Results 21 to 30 of about 24,492 (179)

Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative

Pediatric Rheumatology Online Journal, 2020
Background Rare autoinflammatory diseases (AIDs) including Cryopyrin-Associated Periodic Syndrome (CAPS), Tumor Necrosis Receptor-Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency Syndrome (MKD)/ Hyper-IgD Syndrome (HIDS) are ...
Sandra Hansmann, Elke Lainka, Gerd Horneff, Dirk Holzinger, Nikolaus Rieber, Annette F. Jansson, Angela Rösen-Wolff, Gabi Erbis, Martina Prelog, Juergen Brunner, Susanne M. Benseler, Jasmin B. Kuemmerle-Deschner   +11 more
doaj   +1 more source

State of care for patients with systemic autoinflammatory diseases – Results of a tertiary care survey

World Allergy Organization Journal, 2019
Background: Systemic autoinflammatory diseases (SAIDs) are rare debilitating disorders of which there is limited awareness and a significant delay in diagnosis.
Mati Chuamanochan, Karsten Weller, Eugen Feist, Tilmann Kallinich, Marcus Maurer, Jasmin Kümmerle-Deschner, Karoline Krause   +6 more
doaj   +1 more source

High-Dose Hydrocortisone Treatment Does Not Affect Serum C-Reactive Protein (CRP) Concentrations in Healthy Dogs

Veterinary Sciences, 2023
Measuring C-reactive protein (CRP) in serum is a useful surrogate marker for assessing disease progression and treatment response in dogs with autoinflammatory diseases.
Romy M. Heilmann, Niels Grützner, Peter H. Kook, Stefan Schellenberg, Jan S. Suchodolski, Joerg M. Steiner   +5 more
doaj   +1 more source

Peri- and Postoperative Treatment with the Interleukin-1 Receptor Antagonist Anakinra Is Safe in Patients Undergoing Renal Transplantation: Case Series and Review of the Literature

Frontiers in Pharmacology, 2017
In patients undergoing solid organ transplantation, the presence of an interleukin-1 (IL-1) driven disease may require the addition of IL-1 inhibiting drugs to the standard immunosuppressive regimen to protect against inflammation and negative graft ...
Catharina M. Mulders-Manders, Marije C. Baas, Femke M. Molenaar, Anna Simon   +3 more
doaj   +1 more source

Out of Control: The Role of the Ubiquitin Proteasome System in Skeletal Muscle during Inflammation

Biomolecules, 2021
The majority of critically ill intensive care unit (ICU) patients with severe sepsis develop ICU-acquired weakness (ICUAW) characterized by loss of muscle mass, reduction in myofiber size and decreased muscle strength leading to persisting physical ...
Stefanie Haberecht-Müller, Elke Krüger, Jens Fielitz   +2 more
doaj   +1 more source

Topical inflammasome inhibition with disulfiram prevents irritant contact dermatitis

Clinical and Translational Allergy, 2021
Background The pathogenesis of contact dermatitis, a common inflammatory skin disease with limited treatment options, is held to be driven by inflammasome activation induced by allergens and irritants.
Hanna Bonnekoh, Carolina Vera, Angela Abad‐Perez, Silke Radetzki, Martin Neuenschwander, Edgar Specker, Niklas Amadeus Mahnke, Stefan Frischbutter, Eicke Latz, Marc Nazaré, Jens v. Kries, Marcus Maurer, Jörg Scheffel, Karoline Krause   +13 more
doaj   +1 more source

The paradox of autoimmunity and autoinflammation in inherited neutrophil disorders – in search of common patterns

Frontiers in Immunology, 2023
Congenital defects of neutrophil number or function are associated with a severe infectious phenotype that may require intensive medical attention and interventions to be controlled.
Damian Krzyzanowski, Damian Krzyzanowski, Aleksandra Oszer, Joanna Madzio, Maciej Zdunek, Julia Kolodrubiec, Bartosz Urbanski, Wojciech Mlynarski, Szymon Janczar   +8 more
doaj   +1 more source

Somatic Mutations and the Risk of Undifferentiated Autoinflammatory Disease in MDS: An Under-Recognized but Prognostically Important Complication

Frontiers in Immunology, 2021
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 ...
Abdulla Watad, Abdulla Watad, Mark Kacar, Mark Kacar, Nicola Luigi Bragazzi, Qiao Zhou, Qiao Zhou, Miriam Jassam, Jan Taylor, Eve Roman, Alexandra Smith, Richard A. Jones, Howard Amital, Catherine Cargo, Dennis McGonagle, Sinisa Savic, Sinisa Savic   +16 more
doaj   +1 more source

Isolated neurological presentations of mevalonate kinase deficiency

JIMD Reports, 2023
Mevalonate kinase (MK) deficiency is a rare autosomal recessive metabolic disorder caused by pathogenic variants in the MVK gene with a broad phenotypic spectrum including autoinflammation, developmental delay and ataxia. Typically, neurological symptoms
Eva M. M. Hoytema van Konijnenburg, Esmeralda Oussoren, Joost Frenkel, Peter M. van Hasselt   +3 more
doaj   +1 more source

Mitochondrial Nucleic Acid as a Driver of Pathogenic Type I Interferon Induction in Mendelian Disease

Frontiers in Immunology, 2021
The immune response to viral infection involves the recognition of pathogen-derived nucleic acids by intracellular sensors, leading to type I interferon (IFN), and downstream IFN-stimulated gene, induction.
Alice Lepelley, Timothy Wai, Yanick J. Crow, Yanick J. Crow   +3 more
doaj   +1 more source