Descriptive Epidemiology of Hemophilia and Other Coagulation Disorders in Mansoura , Egypt

Abstract

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 orthopedic 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-diagnosed hemophilia 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 the factor activity level was 6–30%, moderate if 1–5% and severe if <1% of normal.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 Hemophilia 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 8.3%. Hepatitis 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, From this study we can conclude that the most common IBDs in our locality is hemophilia A followed by hemophilia B. The common presenting symptom was bleeding following male circumcision. Hepatitis C infection and arthropathy represented the main complications. The discovery of IBDs in young age children with proper supportive therapy could prevent arthropathy. Proper screening of blood and blood products reduce the risk of viral hepatitis and HIV acquisition.

Lack of Knowledge About Hepatitis C Infection Rates Among Patients With Inherited Coagulation Disorders in Countries Under the Eastern Mediterranean Region Office of WHO (EMRO): A Meta-Analysis

“…In addition, we excluded an article because of its selection bias and overestimation as it was reported previously (32), although there was enough data and an acceptable sample size (33). Three other studies were excluded because only PCR was performed to test for HCV infection (11,34,35). Finally, 30 studies were selected for the analysis (19)(20)(21).…”

“…Moreover, most of the recently admitted HCC patients had viral hepatitis C (10). Hemophilia is the most common inherited congenital bleeding disorder and it is estimated to affect between 1 to 5 individuals per 50,000 male population (11). Hepatitis C is considered responsible for post-transfusion hepatitis and most of the observed liver diseases in treated hemophilia patients (12).…”

Challenges to laboratory hematology practice: Egypt perspective

“…An overview of the current status of the common hereditary blood diseases practice is given in Table …”

Outcome of harmonic scalpel circumcision in children with hemophilia

“…6 In societies where male circumcision is a routine, as in Egypt, bleeding following male circumcision is the most common presenting symptoms of children with hemophilia. 7 Bleeding in hemophiliacs can lead to death especially in developing countries where coagulation factor replacement therapy is poorly developed or not available.…”