Brief Overview about Inherited Rare coagulation disorders

Document Type : Review Articles

Authors

1 Pediatrics Department, Faculty of Medicine, Zagazig University

2 pediatric department, faculity of medicine, zagazig university

3 Public Health and Community Medicine Department, Faculty of Medicine, Zagazig University

Abstract

Background: Numerous rare congenital bleeding disorders include fibrinogen and prothrombin deficiency as well as factor (V, VII, VIII, XI, X, and XIII) deficiency. In the general population, the severe variants of these illnesses are hereditary and possess a frequency that falls somewhere between one in every two million for factor XIII and one in per half a million for factor VII. Patients who have rare congenital bleeding disorders may exhibit a broad range of symptoms, from relatively mild subcutaneous bleeding to potentially life-threatening hemorrhages for example in the central nervous system. The majority of treatment for these disorders when specific plasma-derived or recombinant products are available is to replace the missing factor. Diagnosing and treating rare congenital bleeding disorders can be challenging due to the wide variety of symptoms they manifest in patients. Patients with rare inherited bleeding diseases must be closely monitored at specialized hemophilia treatment centers once they are diagnosed, due to the unique difficulty of their therapy. More research is required to improve the worldwide care for individuals with rare inherited bleeding diseases, despite the fact that a lot has been learned about their frequency, symptoms, and genetic characteristics in the past ten years. We aimed at this review to give a brief overview about Inherited Bleeding Disorders.

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Zagazig University Medical Journal
Volume 30, Issue 7
October 2024
Pages 3414-3422

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