الفهرس 
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Abstract
Inherited bleeding disorders are variable group of disorders that includes VWD, haemophilia A, haemophilia B, rare bleeding disorders and IPFDs. The rare bleeding disorders include FI, FII, FV, combined FV&VIII, FVII, FX, FXI and FXIII deficiencies.
The different inherited bleeding disorders differ in their clinical presentations ranging from asymptomatic or mild conditions as in IPFDs to life threatening bleeding as in FXIII deficiency.
The initial investigations used for screening of suspected bleeding disorders patients include full blood count, blood film microscopy, coagulation screen tests and Von Willebrand factor assays. Prolonged PT with normal APTT is suggestive of factor VII deficiency. Prolonged APTT with normal PT is suggestive of factor VIII, IX and XI deficiency. If both PT and APTT are prolonged suspect factor II deficiency, V deficiency, X deficiency or combined deficiencies such as combined factor V and factor VIII deficiencies. In case of normal PT and APTT, factor XIII deficiency and alpha‐2‐antiplasmin deficiency are suspected.
The available options for treatment of inherited bleeding disorders include: fresh frozen plasma, cryoprecipitate, platelet concentrate, factor concentrate and recombinant products. Fresh frozen plasma can be used in all types of coagulation factor deficiencies. It is the main choice in countries with restricted resources despite its high risk of blood borne infection transmission. Cryopericipitate can be used only in patients with factor I, VIII, XIII deficiencies and VWD. Factor concentrate is used in factor I, V, VII, VIII, X, IX, and XIII deficiencies. Recombinant products are not available for all coagulation factor deficiencies but available only for factor VII, VIII, IX, and XIII deficiencies and VWD.
The aim of the work is to study the inherited bleeding disorders other than haemophilia in Beni-Suef University hospital with special emphasis on clinical spectrum, diagnosis and management. It was a prospective follow up study in which all patients below 18 years old referred to haematology clinic at Beni_Suef univrsity hospital with bleeding tendency or abnormal coagulation profile were subjected to a detailed history and underwent laboratory investigations.
Our findings were: We reported 52 patients with inherited bleeding disorders other than hemophilia. IPFDs showed the highest distribution among them (38.5%), followed by von willebrand disease (26.9%). While (34.6%) were diagnosed as rare bleeding disorders (FI, V, VII, X and XIII deficiencies). FI deficiency (Afibrinogenemia) was the commonest among rare bleeding disorders representing 13.5%, followed by FV deficiency, FVII deficiency (each represented 7.7%), FX deficiency (3.8%), while FXIII deficiency ranked last (1.9%).
Based on data from this registry inherited platelet function disorders were the most common inherited bleeding disorders other than haemophilia in our study. Von Willebrand disease was the second most common. Inherited platelet function disorders are less severe than coagulopathies being usually presented with mucocutaneous bleeding episodes unlike coagulopathies which are usually presented with bleeding in deep internal sites. Fresh frozen plasma is still the most common theraputic modality available for treatment of bleeding episodes.