S. Li Volti

Immune status and the immune response to hepatitis B virus vaccine in thalassemic patients after allogeneic bone marrow transplantation

We evaluated the immune status with respect to HBV and the immune response to readministration of HBV vaccine in a series of 20 patients with homozygous β-thalassemia, aged 6–23 years (mean age: 13.0 ± 4.2) who had undergone allogeneic bone marrow transplantation (BMT). Thirteen of them (group A), had received three doses of plasma-derived HBV vaccine from 7 to 5 years before BMT and 4–5 weeks after the last dose of vaccine, they had had high serum levels of HBV antibodies (anti-HBs). The remaining seven patients (group B) had had clinical symptoms and laboratory evidence of HBV infection in childhood with markedly elevated serum of anti-HBs. Before revaccination, a significantly lower percentage of patients (P < 0.005) with seropositive levels of anti-hbs was observed in group a than in group b. after administration of the second dose of hbv vaccine the percentage of subjects with protective levels of anti-hbs rose to 100% in both groups of patients even if the geometric mean of titers of anti-hbs increased more significantly in group b patients than in group a. we conclude that the serum levels of anti-hbs afforded by hbv vaccine administered from 7 to 5 years previously are very low and probably non-protective in most β-thalassemic patients after allogeneic BMT, and that at least two doses of HBV vaccine should be readministered from 18 to 24 months after BMT to achieve adequate and long-term protection from HBV.

Safety and effectiveness of an acellular pertussis vaccine in subjects with Down's syndrome.

We evaluated the reactogenicity and immunogenicity of an acellular pertussis vaccine in 24 subjects affected by Downs syndrome and in 10 normal infants. Neither general nor local adverse reactions were observed in either group of subjects. The new acellular vaccine administration elicited protective levels of antibodies in all the subjects with Downs syndrome, although the geometric mean titres of IgG antibodies againstBordetella pertussis in these subjects were significantly lower than in normal controls.

Glucose-6-phosphate dehydrogenase deficiency and incidence of cataract in Sicily

The incidence of G6PD deficiency in red blood cells of 241 Sicilian cataractous patients (138 males and 103 females) and in the lens of 32 subjects (15 males and 17 females) of the same group was evaluated. The incidence of G6PD deficiency was significantly higher than expected (p less than 0.001), both in RBCs and in lens. The results suggest that G6PD deficiency is a risk factor for cataract both in hemizygous males and heterozygous females.

Immune response to simultaneous administration of a combined measles, mumps and rubella vaccine with booster doses of diphtheria-tetanus and poliovirus vaccine

A combined vaccine against measles (Edmonston-Zagreb 19 strain), mumps (Rubini strain) and rubella (Wistar RA 27/3 strain) was administered to a group of 46 children aged 10–12 months simultaneously with booster doses of compulsory diphtheria-tetanus toxoid and oral poliovirus vaccine. A second group of 53 children aged 15–24 months who had received booster doses of the compulsory vaccines 5 to 12 months before was also vaccinated.The same seroconversion rates (100%) and similar antibody titers for rubella were observed in both groups. The same seroconversion rates for mumps (93%) and similar rates for measles (98 and 94%) were observed in the two groups.Significantly lower antibody titers for measles and mumps were found in the first group, but they were compensated by an earlier protection, a reduction of number of visits for immunization, costs for the community, and improvement in parental compliance.These results confirm that Edmonston-Zagreb 19 and Rubini strains are still immunogenic even when they are combined with Wistar RA 27/3 strain. Moreover, a long term follow-up in order to verify the persistence of protective antibody levels in both groups of children, could suggest that combined measles, mumps and rubella vaccine could be given earlier (at 10–12 months of age), simultaneously with booster doses of diphtheria and tetanus toxoid and of trivalent oral poliovirus vaccine.

Impaired antioxidant defense mechanisms in two children with hemolytic-uremic syndrome.

