Results 61 to 70 of about 2,294 (167)

Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease

The Journal of Dermatology, Volume 52, Issue 3, Page 400-407, March 2025.
Abstract Generalized pustular psoriasis (GPP) is a severe autoinflammatory keratinization disease (AiKD) characterized by acute flares of widespread sterile pustules and high fever. GPP is potentially life‐threatening. Recently clarified genetic predisposing factors for GPP suggest that the excessive activation of innate immune pathways in the skin ...
Masashi Akiyama
wiley   +1 more source

Long-term follow up of Hyper IgD syndrome: a national collaborative study

, 2011
The paper deals with long-term follow up of children with hyper IgD syndrome in a large national collaborative ...
Rigante, D
core  

Publication Only

HemaSphere, Volume 10, Issue S1, June 2026.
wiley   +2 more sources

Genetic and epigenetic dysregulation of innate immune mechanisms in autoinflammatory diseases

The FEBS Journal, Volume 291, Issue 20, Page 4414-4432, October 2024.
Dysregulation and hyperactivation of innate immune responses can lead to the onset of systemic autoinflammatory diseases, caused by inborn genetic errors alone, or in combination with non‐genetic factors. Innate immune memory (trained immunity) is physiologically beneficial, but can become maladaptive, perpetuating chronic inflammatory activation. Here,
Laura M. Merlo Pich, Athanasios Ziogas, Mihai G. Netea   +2 more
wiley   +1 more source

Mevalonate kinase genotype in children with recurrent fevers and high serum IgD

, 2013
In selected cases, childhood's recurrent fevers of unknown origin can be referred to systemic autoinflammatory diseases as mevalonate kinase deficiency (MKD), caused by mutations in the mevalonate kinase gene (MVK), previously named "hyper-IgD syndrome ...
Rigante, Donato
core   +1 more source

Hyperimmunoglobulinemia D Syndrome Masquerading as Familial Mediterranean Fever, Hidradenitis Suppurativa, and Crohn’s Disease: A Case Report

Saudi Journal of Medicine and Medical Sciences
We report a rare case of hyperimmunoglobulinemia D syndrome (HIDS) in a young man who was initially diagnosed with familial Mediterranean fever, hidradenitis suppurativa, and, eventually, with Crohn’s disease.
Mohamed Juraij, Farah Barakat, Zainab Ifthikar, Fahad Alsohaibani   +3 more
doaj   +1 more source

Hyper-IgD syndrome and other hereditary periodic fever syndromes

, 2004
Hereditary periodic fever syndromes are a group of systemic disorders characterized by recurrent attacks of systemic inflammation (autoinflammation) without infectious or autoimmune cause.
BOCCAFOGLI, Arrigo, VICENTINI, Licia, Scolozzi R.   +2 more
core  

Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome: introducing a vaccination provocation model. [PDF]

, 2005
Contains fulltext : 48588.pdf (Publisher’s version ) (Open Access)BACKGROUND: Hyper-IgD and periodic fever syndrome (HIDS) is an hereditary autoinflammatory syndrome, characterised by recurrent inflammatory attacks. Treatment of HIDS is
Bodar, E.J., Drenth, J.P.H., Simon, A., Meer, J.W.M. van der, Hilst, J.C.H. van der   +4 more
core  

Mevalonate kinase deficiency (hyper IgD syndrome with periodic fever)--different faces with separate treatments: two cases and review of the literature

The Turkish Journal of Pediatrics, 2012
The hyperimmunoglobulinemia D syndrome (HIDS), so-called mevalonate kinase deficiency, is caused by recessive mutations in the gene encoding mevalonate kinase enzyme.
Pınar Gençpınar, Balahan B Makay, Marco Gattorno, Francesco Caroli, Erbil Ünsal   +4 more
doaj  

Abstracts

Molecular Oncology, Volume 19, Issue S1, Page 1-940, June 2025.
Abstracts submitted to the ‘EACR 2025 Congress: Innovative Cancer Science’, from 16–19 June 2025 and accepted by the Congress Organising Committee are published in this Supplement of Molecular Oncology, an affiliated journal of the European Association for Cancer Research (EACR).
wiley   +1 more source