Biomedical subjects
Publications and source records attributed to A Orsini.
Hematological and clinical features of 36 mainly Algerian patients with S-beta thalassemia are reported. These data, compared with those reported in the literature, showed a higher prevalence of aseptic necrosis and gall stones, probably related to the large predominance of S-beta zero-thalassemia (30 cases) and a long (14 years) median follow-up period.
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Hb Bruxelles is a new beta-globin variant producing severe congenital Heinz body anemia. It results from the deletion of one of the two adjacent phenylalanines, beta 41 or beta 42, presumably by frameshift mutagenesis. Its whole blood oxygen affinity is significantly lowered.
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The development of cell-mediated immunological reactivity was studied in fetal lambs infected with Akabane virus. Examination of hepatic cells from fetuses between 40 and 75 days' gestation that had been infected via the transplacental route revealed inconsistent responses to Akabane, together with a uniform failure to respond to non-specific mitogens which contrasted with the behaviour of control, uninfected lambs. Following direct inoculation of fetal lambs with virus between 50 and 120 days' gestation, specific proliferative responses were observed on the part of the spleen cells from some. Direct challenge of fetal lambs of 4 months' gestation evoked cellular responses in lymph draining from the site of virus inoculation similar to those produced by challenge of adult sheep. The proliferative response of lymph-borne cells was substantially better if live, rather than inactivated, virus had been used.
An 18-year-old girl with a left parietal lesion caused by a closed-head injury exhibited a peculiar memory impairment. The patient showed selective "semantic amnesia" (severe loss of the stock of notions acquired prior to the disease), while autobiographic memory and spatial learning ability were preserved. The semantic/episodic distinction is discussed in the light of the patient's performance on neuropsychological tasks.
Spatial span (Corsi's block-tapping test) and verbal spans for digits (Wechsler Digits Forward Test) and for words were measured in 30 normal subjects and in 51 demented patients, divided into two groups (mildly demented and severely demented) according to selective clinical and neuropsychological criteria. Statistical analysis showed significant differences among the three groups for spatial span. By contrast, controls' and mildly demented patients' performances on both verbal spans were not significantly different. These findings are discussed in the light of theory about working memory.
Screening in schools for heterozygote carriers of haemoglobinopathies traits has been carried out experimentally in southeast France ("Bouches du Rhône", the Marseille region) since 1977. The study compares, in cost-effectiveness terms, the screening strategy now followed with 27 alternative strategies in all the pupils (14-16 years). Although the frequency of heterozygote carriers in this school population as a whole is only 7.9% carriers for beta-thalassaemia gene and 2.5% carriers for HbS, prospective costs per diagnosis still seem reasonable: from 1544 to 4997 French francs (1987) depending on the strategy, and 2099 to 7380 francs if beta-thalassaemia alone is screened. In comparison with the current screening strategy, cost-effectiveness ratios could be improved by using haemoglobin electrophoresis as a preselection test, or a blood count if beta-thalassaemia alone is screened. Further improvement of cost-effectiveness ratios could be obtained by limiting screening to ethnic groups most "at risk"; but such strategies raise ethical and acceptability issues.
Spatial span (Corsi's block-tapping test) and verbal span (Wechsler's Digits Forward test) were measured in 651 normal subjects and in three groups of extrapyramidal patients (Progressive supranuclear palsy, Parkinson, and Huntington's Chorea). Analysis showed Huntington's Chorea patients scored lower on both tests than did controls and other groups.
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In pediatrics, intravenous Gamma Globulin therapy is extremely valuable when combined with other antimicrobial therapy or when employed on its own especially in cases of viral infection. The use of the intravenous route allows the injection of large amounts of drug, intramuscular injections, which are often counter-indicated in children, particularly in children suffering from malignant blood dyscrasias, are thus avoided. Furthermore with the intravenous route, a high blood titre of gamma globulins is achieved immediately and the treatment can be maintained as long as necessary. The tolerance to this drug, in our experience, at least with the preparation used, was found to be excellent in the vast majority of cases. These preparations ought to be widely employed in all of the serious digestive disorders and in paediatrics.
We have studied the changes induced by hemodialysis in the blood oxygen affinity in a group of 8 children with chronic kidney failure. Before dialysis: the affinity was low - P50 (7.40) = 28,3 Torr. Such a decreased affinity could explain in part why the important anemia (2 million RBC on average) observed in these patients was well tolerated. The observed decrease in hemoglobin oxygen affinity was attributed to an increase in 2-3 diphosphoglycerate (2-3 DPG) concentration, resulting from a high level of inorganic phosphates. After dialysis: the plasmatic pH increased markedly (7.52) with a consequent increase in the blood oxygen affinity in vivo (Bohr effect). No substantial change in 2-3 DPG concentration on P50 (7.40) occurred during hemodialysis.
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