSc) and occur early in the course of the disease. Pulmonary arterial hypertension (PAH) represents one of the main clinical expressions of the vascular changes in SSc, and the abnormal changes especially capillary density and capillary width are detectable at nailfold videocapillaroscopy (NVC). AIM: The aim of this meta-analysis was to investigate the differences in capillary nailfold changes in SSc patients with and without PAH, and to estimate the early diagnostic value of NVC in systemic sclerosis secondary pulmonary arterial hypertension (SSc-PAH)...
May 15, 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/29760620/anti-endothelial-cell-antibodies-do-not-correlate-with-disease-activity-in-systemic-sclerosis
#17
Małgorzata Michalska-Jakubus, Małgorzata Kowal, Michał Adamczyk, Dorota Krasowska
Introduction: Anti-endothelial cell antibodies (AECA) recognize endothelial cell proteins and are thought to play an important role in vascular damage observed in systemic scleroderma (SSc) and many other autoimmune diseases. In SSc, AECA were found to be more common in patients with pulmonary hypertension, digital ulcers and nailfold capillaroscopic changes. Until now, there have been no studies examining the association between AECA positivity with the activity and duration of the disease...
April 2018: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29760619/adamts13-deficiency-and-immunological-abnormalities-in-patients-with-systemic-sclerosis
#18
Zofia Gerlicz-Kowalczuk, Jolanta D Torzecka, Elżbieta Dziankowska-Zaborszczyk, Alicja Ograczyk, Anna M Zalewska-Janowska, Anna Woźnicka, Bożena Dziankowska-Bartkowiak
Introduction: Systemic sclerosis (SSc) is a chronic autoimmune connective tissue disorder characterized by immunological deviations and generalized microvascular damage. Aim: To determine the serum level of the von Willebrand factor cleaving protease (ADAMTS13) in 39 SSc patients and healthy controls. Material and methods: ADAMTS13 serum level was determined in 39 SSc patients and 11 healthy controls. Complete history of the patients was recorded and thorough clinical, rheumatological, and dermatological examinations were performed...
April 2018: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29760157/methyl-cpg-binding-protein-2-mediates-antifibrotic-effects-in-scleroderma-fibroblasts
#19
Ye He, Pei-Suen Tsou, Dinesh Khanna, Amr H Sawalha
OBJECTIVE: Emerging evidence supports a role for epigenetic regulation in the pathogenesis of scleroderma (SSc). We aimed to assess the role of methyl-CpG-binding protein 2 (MeCP2), a key epigenetic regulator, in fibroblast activation and fibrosis in SSc. METHODS: Dermal fibroblasts were isolated from patients with diffuse cutaneous SSc (dcSSc) and from healthy controls. MeCP2 expression was measured by qPCR and western blot. Myofibroblast differentiation was evaluated by gel contraction assay in vitro...
May 14, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29755313/individualized-functional-parcellation-of-the-human-amygdala-using-a-semi-supervised-clustering-method-a-7t-resting-state-fmri-study
#20
Xianchang Zhang, Hewei Cheng, Zhentao Zuo, Ke Zhou, Fei Cong, Bo Wang, Yan Zhuo, Lin Chen, Rong Xue, Yong Fan
The amygdala plays an important role in emotional functions and its dysfunction is considered to be associated with multiple psychiatric disorders in humans. Cytoarchitectonic mapping has demonstrated that the human amygdala complex comprises several subregions. However, it's difficult to delineate boundaries of these subregions in vivo even if using state of the art high resolution structural MRI. Previous attempts to parcellate this small structure using unsupervised clustering methods based on resting state fMRI data suffered from the low spatial resolution of typical fMRI data, and it remains challenging for the unsupervised methods to define subregions of the amygdala in vivo ...
2018: Frontiers in Neuroscience
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