BACKGROUND: Hypersensitivity reactions (HR) are common in mastocytosis. However, little is known about triggers and risk factors. The registry of the European Competence Network on Mastocytosis (ECNM) enables reliable studies in a larger cohort of mastocytosis patients. We assessed prevalence, triggers and risk factors of HR in adults with mastocytosis in the ECNM registry. METHODS: Data were collected in 27 ECNM centers. We analyzed potential triggers (Hymenoptera venoms, food, drug, inhalant and others) and risk factors at diagnosis and during follow-up. The study group consisted of 2485 adults with mastocytosis, 1379 women (55.5%) and 1106 men (44.5%). Median age was 48.2 years (range 18-91 years). RESULTS: Nine hundred and forty eight patients (38.1%) reported one or more HR`. Most common triggers were Hymenoptera venoms in cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), whereas in advanced SM (advSM), most common elicitors were drugs, including nonsteroidal anti-inflammatory agents and penicillin. In multivariate analyses, tryptase level < 90 ng/mL, <15% infiltration by mast cells in bone marrow biopsy-sections, and diagnosis of ISM were identified as independent risk factors for HR. For drug-induced HR, prominent risk factors were advSM and high tryptase levels. New reactions were observed in 4.8% of all patients during 4 years follow-up. CONCLUSIONS: HR are mainly triggered by Hymenoptera venoms in patients with CM and ISM and by drugs in patients with advSM. Tryptase levels <90 ng/mL, mast cell bone marrow infiltration <15%, and WHO category ISM are predictors of HR. New HR occur in 4.8% of all patients within 4 years.

• MeSH
• Hypersensitivity epidemiology   diagnosis   MeSH
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Mastocytosis * epidemiology   diagnosis   complications   MeSH
• Adolescent MeSH
• Young Adult MeSH
• Pilot Projects MeSH
• Prevalence MeSH
• Registries * MeSH
• Risk Factors MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Male MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH

BACKGROUND: Organomegaly, including splenomegaly, hepatomegaly, and/or lymphadenopathy, are important diagnostic and prognostic features in patients with cutaneous mastocytosis (CM) or systemic mastocytosis (SM). OBJECTIVES: To investigate the prevalence and prognostic impact of 1 or more organomegalies on clinical course and survival in patients with CM/SM. METHODS: Therefore, 3155 patients with CM (n = 1002 [32%]) or SM (n = 2153 [68%]) enrolled within the registry of the European Competence Network on Mastocytosis were analyzed. RESULTS: Overall survival (OS) was adversely affected by the number of organomegalies (OS: #0 vs #1 hazard ratio [HR], 4.9; 95% CI, 3.4-7.1, P < .001; #1 vs #2 HR, 2.1, 95% CI, 1.4-3.1, P < .001; #2 vs #3 HR, 1.7, 95% CI, 1.2-2.5, P = .004). Lymphadenopathy was frequently detected in patients with smoldering SM (SSM, 18 of 60 [30%]) or advanced SM (AdvSM, 137 of 344 [40%]). Its presence confered an inferior outcome in patients with AdvSM compared with patients with AdvSM without lymphadenopathy (median OS, 3.8 vs 2.6 years; HR, 1.6; 95% CI, 1.2-2.2; P = .003). OS was not different between patients having organomegaly with either ISM or SSM (median, 25.5 years vs not reached; P = .435). At time of disease progression, a new occurrence of any organomegaly was observed in 17 of 40 (43%) patients with ISM, 4 of 10 (40%) patients with SSM, and 33 of 86 (38%) patients with AdvSM, respectively. CONCLUSIONS: Organomegalies including lymphadenopathy are often found in SSM and AdvSM. ISM with organomegaly has a similar course and prognosis compared with SSM. The number of organomegalies is adversely associated with OS. A new occurrence of organomegaly in all variants of SM may indicate disease progression.

• MeSH
• Mastocytosis, Cutaneous * MeSH
• Humans MeSH
• Lymphadenopathy * MeSH
• Mastocytosis * diagnosis   MeSH
• Prognosis MeSH
• Disease Progression MeSH
• Mastocytosis, Systemic * diagnosis   epidemiology   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH

In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level ≥125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p < 0.05) and better overall survival (p < 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels <125 ng/mL.

• MeSH
• Adult MeSH
• Bone Marrow metabolism   pathology   MeSH
• Skin Diseases physiopathology   MeSH
• Middle Aged MeSH
• Humans MeSH
• Mastocytosis diagnosis   epidemiology   metabolism   MeSH
• Mast Cells metabolism   pathology   MeSH
• Survival Rate MeSH
• Follow-Up Studies MeSH
• Prognosis MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Mastocytosis, Systemic diagnosis   epidemiology   metabolism   MeSH
• Tryptases metabolism   MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Geographicals
• Europe MeSH

Cílem textu je ozřejmit závažnost celé problematiky a upozornit na dílčí aspekty kybernetické bezpečnosti, která se týká téměř všech. Určeno pracovníkům v managementu informačních a komunikačních technologií, správcům sítí a odborné veřejnosti.

• Conspectus
• Úkoly veřejné správy, správní opatření, legislativa

BACKGROUND: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. METHODS: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. RESULTS: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. CONCLUSION: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.

• MeSH
• Bone Marrow MeSH
• Humans MeSH
• Mastocytosis * MeSH
• Mast Cells MeSH
• Prognosis MeSH
• World Health Organization MeSH
• Mastocytosis, Systemic * diagnosis   epidemiology   MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH

• Publication type
• Textbook MeSH

• Conspectus
• Patologie. Klinická medicína
• NML Fields
• vnitřní lékařství
• NML Publication type
• kolektivní monografie

The current research confirmed the presence of mi-croplastics in the environment and also in living organ-isms. Collection, separation and identification of micro-plastics from environment are very desirable activities, important for monitoring these undesirable materials. In order to determine the real risk brought about by plastic fragments, it is necessary to implement a standard protocol for their sampling, quantification and identification. In this study, analytical techniques, such as SEM, py-GC-MS, FTIR, NMR and Raman spectroscopy, often used for the identification of microplastics, are discussed, together with novel methods used to identify the pollutants adsorbed on them, e.g., pharmaceuticals and metals.

