Št. zadetkov: 11
Kratki znanstveni prispevek
Oznake:
nonclonal mast cell activation syndrome;
Selective response to omalizumab in a patient with concomitant ncMCAS and POTS : what does it teach us about the underlying disease?
Leto:
2018
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Pregledni znanstveni članek
Oznake:
autoinflammatory diseases;pyrin-associated autoinflammatory diseases;NLRP3-related autoinflammatory diseases;undifferentiated systemic autoinflammatory disease;relopathies;
The systemic autoinflammatory diseases are disorders of the innate immune system distinguished by severe inflammation resulting from dysregulation of the innate immune system. Hereditary fever syndromes, such as FMF, TNF receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes ...
Leto:
2019
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Izvirni znanstveni članek
Oznake:
Covid-19 -- drug therapy;hydroxychloroquine;chloroquine;
Background: Hydroxychloroquine and chloroquine have been proposed as treatments for coronavirus disease 2019 (Covid-19) on the basis of in vitro activity and data from uncontrolled studies and small, randomized trials. Methods: In this randomized, controlled, open-label platform trial comparing a ra ...
Leto:
2020
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Izvirni znanstveni članek
Oznake:
Covid-19 -- drug therapy;dexamethasone;
Background: Coronavirus disease 2019 (Covid-19) is associated with diffuse lung damage. Glucocorticoids may modulate inflammation-mediated lung injury and thereby reduce progression to respiratory failure and death. Methods: In this controlled, open-label trial comparing a range of possible treatmen ...
Leto:
2020
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Pregledni znanstveni članek
Oznake:
canakinumab;anti-IL1 terapija;anti-IL1 therapy;
Familial Mediterranean Fever (FMF) is the most prevalent genetic autoinflammatory disorder. In most patients, treatment with colchicine can prevent attacks of fever and inflammation. However, 5%-10% of patients are resistant to colchicine treatment, while a similar percentage cannot tolerate colchic ...
Leto:
2020
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Pregledni znanstveni članek
Oznake:
triptaza;hereditarna alfa-triptazemija;tryptase;hereditary alpha-tryptasemia;
The association between Hymenoptera venom-triggered anaphylaxis (HVA) and clonal mast cell-related disorders (cMCD) has been known for decades. However, recent breakthroughs in peripheral blood screening for KIT p.D816V missense variant have revealed the true extent of this clinical association whil ...
Leto:
2022
Vir:
Medicinska fakulteta (UL MF)
Kratki znanstveni prispevek
Oznake:
undifferentiated systemic autoinflammatory disease;disease-modifying antirheumatic drugs;
This clinical case series describes our experience with the use of Janus kinase 1/2 inhibitor baricitinib in two patients suffering from refractory adult-onset Still's disease (AOSD) as well as in one case suffering from AOSD-like autoinflammatory disease in the context of myelodysplastic syndrome. ...
Leto:
2020
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Izvirni znanstveni članek
Oznake:
avtovnetne bolezni;nediferencirana avtovnetna bolezen;molekularna karakterizacija;somatske mutacije;autoinflammation;undifferentiated autoinflammatory disease;molecular characterization;somatic mutations;
Objectives: We theorized that myelodysplastic syndrome (MDS) with somatic mutations and karyotype abnormalities are associated with autoinflammation, and that the presence of autoinflammatory disease affected prognosis in MDS. Methods: One hundred thirty-four MDS patients were assessed for the preva ...
Leto:
2021
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Izvirni znanstveni članek
Oznake:
sindrom H;sistemska avtovnetna bolezen;H syndrome;systemic autoinflammatory disease;
Biallelic mutations in SLC29A3 cause histiocytosis-lymphadenopathy plus syndrome, also known as H syndrome (HS). HS is a complex disorder, with ~ 25% of patients developing autoinflammatory complications consisting of unexplained fevers, persistently elevated inflammatory markers, and unusual lympha ...
Leto:
2021
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije
Shelly Pathak,
Dorota Rowczenio,
Samuel Lara-Reyna,
Mark Kačar,
Roger Owen,
Gina Doody,
Karoline Krause,
Helen J Lachmann,
Rainer Doffinger,
Darren Newton,
Sinisa Savic
Izvirni znanstveni članek
Oznake:
autoinflammatory diseases;IgM;
The Schnitzler Syndrome (SchS) is an acquired, autoinflammatory condition successfully treated with IL-1 inhibition. The two main defining features of this late-onset condition are neutrophilic urticarial dermatoses (NUD) and the presence of an IgM monoclonal component. While the former aspect has b ...
Leto:
2020
Vir:
Digitalni repozitorij raziskovalnih organizacij Slovenije