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AFİBRİNOJENEMİ


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Medicine and Hematology Glossary

AFİBRİNOJENEMİ

(Medicine and Hematology Glossary) :
Otozomal resessif geçiş gösteren, fibrinojen yokluğu veya düzeyinin az olmasıyla (hipofibrinojenemi) karakterli bir herediter durumdur. Hipofibrinojenemi olguları heterozigotlardır. Herediter afibrinojenemi fibrinojen biyosentezindeki yetersizlik sonucu gelişir. Kanama eğilimi doğumdan itibaren vardır, umblikal korddan veya sünnet sonrası kanama olabilir. Diğer klinik bulgular arasında; önemsiz travmalar veya cerrahi sonrası kanama, subkutan hemoraji, epistaksis, diş çekimi sonrası kanama, yara iyileşmesinde defekt sayılabilir. Spontan dalak rüptürü, spontan hemoperikardium, masif over hemorajisi vakaları vardır. Hemartroz nadirdir. Kadınlarda menoraji görülebilir. Kanama zamanı, pıhtılaşma zamanı uzar. Fibrinojen paltelet agregasyonu için gereklidir. Bu nedenle çoğu hastada platelet fonksiyon bozukluğu vardır. Kanama epizodları plazma veya kriyopresipitat gibi fibrinojen kaynakları ile ile tedavi edilebilir.
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