In the present study we have assayed antioxidant enzymatic activities of SOD, CAT, GSH-Px, GSH-Red, and G6PD in erythrocytes from two children with hemolytic-uremic syndrome (HUS) during the acute phase of the disease and after their recovery; in addition, we have tested the percentage of hemolysis after 24-h incubation in PBS containing glucose (1 g/1000 mL) or in the presence of their own plasma. Endogenous plasmatic MDA levels were also evaluated as lipid peroxidation marker. A significant decrease in SOD activity was found in erythrocytes from HUS patients, and the addition of their own plasma further decreased SOD activity. Elevated percentage of hemolysis was found in HUS patients when RBCs were incubated in their own plasma; this last effect was less evident in PBS + glucose.

Clinical and biochemical reactivation of HBV infection in a thalassemic patient after bone marrow transplantation

SummaryThe case of a young man effected by homozygous beta-thalassemia is reported who had serologic findings of a prior HBV infection and who presented with clinical and biochemical acute HBV infection probably caused by HBV reactivation after allogeneic bone marrow transplantation. The patients clinical history suggests that HBV can persist without serological findings of HBsAg and HBV-DNA in persons previously infected by HBV and that HBV reactivation can occur 2 years after allogeneic bone marrow transplantation, as a result of immunosuppressive therapy or an HCV activation.

Duration of hepatitis B antibody response in children immunised with hepatitis B and compulsory vaccines.

Twenty four subjects were simultaneously administered DT toxoids, OPV and HBV vaccines at the age of 3, 4–5 and 11 months and then followed up for 2 and 4 years in order to evaluate the duration of the immune response and the need and the timing of HBV revaccination. A fall in anti-HBs titre below 10 mIU/ml was observed at the follow up in 4/24 (16.7%) of the subjects. In other 5 children (20.8%) anti-HBs titre was found to be just above 10 mIU/ml. This would suggest that a revaccination is indicated and it could be performed at the age of 5–6 years when children enter school. This schedule is simple, effective and money saving since it reduces the cost/benefit ratio and the number of visits for immunisations, and it is expected to improve the compliance for the vaccination.

Cephalosporin-induced Hemolytic Anemia in a Sicilian Child

A 27-month-old child developed acute hemolysis on two occasions after the administration of cephalosporin. On the first occasion, hemolysis was intravascular and was due to the formation of complexes between antibodies and the drug, which bound to red blood cells and caused severe hemolysis. On the second occasion, hemolysis was extravascular and was probably due to antibody-dependent cell mediated cytotoxicity. Marked increases in levels of CD19+, and CD57+ CD8+ cells were detected among the subpopulations of the patients lymphocytes but only in the level of CD19+ cells from the patients father, after incubation of a sample of whole blood with a solution of cephalosporins. These results might explain the differences between the immune response of the patient and those of other members of his family and of an unrelated control.

Evidence for the Single Origin of Hb G-San Jose in Sicily

Hb G-San Jose [alpha 2 beta 2 7(A4)Glu----Gly] was detected in four families and three unrelated individuals from Eastern Sicily. Polymorphic restriction sites within the beta-globin gene cluster bearing the mutation were characterized. A complete association of beta-G-San Jose alleles with Mediterranean haplotype IV was found in the families examined and the same haplotype was also present in the unrelated individuals. These findings support the hypothesis of an unicentric origin of the beta-G-San Jose mutation which may have arisen in Eastern Sicily.

Superoxide dismutase activity and reduced glutathione content in cataractous lens of patients with glucose-6-phosphate dehydrogenase deficiency

In the present research we have assayed the glucose-6-phosphate dehydrogenase, superoxide dismutase activities and reduced glutathione content in human cataractous lenses of 83 Sicilian subjects. Five of 45 males were G6PD deficient, whereas eight of 38 females showed a significant reduction in G6PD by 50%. The five males hemizygous for G6PD defect showed undetectable G6PD activity and low GSH levels in their lenses when compared to cataractous patients without erythrocyte G6PD deficiency; on the contrary, the specific activity of lenticular total SOD was found to be significantly increased. The G6PD and SOD activities as well as GSH levels, in the lenses of eight females with intermediate erythrocyte G6PD levels, were not significantly different when compared to females with normal erythrocyte G6PD activity.