A Case Report Of Congential Dysfibrinogenemia And Literature Review

Objective:This article analyzes a clinical data of a patient with hereditary abnormal fibrinogenemia in order to improve the level of knowledge,diagnosis,and treatment of the rare genetic disease-Congenital Dysfibrinogenemia.Methods:To discuss the clinical charactstics,diagnosis,and treatment of 1case patient with congenital dysfibrinogenemia,who was diagnosed,and treated in The First Affiliated Hospital of Guangxi Medical University.And made a literature review.Results : The preoperative examination confirmed the diagnosis of asymptomatic congenital dysfibrinogenemia.No preoperative fibrinogen replacement therapy or other related treatment was performed before brain surgery.There was no abnormal bleeding during and after the surgery,and postoperative recovery is well.Conclusions:The clinical charactstics of congenital dysfibrinogenemia are diverse,may not have any clinical symptoms,and may also manifest as bleeding or thrombosis.The diagnosis of the disease is based on family history,clinical charactstics,abnormal coagulation detection and molecular defect identification.The treatment of this disease should be based on the patient'spersonal history and family history,and adhere to the individualized principle.According to current opinion,there is no need to provide special treatment for asymptomatic congenital dysfibrinogenemia patients with no hemorrhage or thrombosis personal history and family history.