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Chronic infantile neurological cutaneous and articular (CINCA) syndrome is an autoinflammatory disease, defined by the triad of urticarial rash, neurological manifestations, and arthropathy, accompanied by recurrent fevers and systemic inflammation. Increasing neurological deficits result from aseptic meningitis. Sensorineural hearing loss and progressive(More)
Because of its distinct clinical and laboratory features, reflecting systemic inflammation, sJIA can be distinguished from other forms of JIA which usually present as a milder phenotype. The exact pathophysiology of sJIA, however, remains unknown. Profound dysregulation of innate pro- and anti-inflammatory cytokines, and rapid clinical response to cytokine(More)
Systemic onset juvenile idiopathic arthritis (SoJIA) is a rare inflammatory disorder. It can result in disease and treatment-related disability. SoJIA is characterized by remitting fevers, evanescent rash, generalized lymphadenopathy, hepatomegaly/splenomegaly, and/or serositis. Non-responsiveness to standard therapy with corticosteroids and disease(More)
We describe 2 patients with systemic juvenile idiopathic arthritis and macrophage activation syndrome. Treatment with recombinant interleukin 1 receptor antagonist (anakinra) and a corticosteroid rapidly induced remission, which could be maintained with anakinra monotherapy at a stable dose of 2 mg/kg per day. Pain at the injection site during the initial(More)
Cryopyrinopathies are a subgroup of autoinflammatory syndromes. Most cases have mutations in the CIAS1/NLRL3 gene, encoding the cryopyrin/NLRP3 protein. Cryopyrin, together with other proteins, is involved in the assembly of the cryopyrin/NLRP3 inflammasome. Mutations in CIAS1/NLRP3 result in increased IL-1β cleavage from biologically inactive pro-IL-1β.(More)
A 12-year-old girl developed influenza B virus infection proven by typical symptoms and detection of the virus in a nasopharyngeal swab by culture and PCR. Two weeks later she developed an otherwise unexplained transient oligoarthritis of small joints of the left foot. Influenza viruses may be a hitherto underappreciated cause of a post-infectious arthritis.
Dear Editor, To the best of our knowledge, this is the first report of the use of rasburicase in a pregnant woman with imminent tumour lysis syndrome (TLS) giving birth to a healthy child 2 days after treatment. TLS is a common complication in highly proliferative haematological malignancies and an oncologic emergency. Due to spontaneous lysis of malignant(More)
Chronic non-bacterial osteomyelitis (CNO) is an inflammatory, non-infectious disorder of the skeletal system with unknown aetiology. Therapeutic options are NSAIDs, steroids and DMARDs (MTX or sulfasalazine). However, a considerable number of patients have a severe disease course and bisphosphonates or TNF-a blockade might be a therapeutic option. We(More)
Endoscopic surgery has changed the philosophy and practice of modern surgery in all aspects of medicine. It gave rise to minimally invasive surgery procedures based on the ability to visualize and to operate via small channels. In maxillofacial surgery, our ability to see clearly the surgical field opened an entirely new world of exploration, as conditions(More)
Osteoidosteome sind schmerzhafte Tumoren vorwiegend des Kindes- und frühen Erwachsenenalters. Die genaue Pathogenese ist nach wie vor ungeklärt. Im Spontanverlauf zeigt sich eine Remission meist nach einem Zeitraum von mehreren Monaten bis Jahren. Dies wirft die Frage auf, ob immer eine chirurgische Intervention vonnöten ist. Aufgrund der schmerzbedingt(More)