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Abstract: Background: Chronic urticaria (CU) is a common disease, characterized by the recurrent appearance of wheals, angioedema, or both for more than 6 weeks. Its underlying biology is not well understood, and many patients do not obtain sufficient relief from recommended treatments. Patient registries are rapidly growing as a form of research, because they can provide powerful, data-driven insights about the epidemiology of diseases, real-world effectiveness of treatments, rare patient types, safety monitoring, healthcare costs, and opportunities for quality improvement of healthcare delivery. Objectives: The Chronic Urticaria Registry (CURE) has been designed to improve the scientific understanding, clinical treatment, and healthcare planning of chronic urticaria patients. This report describes the rationale, methods, and initial implementation of this registry. Methods: CURE is an ongoing, prospective, international, multicenter, observational, voluntary registry of patients with CU. Participation in CURE is open to any physician treating CU patients, regardless of location, medical specialty, or type of practice setting. CURE aims to collect data on all CU patients, with no intentional selection or exclusion criteria. It collects baseline and follow-up data on the patient's demographics, history, symptoms, trigger and risk factors, therapies, and healthcare utilization. Results: CURE is a landmark achievement of the global urticaria medical community. As of 26 February 2020, 39 centers around the world have joined the registry and 35 have entered baseline data on a total of 2946 patients. Publications of this data will be forthcoming soon. Conclusions: CURE is eagerly seeking the participation of more physicians and the support of more governmental, charitable, and commercial sponsors from around the world. Here, in this paper, we invite other physicians to join this unique project to improve the lives of patients with CU.
Keywords: urticaria, registries
Published in DiRROS: 07.10.2020; Views: 1365; Downloads: 756
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Abstract: Background: An elevated basal serum tryptase level is associated with severe systemic anaphylaxis, most notably caused by Hymenoptera envenomation. Although clonal mast cell disease is the culprit in some individuals, it does not fully explain this clinical association. Objective: Our aim was to determine the prevalence and associated impact of tryptase genotypes on anaphylaxis in humans. Methods: Cohorts with systemic mastocytosis (SM) and venom as well as idiopathic anaphylaxis from referral centers in Italy, Slovenia, and the United States, underwent tryptase genotyping by droplet digital PCR. Associated anaphylaxis severity (Mueller scale) was subsequently examined. Healthy volunteers and controls with nonatopic disease were recruited and tryptase was genotyped by droplet digital PCR and in silico analysis of genome sequence, respectively. The effects of pooled and recombinant human tryptases, protease activated receptor 2 agonist and antagonist peptides, and a tryptase-neutralizing mAb on human umbilical vein endothelial cell permeability were assayed using a Transwell system. Results: Hereditary [alpha]-tryptasemia (H[alpha]T)--a genetic trait caused by increased [alpha]-tryptase-encoding Tryptase-[alpha]/[beta]1 (TPSAB1) copy number resulting in elevated BST level--was common in healthy individuals (5.6% [n = 7 of 125]) and controls with nonatopic disease (5.3% [n = 21 of 398]). H[alpha]T was associated with grade IV venom anaphylaxis (relative risk = 2.0; P < .05) and more prevalent in both idiopathic anaphylaxis (n = 8 of 47; [17%; P = .006]) and SM (n = 10 of 82 [12.2%; P = .03]) relative to the controls. Among patients with SM, concomitant H[alpha]T was associated with increased risk for systemic anaphylaxis (relative risk = 9.5; P = .007). In vitro, protease-activated receptor-2-dependent vascular permeability was induced by pooled mature tryptases but not [alpha]- or [beta]-tryptase homotetramers. Conclusions: Risk for severe anaphylaxis in humans is associated with inherited differences in [alpha]-tryptase-encoding copies at TPSAB1.
Keywords: mastocytosis, venoms, hypersensitivity, anaphylaxis - diagnosis, mast cells, idiopathic anaphylaxis, mast cell activation, hereditary alpha-tryptasemia
Published in DiRROS: 11.09.2020; Views: 2010; Downloads: 371
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Published in DiRROS: 19.03.2018; Views: 2918; Downloads: 1293
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Abstract: Dendrochronological analyses were made in two violins from private collectionsin Slovenia with an aim to date the wood of their resonance boards,to assess the time of their fabrication, to define the provenance of the wood, and to establish whether the labels of the instruments were original. The origin, the history and the exact age of the instruments had previously not been known. Tree-ring measurements were done on the surface of the bellies. In violin 1, it was made of one radial board, in violin 2 of two radial boards of Norway spruce (Picea abies). In violin 1, we measured 248 tree-rings and the year of the youngest ring was 1808. The dating was confirmed with more than 20 tree-ring chronologies from Austria, Germany, and Italy. The maximal value of the cross-dating parameter t-value after Hollstein(TH) was 12.4. In violin 2, we measured 141 and 137 tree-rings on each side of the belly, and the year of the youngest ring was 1640. The dating was also obtained by cross-dating with over 20 chronologies and confirmed with statistically significant TH up to 9.2. The year of the last ring in both cases corresponded with the terminus post quem, which indicated that the belly (instrument) was fabricated after the year of formation of the youngest ring. There is no evidence about the duration of seasoning and storage of the lumber, or how many tree-rings were removed when the instrumentwas manufactured. Our dendro-provenance study showed that the violin 1 most likely originated from Austria or southern Germany. The wood for the belly of the violin 2 possibly originated from Austria and the sequence significantly matched a chronology built from the instruments made by Jacob Stainer. In both instruments, the dendrochronological dating did not confirm the inscriptions on the labels on the inside of the instrument. The presented investigation was performed in 2008 and 2009 and is to our knowledge the first dendrochronological dating of music instruments in Slovenia.
Published in DiRROS: 12.07.2017; Views: 3762; Downloads: 1687
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