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Subject Area

Pediatrics

Article Type

Original Study

Abstract

Objective The aim was to study the inherited bleeding disorders other than hemophilia with special emphasis on clinical spectrum, diagnosis, and management. Background Inherited bleeding disorders are a variable group of disorders that vary in incidence and clinical presentations. They include hemophilia A, hemophilia B, von Willebrand disease (VWD), inherited platelet-function disorders (IPFDs), and rare bleeding disorders (factors I, II, V, combined Vand VIII, VII, X, XI, and XIII deficiencies). Patients and methods It is a prospective follow-up study over a period of 5 years, in which patients suffering from bleeding tendency and referred to the hematology clinic were included and prospectively followed up. They were subjected to detailed history, full examination, and laboratory investigations; complete blood count, prothrombin time, and activated partial thromboplastin time; and accordingly other investigations were done, such as factor assay and platelet-function tests. Results The authors reported 52 patients with inherited bleeding disorders other than hemophilia. Among them, IPFDs were the most common (38.5%), followed by VWD (26.9%). While 34.6% were diagnosed as rare bleeding disorders (FI, V, VII, X, and XIII deficiencies). Conclusion IPFDs were the most common inherited bleeding disorders other than hemophilia followed by VWD. Among the rare bleeding disorders, FI deficiency was the most common, while FXIII and FVII deficiency were the most serious. Also, fresh frozen plasma was the most frequent therapeutic product used to control bleeding episodes in our patients.

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