Le sindromi autoinfiammatorie ereditarie in eta’ pediatrica

[Autom. eng. transl.] The hereditary autoinflammatory syndromes constitute a group of affections of innate immunity, characterized by the periodic recurrence of febrile episodes that last from days to weeks and are accompanied by signs of multi-district inflammation of variable gravity affecting the skin, the serous, of the tube gastrointestinal, osteo-articular system, etc. The vast majority of these conditions are caused by mutations in genes involved in the control of inflammation and apoptosis mechanisms. The group includes hereditary periodic fevers, pyogenic disorders, and immune-mediated granulomatous diseases. The diagnostic identification of these conditions derives from the integration of anamnestic-clinical and biohumoral data, but can in some cases be supported by genotypic studies.