Medicina clinica
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Histiocytosis is a group of rare diseases characterized by inflammation and accumulation of cells derived from monocytes and macrophages in different tissues. The symptoms are highly variable, from mild forms with involvement of a single organ to severe multisystem forms that can be life compromising. ⋯ During the last decade, some mutations have been identified in the affected tissue that condition activation of the MAPK/ERK and PI3K/AKT pathway, in a variable proportion depending on the type of histiocytosis. In this review we mainly focus on Langerhans Cell Histiocytosis, Erdheim-Chester Disease and Rosai-Dorfman Disease.
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Antineutrophil cytoplasmic antibody-associated vasculitides are primary vasculitides that affect small vessels in various organs, including the kidney. Renal involvement is characterized by the presence of glomerulonephritis with crescents and necrosis in light microscopy and a pauci-immune pattern in immunofluorescence. The participation of complement in the pathogenesis of these entities has been valued in recent years, initially in animal models and later in studies in humans, by demonstrating the presence of fragments of the alternative complement pathway, in plasma and urine, together with complement deposits in glomeruli and small vessels of patients affected by antineutrophil cytoplasmic antibody vasculitis. The presence of complement in these entities confers a worse general and renal prognosis.
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Peripheral nerve blocks have been a common treatment for multiple headaches. By far, the greater occipital nerve block is the most used and with the stronger body of evidence in routine clinical practice. ⋯ Greater occipital block is an effective and safe technique, easy to perform and which has shown its usefulness in migraine, cluster headache, cervicogenic headache and Post-dural puncture headache. However, more studies are needed to clarify its long-term efficacy, its place in clinical treatment, the possible difference between different anaesthetics, the most convenient dosage and the role of concomitant use of corticosteroids.
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Myasthenia gravis is an autoimmune disease caused by the presence of specific antibodies targeting different postsynaptic components of the neuromuscular junction, and is clinically characterized by the presence of fatigueable muscle weakness. In the etiopathogenesis plays a central role the thymus and the most frequently detected pathogenic autoantibodies are targeted to the acetylcholine receptor. The increase in the knowledge of the immunological components of the neuromuscular junction in the last two decades has been fundamental to identify new pathogenic antibodies, reduce the percentage of patients with seronegative myasthenia, and propose a classification of patients into subgroups with clinical-therapeutic interest. In addition, in recent years, new drugs have been developed for the treatment of patients with myasthenia gravis that are refractory to conventional immunosuppressive treatment.