Mediterranean Journal of Hematology and Infectious Diseases

Hemophilia represent the most severe inherited bleeding disorder (INB) , it’s thought to affect inviduals from all geographical areas in equal frequency. In Egypt which has a population of approximately (80million) consanguineous marriage are frequent, therefore autosomal recessive coagulation disorders reach a higher prevalence than in many other countries. The primary aim of this study was to describe the epidemiological situation of hemophilia in Mansoura, Egypt ,as based on retrospective analysis of clinical records Mansoura University Children Hospital between years 2000 and 2008. The second aim was to assess the o complications and occurrence of hepatitis C in those patients and relate this status to the type of replacement therapy received prior to the study. The study included 72 children with hematological disorders registered from 2000 to 2008 in MUCH. The hemophilic patient was defined as a person with physician A or B and a measured factor VIII or IX activity level of 30% or less. Persons with acquired inhibitors of FVIII or FIX excluded. Severity level was categorized as mild if activity level was 6–30%, moderate if 1 The severe presentation represents the majority in 76.7% followed by moderate severity in 17.2%.The commonest IBDs was hemophilia A affecting 44 patients, followed by Hemop B affecting 15 patients. The rare types were Factor XI deficiency, Factor V deficiency, Factor VII deficiency and combined FVIII, FIX and FX deficiency. The commonest orthopedic manifestation needing therapy was found among hemophilia A representing C viremia detected by PCR was found in 11.1% of patients. The bleeding complications as hematoma or hemarthrosis were the common complications. Nevertheless, 44.4% of patients had no complications, www.mjhid.org ISSN 2035-3006 f Hemophilia and Other Coagulation Disorders Analysis. 1 and Maysaa El Sayed Zaki. at no competing interests exist. ): e2010025, DOI 10.4084/MJHID.2010.025 ), which permits unrestricted use, distribution, and reproduction in any -diagnosed hemophilia –5% and severe if <1% of normal. in