Journal: |
Annals of the Romanian Society for Cell Biology
Association of Cell Biology Romania
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Volume: |
25
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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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