Arthritis &Rheumatology, EarlyView.Objective Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel vasculitis characterized by eosinophilia, asthma, and ear, nose, and throat (ENT) involvement. Although glucocorticoids (GCs) are effective in controlling symptoms, relapses and GC dependence are common. The aim of this study was to develop predictive models for vasculitis
Matthias Papo +35 more
wiley +1 more source
Deficiency of Adenosine Deaminase 2 Presenting as Periodic Fever at the Mainland of Familial Mediterranean Fever. [PDF]
J Clin Immunol, 2022 Çakan M, Sözeri B.
europepmc +1 more source
Arthritis &Rheumatology, EarlyView.Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller +16 more
wiley +1 more source
Spontaneous, simultaneous bilateral osteonecrosis of the femoral heads in a patient with sideroblastic anaemia with B-cell immunodeficiency, periodic fever and developmental delay syndrome. [PDF]
BMJ Case Rep, 2023 Cai G, Jayaraman D.
europepmc +1 more source
The 3‐Hit Metabolic Signaling Model for Autism Spectrum Disorder: A Summary
Autism Research, EarlyView.ABSTRACT Autism spectrum disorder (ASD) is a highly heritable yet environmentally sensitive neurodevelopmental condition whose biological heterogeneity has resisted a unifying causal explanation for over 100 years. The 3‐hit metabolic signaling model proposes that ASD arises from abnormal persistence of an evolutionarily conserved stress‐response ...
Robert K. Naviaux
wiley +1 more source
Familial Mediterranean fever in northwest of Iran (Ardabil): The first global report from Iran [PDF]
Familial Mediterranean fever (FMF), which is the prototype of the hereditary periodic fever syndromes, is common in the countries around the Mediterranean Sea. Considering its geographical position in the northwest of Iran, with its population of Turkish
امامی, دینا +10 more
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＜Editors' Choice＞ Myelodysplastic syndrome with trisomy 8 presenting periodic fever and multiple MEFV gene variants outside exon 10: a case report. [PDF]
Nagoya J Med Sci, 2023 Takahashi N +10 more
europepmc +1 more source
British Journal of Clinical Pharmacology, EarlyView.With multiple disease‐modifying therapies now available, treatment switching has become an important clinical consideration in the management of spinal muscular atrophy (SMA). While some switches are prompted by suboptimal clinical response, more commonly they are driven by treatment burden, convenience, or adverse events.
Andrej Belančić +4 more
wiley +1 more source
Increased Frequency of Febrile Seizures in Two Periodic Fever Syndromes: Familial Mediterranean Fever and PFAPA Syndrome. [PDF]
Turk Arch Pediatr, 2022 Kılıç H +7 more
europepmc +1 more source
Navigating adverse immunostimulation: A practical guide for clinical researchers
British Journal of Clinical Pharmacology, EarlyView.Problem Setting As drug development moves towards more complex products, early clinical development programmes are increasingly hampered by unwanted and/or unexpected activation of the immune system (adverse immune stimulation, AIS). Solution At the Centre for Human Drug Research, we have introduced standardized procedures to make AIS manageable, while
Juliette A. van den Noort +3 more
wiley +1 more source