Diabetes insipidus 9 years after cessation of therapy for acute lymphoblastic leukemia.

A case of a 16-year-old who developed diabetes insipidus (DI) 9 years after cessation of therapy for ALL is reported. Because hereditary and traumatic factors are excluded as a cause of DI in this patient, possible explanations may be leukemic CNS relapse, secondary brain tumor, primitive idiopathic DI, and late sequelae of CNS radiochemotherapy.

Studies of delayed hypersensitivity responses in children in an industrialized region of Italy.

The purpose of the present study was to evaluate the effects of chronic exposure to chemical pollutants on cell mediated immune responses in a pediatric population living in Priolo, an industrialized area of Italy, by means of skin test (Multitest CMI). The results suggest that children living in Priolo display significantly lower delayed type hypersensitivity (DTH) response than those seen in an age-matched and socioeconomically similar group of children living in Taormina, a nonindustrialized area. The lowered incidence of DTH scores in Priolo is not due to the number of positive skin test responses to individual antigens, but rather to the size of individual reactions.

High performance liquid chromatography (HPLC): a simple method to quantify Hb C, O-Arab, Agenogi and F.

Differentiation of some abnormal haemoglobins, such as Hb C, O-Arab, Agenogi, E, O-Indonesia, C-Harlem, and Siriraj, is difficult and quantitation of the various fractions is impossible with cellulose acetate electrophoresis. The authors report 13 cases of Hb C, 10 of Hb O-Arab and 5 of Hb Agenogi whose haemoglobin fractions were quantitated by HPLC during a thalassaemia screening programme. Hb F was determined by both Betke's method and HPLC. Analysis of data by linear regression demonstrates that the methods furnish overlapping results. Our findings show that HPLC is a rapid and easily reproduced method which allows quantitative and qualitative discrimination of the various haemoglobin fractions, making it a valid tool in screening programmes for haemoglobinopathies.

A comparative immunogenicity-reactogenicity dose-response study of influenza vaccine.

This study examined the immunogenic and reactogenic responses of influenza vaccine in 29 healthy nonallergic adults at three vaccine dosages: 0.5 mL, 0.1 mL, and 0.05 mL. After immunization a 7-day assessment of adverse reactions was made and serial serum hemagglutination-inhibition (HAI) antibody responses were measured during a 28-day period. The incidence of adverse reactions was significantly decreased in the group receiving 0.1 mL and 0.05 mL compared with the group receiving 0.5 mL of vaccine. After immunization with 0.1 mL or 0.05 mL vaccine increases in serum HAI antibody to A/Leningrad, A/Taiwan, and B/Ann Arbor influenza antigens were seen comparable to those observed after 0.5 mL. However the magnitude of these rises were lower and were directly correlated with the dose of vaccine. Since immunization of egg-sensitive allergic patients with influenza vaccine poses a risk of localized and systemic reactions, a common clinical practice is to prevent such reactions by vaccine dilution. Although the results of the present study suggest that vaccine dilution results in a decrease in adverse reactions, there is also the risk of decrease protective immunity with this procedure and therefore the practice should not be condoned.

Unusual sickle cell disease observed for the first time in Italy: Hb S-Hb D Los Angeles.

In Italy sickle cell disease is mainly represented by sickle cell anemia (beta s/beta s) and sickle cell thalassemia (beta s/beta oth or beta s/beta+ th). Association of Hb S with other beta variants has been observed in other ethnic groups. Since some of these variants have electrophoretic mobility at alkaline pH similar to Hb S, they are frequently misinterpreted as Hb S in the homozygote state. This paper reports the first case of Hb S/Hb D-Los Angeles observed in Italy. The authors underline the need to perform accurate and specific tests in all patients with sickle cell disease and available relatives, in order to exclude combinations of Hb S with other beta hemoglobin variants.

Intrathecal interferon in subacute sclerosing panencephalitis.

Three patients at Stage II of subacute sclerosing panencephalitis (SSPE) were treated with semipurified alpha-interferon (IFN) using different combinations of intrathecal and intravenous routes: 1 x 10(6) IU of alpha-IFN were given every other day up to a total of 15 x 10(6) IU. Transient improvement of neurological symptoms and electroencephalogram were noted in all 3, while cognitive function slightly improved in 2 of them. Clinical benefits gradually disappeared 2 to 6 months after cessation of IFN. Intrathecal antibody production did not change substantially, but CSF Leu 3a/Leu 2a ratio appeared to increase. No significant side effects were observed, except for a mild meningeal inflammatory reaction after each intrathecal administration of IFN.

Leukocyte alkaline phosphatase (LAP). A useful marker of zinc status in beta-thalassemic patients.

Intensive desferrioxamine treatment may negatively affect zinc stores in thalassemic patients. Two groups of young thalassemic patients, with good and poor compliance to desferrioxamine treatment, and a control group were investigated. Leukocyte alkaline phosphatase (LAP), zinc content in neutrophils, plasma zinc levels, and urinary zinc excretion were determined. LAP score, and neutrophil zinc and plasma zinc levels of the good compliance patients were statistically lower than those seen in the controls and in the poor compliance patients. Urinary zinc excretion was statistically higher in good compliance patients than in controls and poor compliance patients only during high-dose intravenous desferrioxamine. On the contrary, poor compliance patients had LAP scores, neutrophil zinc, plasma zinc, and urinary zinc excretion values within the normal range. We conclude that high-dose desferrioxamine produces a reduction of zinc stores in thalassemic patients. LAP scores determinations, which are a reliable and inexpensive marker of body zinc, could become a routine test for monitoring thalassemic patients undergoing hypertransfusion programs.

Increase of F cells during acute hemolysis in glucose-6-phosphate dehydrogenase-deficient males.

Five male Sicilian children with glucose-6-phosphate dehydrogenase deficiency were studied shortly after hemolytic crisis in order to evaluate the immediate effects of massive hemolysis on fetal Hb (HbF) levels and the number of circulating F cells. Hematological values seen 4 months after the children recovered from the crisis were considered representative of the patients' steady state. All patients had an increase in HbF levels (2.26 +/- 0.24%) and F cell number (29 +/- 4.79%) in the acute phase and their HbF values and F cells returned to normal range at control. Globin synthesis was balanced in the peripheral blood and bone marrow and there was a small peak of gamma chains. Globin chain electrophoresis showed that both G gamma and A gamma genes were active in all patients. These results confirm that hemolytic stress produces increased F cell release in peripheral blood. Such release is rapid enough (less than 72 h) to be consistent with the hypothesis of an induction of HbF synthesis in late erythroid precursors.