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Biomedical subjects

A Sciotto

Publications and source records attributed to A Sciotto.

At least 19 recordsLinked to original sources

Immune responses to administration of a vaccine against Haemophilus influenzae type B in splenectomized and non-splenectomized patients.

OBJECTIVES: we investigated the cause of hypo-responsiveness to vaccines in splenectomized subjects. METHODS: we evaluated the immune responses to a Haemophilus influenzae type b vaccine and the sizes of lymphocyte subpopulations in 25 splenectomized and 45 non-splenectomized thalassaemic patients, in 12 individuals who had been splenectomized after trauma and in 20 controls. RESULTS: the immune response in the controls was significantly higher (P < 0.001) than in splenectomized patients after trauma and in both, the response was higher (P < 0.001) than in thalassaemic patients. In asplenic subjects after trauma, percentages of CD3 and CD4 cells were lower (P < 0.001) than in patients in the other groups; the controls had higher percentages of CD8 cells (P < 0.001) than patients in the other groups. The natural logarithm of the mean percentage of (CD19 showed a quadratic trend from thalassaemic patients through asplenic subjects to controls (P < 0.001). Levels of CD16+ natural killer (NK) cells were higher (P < 0.001) only in asplenic subjects after trauma. CONCLUSIONS: the significant decrease in the immune response of the splenectomized thalassaemic patients vs. non-splenectomized thalassaemic patients may, in part, be due to their basic immunological condition. Thus, the best strategy for protecting these subjects is to vaccinate them before the splenectomy.

Adolescent↗

Peripheral lymphocyte subsets and other immune aspects in Rett syndrome.

A possible role of the immune system in the pathogenesis of some neurologic disorders, including infantile autism, was recently postulated. This observation prompted the authors to investigate some immunologic aspects in a group of patients with Rett syndrome, a disorder still not completely clarified but with some points of commonality with infantile autism. Humoral and cell-mediated immunity were investigated in 20 females with Rett syndrome. Peripheral lymphocyte subsets revealed a reduced percentage of CD8+ suppressor-cytotoxic cells in all of the patients with Rett syndrome, resulting in an increased CD4+/CD8+ ratio. In addition, 15 (75%) of the 20 patients had low levels of natural killer cells. Soluble interleukin-2 receptor was elevated in the youngest patients. Antineuronal and antimyelin ganglioside antibodies were absent, as were antinuclear antibodies, antistriated muscle antibodies, and antismooth muscle antibodies. Immunoglobulin fractions and complement were normal for age in all of the patients.

Adolescent↗

Effect of alpha-interferon on natural killer cell activity and lymphocyte subsets in thalassemia patients with chronic hepatitis C.

The variation of natural killer (NK) cell activity and lymphocyte subsets 20 h after a single test dose of alpha-IFN, was studied in 17 thalassemic patients with chronic hepatitis C. All patients had suspended the alpha-IFN therapy at least 12 months before the study: 10 were considered responders and 7 nonresponders. Also NK cell cytotoxicity after in vitro incubation with alpha-IFN was studied. The administration of a single dose of alpha-IFN increased NK cell cytotoxic activity significantly in the group of responders and in non-responders; moreover the NK cell cytotoxic activity after alpha-IFN in vitro incubation increased both in responders and nonresponders, but to a lesser degree than in healthy controls. Absolute values of CD4+ and CD8+ lymphocytes decreased significantly only in responders. In conclusion, our data suggest that the variation of NK cytotoxic activity and lymphocyte subsets after a test dose of alpha-IFN can be considered a parameter related to IFN biological effects.

