Clinical, biological and evolutional profile of beta-thalassemia in Kairouan. About 55 cases

ABSTRACT

The authors report a retrospective study concerning 35 cases of beta-thalassemia major, collected in the pediatric service of Ibn El Jazzar Hospital in Kairouan between January 1987 and June 2004. This disease represents 1.19% of hospitalisations. The middle age of diagnosis is of 22 months [3 months - 6 years] with a sex-ratio of 0.6. Clinical symptomatology is dominated by paleness [100% of cases] and splenomegaly [97% of cases]. All patients were treated by long-term chronic blood transfusions with chelation therapy. Eleven partial splenectomies were practiced in ten patients. Three patients died at an average age of 7 years. The prenatal diagnosis, using the amniocentesis, was performed in five cases