Screening for beta-thalassaemia carriers in Egypt: significance of the osmotic fragility test

To estimate beta-thalassaemia carrier rate and to determine an accurate mass screening test, we tested 1000 randomly selected children aged 5-16 years from different geographical areas of Egypt. Microcytosis was present in 412 participants. The osmotic fragility test was positive in 81.1% of the 90 beta-thalassaemia carriers; in the indeterminate group [12 participants], the test was positive in 83.3%; in the 310 who were iron deficient, the test was positive in 63.9%. beta-thalassaemia carrier rate was >/= 9%. Serum iron, microcytosis, HbA2 level and transferrin saturation were accurate tests for detecting carriers. For the one-tube osmotic fragility test, sensitivity was 87.0% and specificity 34.1%; the test has limited use for a mass screening programme in Egypt, where iron deficiency is prevalent

Effects of L-carritine on grawth of Egypt children with sickle cell anaaenia

Growth improvement on L-Garnitine therapy has been observed in transfusion dependent sickle cell anaemia patients. We plan to study 27 Egyptian sickle Jell patients on low transfusion regimen for 6 months follow-up study period. The study included .13 males and 14 females aged [546 years]. Anthropometric measurements were done before starting L-Carnitine and six months after L-Carnitine [50 mg/kg orally]. Measurements included weighs, height, body mass index, skeletal muscle development and measurements of body fat. Twenty patients [10 males and 10 females.] of the same age and social classes were followed in the study as a control. Patients were divided according to their ages into 2 groups [5-10 years] and [11-26 years,]. The study showed increase in the standard deviation score [SDS] of weight and height per age of the supplemented children compared to control groups. There were increase in the chest/head ratio [C/H R], deltoid circumference, mid arm muscle circumference, mid arm fat circumference and subscapaular and supra iliac skin fold thicknesses compared to controls. These results were more apparent in the young age groups. We concluded the beneficial effect of L-Carnitine on the physical growth of patients with sickle cell anaemia especially the young, a longer period of supplementation is recommended

Profile of hepatitis B and C in multitransfused infants and children

72 multitransfused infants and children and 21 age matched healthy control subjects were subjected to thorough physical examination and laboratory investigations including hemogram, liver function tests and hepatitis B and C markers. Sixty two cases [86.1%] had HCV antibodies. 44 cases [66.1%] had at least one of the hepatitis B markers: 12 cases [16.7%] were hepatis surface antigen positive [HBsAg], 30 cases [41.6%] were positive for anticore antibodies [Anti HBe], 21 cases [41.6%] were positive for hepatitis B surface antigen antibodies [Anti HBs]. 6 cases [8.3%] were immune to hepatitis B, five of them were HGV antibody positive, 3/5 had mostly active HCV infection. 5 cases were negative for both hepatitis B and C. 12 cases [16.7%] suffered chronic liver affection. The lowering incidence of hepatitis B markers might be due to the recent screening of blood products for hepatitis B. The study showed a higher incidence of hepatitis C virus antibodies compared with other studies as HCV screening is not yet established in all blood banks, also the risk for HCV infection increased in relation to the number of blood units received. So, the introduction of donor screening for anti HCV will lower the frequency of transfusion associated hepatitis