SummaryA 13-year-old Thai female with hemoglobin E-/3-thalassemia was evaluated for anemia and splenomegaly. Globin chain synthesis in a whole cell system revealed an absence of PA chains and excessive a chains. The a/PE + y ratio was 1.26 in bone marrow and 1.90 in peripheral blood. The average y/PE ratio in bone marrow and peripheral blood was 0.36 compared to peripheral blood concentrations of 49% hemoglobin E and 51% hemoglobin F. Homologous red cell 51Cr half-life increased from 22.7 days to 32.8 days after splenectomy. Total circulating hemoglobin increased from 112.9 to 149.7 g. Endogenous carbon monoxide productive (Vco) as a measure of total heme catabolism decreased from 2.00 to 1.54 pmol/hr/kg, Ineffective erythropoiesis was manifested by an increased Vco/Vheme., ratio of 7.52.
SpeculationSplenic sequestration may occur as a complicating factor in the anemia of hemoglobin E-P-thalassemia. The mechanism of this disorder is probably related to excessive a chain production and hemoglobin E instability.
The first licensed Haemophilus influenzae type B (Hib) vaccines were of a purified capsular oligosaccharide. Unfortunately, oligosaccharides are poorly immunogenic in younger children and resulted in these vaccines not routinely being used until past the period of peak incidence of Hib disease. Subsequent Hib vaccine research revealed that conjugating the oligosaccharide with various carrier proteins could produce vaccines with varying degree of improved immunogenicity. We investigated the antibody response and side effects of HibTITTER, one such vaccine, in 96 young infants in our multi-ethnic, border population. A three-dose course was given concomitantly with DPT and OPV vaccines at 2, 4, and 6 months of age. Seventy-nine percent of the infants had obtained antibody levels reportedly associated with clinical immunity after the second vaccination and 96% after the third vaccination. There was no significant difference in response due to sex, race, or vaccine lot. Currently HibTITER is recommended for use at 18 months of age. Our data strongly suggest that it may be effectively used much earlier, thereby immunizing infants against Hib disease before they reach their age of greatest vulnerability.
Despite optimal antibiotic therapy, H. b (Hib) meningitis often causes permanent sequelae and occasionally death. We examined the efficacy of immune globulin (IG) therapy alone or in combination with antibiotic therapy in the infant rat model of Hib infection. Two IG preparations were used: (1) Sandoglobulin (IVIG), at 5% protein, containing 25pg PRP Ablml and (2) Bacterial Polysaccharide Immune Globulin (BPIG) prepared from donors immunized with Hib vaccine, at 166 protein containing 450 fig PRP Ablml. Infant rats were challenged with 103 Hib ip resulting in 99% bacteremia and 88% meningitis at 18h. Therapy (Rx) given sc was Cefniaxone (CEF; 75mglkg qd), IVIG (15mVkg qd), BPIG (1SmVkg qd) and combinations of CEF + 113' s. Blood and CSF were cultured 24h and 72h after Rx.
Side-effects and immunogenicity of the Haemophilus influenzae type B polysaccharide vaccine (Hbpv) were reported following introduction in a multi-ethnic border population of 2- to 6-year-old U.S. military dependents. Eighty percent of 659 vaccinees were reported by parents to have no reactions within 48 hours following vaccination; 20% were reported to have local and/or systemic reactions. Each reaction was reported with one-fifth or less the frequency previously reported following DPT vaccination. Serologic data are presented on 114 of the vaccinees. The frequency of pre-vaccination levels reportedly associated with immunity ranged from a lower than expected 54% of 2-year-olds to an expected 86% of 5-year-olds. Post-vaccination levels reportedly associated with immunity ranged from a lower than expected 80% of 2-year-olds to an expected 93% of 5-year-olds. Of the 114 vaccines with serologic data, those with side-effects had serologic changes comparable to those without side-effects. There was no difference in side-effects or immunologic response across ethnic groups. Therefore, side-effects, immunologic responses, and ethnic group appear to be independent variables.
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