Neutropenia hereditary

Neutropenia hereditaryNEUTROPENIA HEREDITARY - a group of rare hereditary diseases with an almost complete absence of blood neutrophils detected continuously (persistent neutropenia) or at regular intervals (periodic neutropenia). As a rule, the disease is inherited recessive. The pathogenesis of primary mechanisms of neutropenia is poorly understood infectious inflammatory processes due to neutropenia. The clinical picture is prone to infection (usually staphylococcal), severe alveolar Peoria since childhood (naked necks of teeth, loosening and loss). When the periodic form of the disease - fever and infection is strictly periodic and correspond to the days of neutropenia. In blood almost completely devoid of neutrophils, increased number of eosinophils and monocytes. When the periodic form these changes persist for several days, appearing through a well-defined periods of time. Anemia, thrombocytopenia no. In bone marrow-breakage maturation of neutrophils at the stage of promyelocyte (less myelocyte), monocytosis (children often many promonocytes), eosinophilia. Treatment. Antibiotics in the presence of infectious complications, continuous therapy alveolar Pirai. The forecast. Elimination of infections allows you to save the lives of sick children; over the years, the severity of the disease weakens. Prevention. When the periodic form prescribed antibiotics (e.g., oxacillin) before the crisis, allowing you to drastically lessen the severity of infection..



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