?Kniha provede procesem zavedení povinného nařízení EU o ochraně osobních údajů známého pod názvem General Data Protection Regulation - GDPR. Povinnost dopadne na všechny subjekty, které zpracovávají osobní údaje občanů EU - tedy na valnou většinu firem, organizací, obcí, škol nebo státních institucí.

• MeSH
• Confidentiality legislation & jurisprudence   MeSH
• European Union MeSH
• Civil Rights MeSH
• Publication type
• Handbook MeSH

• Conspectus
• Ústavní právo. Správní právo
• NML Fields
• právo, zákonodárství

• About
• Evropská unie. Authority

​Kniha provede procesem zavedení povinného nařízení EU o ochraně osobních údajů známého pod názvem General Data Protection Regulation - GDPR. Povinnost dopadne na všechny subjekty, které zpracovávají osobní údaje občanů EU - tedy na valnou většinu firem, organizací, obcí, škol nebo státních institucí.; ?Kniha provede procesem zavedení povinného nařízení EU o ochraně osobních údajů známého pod názvem General Data Protection Regulation - GDPR. Povinnost dopadne na všechny subjekty, které zpracovávají osobní údaje občanů EU - tedy na valnou většinu firem, organizací, obcí, škol nebo státních institucí.

• Keywords
• Právo,
• MeSH
• Confidentiality legislation & jurisprudence   MeSH
• European Union MeSH
• Civil Rights MeSH

• NML Fields
• právo, zákonodárství

Kmenové bu ň ky se stávají nad ě jným nástrojem bun ěč né terapie pro lé č bu ř ady doposud nelé č itelných defekt ů a onemocn ě ní. Aby bu ň ka mohla být ozna č ena jako kmenová, musí spl ň ovat dv ě základní kritéria – musí mít schopnost sebeobnovy a schopnost diferenciace do jiných typ ů bun ě k. Tyto požadavky spl ň ují embryonální kmenové bu ň ky, kmenové bu ň ky z pupe č níkové krve, kmenové bu ň ky získané z dosp ě lého orga- nisnu a um ě le p ř ipravené, tzv. indukované pluripotentní kmenové bu ň ky. Další významnou vlastností kmenových bun ě k je schopnost regulovat imunitní reakce. Bylo prokázáno, že r ů zné typy kmenových bun ě k inhibují aktivitu bun ě k p ř irozené i adaptivní imunity, potla č ují proliferaci aktivovaných leukocyt ů , inhibují produkci cytokin ů a potla č ují vývoj cytotoxických T a NK bun ě k. V preklinických modelech in vivo bylo prokázáno, že podávání mesenchymálních kmenových bun ě k prodlužuje p ř ežívání alotransplantát ů , inhibuje rozvoj a projevy autoimunitních onemocn ě ní a potla č uje zán ě tlivé reakce. Tyto poznatky vedly k zahájení klinických studií využívajících kmenové bu ň ky. Kmenové bu ň ky lze podávat lokáln ě nebo systémov ě a jejich efekt je spojen s produkcí ř ady r ů stových a trofických faktor ů , diferencia č ním potenciálem a imuno- supresivním p ů sobením, kdy potla č ují nežádoucí imunologické reakce. A č koliv z ů stává mnoho nezodpov ě zených otázek spojených s užitím terapie založené na kmenových bu ň kách, tyto bu ň ky mají nepochybn ě velký potenciál v regenerativní a reparativní medicín ě .

Stem cells become a perspective and promising tool for cellular therapy to treat a number of so far uncurable defects and disea ses. To be cell recognized as stem cell, it must fulfil two basic criteria – the ability of self-renewal and the capability to differentiate in to other cell types. These characteristics have embryonic stem cells, the cells from umbilical cord, stem cells isolated from tissues of adult organ ism and arteficially prepared so called induced pluripotent stem cells. Another important property of stem cells is their ability to modulate immune response. It has been shown in vitro , that various types of stem cells inhibit the activity of cells of both natural and adaptive immunity, suppress proliferation of activated leukocytes, attenuate production of cytokines and suppress development of cytotoxic T and NK cells. In preclinical models it has been shown that administration of mesenchymal stem cells prolongs survival of allografts, attenuates development and manifestat ion of auto- immune diseases and inhibits inflammatory reactions. These observations iniciated numerous clinical studies utilizing stem cell s for cell-based therapy. Stem cells are applied locally or systemically and their therapeutic potential is mediated by differentiation into oth er cell types, by production of numerous growth and trophic factors and by their ability to suppress harmful immune reactions. Although there is a number of unanswered questions associated with the use of stem cell-based therapy, stem cells have undoubtedly enormous potential in rege nerative and reparative medicine.

• MeSH
• Cell- and Tissue-Based Therapy * methods   MeSH
• Immune Tolerance MeSH
• Stem Cells * physiology   immunology   classification   MeSH
• Humans MeSH
• Lymphocytes immunology   MeSH
• Cell Transplantation methods   MeSH
• Mesenchymal Stem Cell Transplantation methods   MeSH
• Transplantation Tolerance MeSH
• Stem Cell Research MeSH
• Check Tag
• Humans MeSH
• Publication type
• Clinical Trial MeSH
• Research Support, Non-U.S. Gov't MeSH
• Review MeSH