Results 21 to 30 of about 10,022 (165)

Monogenic autoinflammatory diseases in adults – a challenge to rheumatologic practice at the onset of the Polish national programme of interleukin 1 inhibitor treatment

open access: yesRheumatology, 2019
Monogenic autoinflammatory diseases (AIDs, formerly known as hereditary periodic fever syndromes) cover a spectrum of diseases which lead to chronic or recurrent inflammation caused by activation of the innate immune system. The most common monogenic AID
Marcin Milchert   +4 more
doaj   +1 more source

Biological Treatments: New Weapons in the Management of Monogenic Autoinflammatory Disorders

open access: yesMediators of Inflammation, 2013
Treatment of monogenic autoinflammatory disorders, an expanding group of hereditary diseases characterized by apparently unprovoked recurrent episodes of inflammation, without high-titre autoantibodies or antigen-specific T cells, has been revolutionized
Antonio Vitale   +11 more
doaj   +1 more source

Síndromes autoinflamatórias hereditárias na faixa etária pediátrica Pediatric hereditary autoinflammatory syndromes

open access: yesJornal de Pediatria, 2010
OBJETIVO: Descrever as principais síndromes autoinflamatórias hereditárias na faixa etária pediátrica. FONTES DOS DADOS: Foi realizada uma revisão da literatura nas bases de dados PubMed e SciELO, utilizando as palavras-chave "síndromes autoinflamatórias”
Adriana Almeida Jesus   +7 more
doaj   +1 more source

Familial Mediterranean fever (a periodic disease): The present-day view of the problem

open access: yesСовременная ревматология, 2013
The paper deals with the most common classical autoinflammatory disease familial Mediterra-nean fever (FMF)/periodic disease. This is a monogenic hereditary disease caused by mutations with an autosomal recessive pattern of inheritance.
Evgeny Stanislavovich Fedorov   +2 more
doaj   +3 more sources

Inflammasomes and dermatology [PDF]

open access: yesAnais Brasileiros de Dermatologia, 2016
: Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases.
Daniel Coelho de Sá, Cyro Festa Neto
doaj   +1 more source

The Clinical Chameleon of Autoinflammatory Diseases in Children

open access: yesCells, 2022
The very first line of defense in humans is innate immunity, serving as a critical strongpoint in the regulation of inflammation. Abnormalities of the innate immunity machinery make up a motley group of rare diseases, named ‘autoinflammatory’, which are ...
Eugenio Sangiorgi, Donato Rigante
doaj   +1 more source

Muckle–Wells Syndrome in a Child With Recurrent Urticaria

open access: yesВопросы современной педиатрии, 2017
Cryopyrin associated periodic syndromes (CAPS) are rare monogenic autoinflammatory diseases from the group of hereditary periodic syndromes caused by a regulation defect of inflammatory cytokines, in particular interleukin 1β.
Elena M. Kamaltynova   +5 more
doaj   +1 more source

Insights into CANDLE Syndrome

open access: yesRevista da Sociedade Portuguesa de Dermatologia e Venereologia, 2020
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a recently described chronic autoinflammatory disease, pathophysiologically related to intracellular proteasome/immunoproteasome dysfunction.
Katarina Kieselová   +2 more
doaj   +1 more source

Familial Mediterranean fever: Health-related quality of life and associated variables in a national cohort

open access: yesThe Turkish Journal of Gastroenterology, 2021
Objectives: This study aims to evaluate the effectivity of Familial Mediterranean Fever Quality of Life (FMF-QoL) Scale for the measurement of QoL in patients with FMF and to perform correlations between related clinical variables in Turkish patients.
Hatice BODUR   +30 more
doaj  

NLRP3 inflammasome and NLRP3-related autoinflammatory diseases: From cryopyrin function to targeted therapies

open access: yesFrontiers in Immunology, 2022
The NLRP3 inflammasome is one of the NOD-like receptor family members with the most functional characterization and acts as a key player in innate immune system, participating in several physiological processes including, among others, the modulation of ...
Chiara Moltrasio   +5 more
doaj   +1 more source

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