RETROSPECTIVE STUDY OF INHERITED FVIII DEFIECENCY IN PEDIATRICS

Abstract

Haemophilia is the second most common bleeding disorder which is transmitted as an X-linked recessive disorder. This study was applied on patients attending the Hematology Clinic of the New Children Hospital ,aiming to do a retrospective study of inherited FVIII deficiency and evaluation of the effect of actual management regimen ,still depending on either plasma or cryoprecepitate. This study included 55 patients, all of them were subjected to full clinical examination ,routine hematological investigations (BT ,PTT ) FVIII activity assay ,VWF:Ag activity assay and FVIII binding activity assay. The study was conducted on 49 proposed haemophilia patients ,their ages ranged between (1.5-14 years) with a mean of , 4 females and 45 males ,they were compared to 6 age-matched VWD patients ,their ages ranged between (1-13 years) ,they were 2 females and 5 males . The results of the study divided the 49 patients into 2 groups : haemophilia group ,42 cases ,40 males (95.2%) and 2 females (4.8%), while the newly diagnosed group was 7 cases (16.6%) , 5 of them represented the quantitative type of VWD and the other 2 cases represented the qualitative type of VWD .