Epidemiological profile of Inherited Bleeding Disorders in Egyptian Pediatric Patients: 4 Centers Experience, Retrospective Analysis

Faculty Medicine Year: 2021
Type of Publication: ZU Hosted Pages: 14152-14165
Authors:
Journal: Annals of the Romanian Society for Cell Biology Association of Cell Biology Romania Volume: 25
Keywords : Epidemiological profile , Inherited Bleeding Disorders , Egyptian Pediatric    
Abstract:
Purpose:In Egypt, wedon't have a registry for inheritedbleedingdisorders. Geneticdiseases do not receiveany public health support becausethey are considered rare conditions withlowprevalence. Furthermore, actualstatistics on the demography of geneticdisorders in the Egyptian population are largelyunavailable. So The purpose of thisstudyis to generatebaselineepidemiological information on the status of IBDsin 4 Egyptianpediatric centers. Patients and methods:A cross-sectional study of patients' records, including732patients of pediatric age (from birth to 18 years) with inherited bleeding disorders in 4 EgyptianPediatricHospitals. Results:Medianage of the studied patients was 9.0 yearsold. Most of the studied patients were males (87.8%), almosthalf of themwere rural residents (56.7%). Only 23.2% had a positive familyhistory, and 16.3% had positive consanguinity. The age of first bleedingwaslessthan one yearoldin 47.4% of the studied patients. Ecchymosis (57.2%), bleedingwithcircumcision (29.9%), and bleedingwith minor trauma (24.6%) were the mostfrequentclinical manifestations of bleeding. The mostfrequentbleedingdisorder in the studied patients wasHemophilia A (61.2%), followed by Hemophilia B (15.4%), Plateletdisorders (7.1%), VWD (6.4%), and factor VII deficiency (4.1%). Hemophilia and coagulation disordersotherthan VWD hadhigher proportions of male sexcompared to otherIBDs. Plateletdisordershadhigher proportions of rural residents, positive familyhistory, and positive consanguinitythanotherIBDs. Hemophilia and coagulation disordersotherthan VWD wereassociatedwith a youngage of first bleeding (lessthan one-year-old), hemarthrosis, ecchymosis, and bleedingwithcircumcision. Plateletdisorderswereassociatedwithepistaxis and Bleedingwithcircumcision Conclusion : The mostcommonIBDs in ourlocality are hemophilia A, followed by hemophilia B, and Plateletdisorders. Consanguineousmarriageisfrequent in Egypt, with a high prevalence of inheritedbleedingdisorders in pediatricage. VWD and
   
     
 
       

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