RESUMEN
Congenital fibrinogen deficiency is an inherited disorder due to genetic mutations with diverse presentations arising from reduced fibrinogen levels (hypofibrinogenemia), absence of fibrinogen in circulation (afibrinogenemia), abnormal functioning (dysfibrinogenemia) or both reduced levels and abnormal functioning (hypodysfibrinogenemia) of fibrinogen. The decreased fibrinogen concentration in congenital fibrinogen deficiency necessitates fibrinogen replacement therapy with fresh frozen plasma, cryoprecipitate, or human fibrinogen concentrate. However, the use of fresh frozen plasma and cryoprecipitate is limited owing to their longer transfusion time, requirement of high doses, volume overload, risk of viral transmission, and other safety concerns. The availability of human fibrinogen concentrate has made it the preferred replacement alternative due to its reduced risk of viral transmission, smaller infusion volume, and accurate dosing. The hemostatic efficacy and safety of human fibrinogen concentrate in congenital fibrinogen deficiency is well established in the literature. We review the prevalence of congenital fibrinogen deficiency in India and the current role of human fibrinogen concentrate in its management.
Asunto(s)
Afibrinogenemia/terapia , Fibrinógeno/uso terapéutico , Afibrinogenemia/tratamiento farmacológico , Afibrinogenemia/patología , Transfusión Sanguínea , Fibrinógeno/química , Guías como Asunto , Humanos , India , Plasma/químicaRESUMEN
Kimura's disease is a chronic, benign inflammatory condition of the subcutaneous tissue. It presents as painless, subcutaneous nodules of the head and neck, which are firm, painless, and may be single or multiple. It is most commonly seen in young adult Asian men. The nodes remain stable or may slowly enlarge over time. Elevated serum immunoglobulin E (IgE) levels, peripheral blood eosinophilia and lymphoid proliferation with eosinophilic infiltration on histopathological examination are the characteristic features. Spontaneous regression is usually seen. The diagnosis of Kimura's disease can be difficult and misleading, and it is important not to ignore histopathological features.