Adolescent↗

Efficacy of an analysis of lymphocyte subsets in predicting the clinical response to alpha-interferon therapy in thalassaemia patients with chronic infection by hepatitis C virus: a pilot study.

alpha-interferon (alpha-IFN) has been used to treat chronic non-A non-B hepatitis in thalassaemic patients with response rates from 45% to 83%. Unfortunately, treatment with alpha-IFN is associated with side-effects which have a negative effect on the quality of life of the patient. Therefore it would be useful if we could distinguish in advance those patients who would benefit from such therapy from those who would not. In the present study we found that the modification of lymphocyte subsets 20 h after the administration of the first dose of alpha-IFN revealed that relative numbers of T helper lymphocytes (CD4+) increased in three non-responding patients and decreased in five responding patients, whereas those of T suppressor lymphocytes (CD8+), and natural killer cells (CD57+, CD16+) decreased in non-responding patients and increased in responding patients. Therefore analysis of the lymphocyte subsets CD4, CD8, CD57 and CD16 before and 20 h after the administration of alpha-IFN can be used to predict the clinical response to treatment with alpha-IFN.

Adolescent↗

Low serum levels of interleukin-6 in children with post-infective acute thrombocytopenic purpura.

Interleukin-6 plays an important role in host defense mechanisms and it appears to be a major mediator of the acute-phase response. IL-6 is also an important thrombocytopoietic factor. High serum levels of IL-6 are present in reactive thrombocytosis. The number and function of circulating platelets are the major factors that affect megakaryocytopoiesis by thrombopoietin. High levels of thrombopoietin have been observed in patients with thrombocytopenic purpura. To evaluate a possible thrombopoietin-like function of IL-6, we measured IL-6 levels in the serum of patients affected by post-infective acute thrombocytopenic purpura using a sensitive ELISSA assay. As controls, we studied normal subjects and patients with reactive thrombocytosis. No significant difference was observed between thrombocytopenic patients and normal controls. High IL-6 levels were present in patients with reactive thrombocytosis. In conclusion, we had not observed high levels of IL-6 in acute thrombocytopenic purpura and, very probably, IL-6 is not involved in the regulation of platelet mass for the hemostatic function. The thrombocytopoietic activity of IL-6 is another acute-phase response and it is consistent with the other functions of this cytokine. This suggests an active participation of platelets in host defense mechanisms.

Adolescent↗

Lymphocyte subsets in kala-azar.

We examined lymphocyte subsets in peripheral blood from children with visceral leishmaniasis both in the active stage of the disease and after recovery. At diagnosis, CD2+ and CD4+ cells showed a significant decrease while CD8+ cells were significantly increased when compared with controls; CD4/CD8 ratio was inverted. All of these alterations returned to normal values 3 months after recovery.

Anemia↗

Immunological changes in beta-thalassemia major patients positive for HTLV-III antibodies.

Antibodies to HTLV-III were found in 6 of 206 Sicilian patients with thalassemia major, aged 1 to 30 years. None of the patients showed clinical signs related to immuno-deficiency syndromes. Immunological investigations carried out in all positive patients showed alterations similar to those found in thalassemic patients without antibodies to HTLV-III. These immunological changes could not depend on the HTLV-III infection, but could be related to the thalassemic condition.

Adolescent↗

T-lymphocyte subpopulation changes during hemolysis in glucose-6-phosphate dehydrogenase (G6PD)-deficient children.

Lymphocyte subpopulations were studied by means of sheep red blood cell (SRC)-rosetting technique and OKT11, OKT3, OKT4, and OKT8 monoclonal antibodies in ten Sicilian glucose-6-phosphate dehydrogenase-deficient children during the hemolytic crisis due to fava bean ingestion. The number of SRC-rosetting lymphocytes was significantly reduced, while the number of OKT3-positive cells was normal. An inversion of the OKT4/OKT8 ratio was observed, due to a decrease of OKT4-positive cells and an increase of OKT8-positive cells. All these abnormalities reverted to normal in the four children studied after recovery.

Antibodies, Monoclonal↗

Lymphocyte changes in favism: in vitro evidence of a modifying effect of bilirubin and hemoglobin on T-lymphocyte receptors.

Lymphocyte subpopulations were studied in 13 Sicilian glucose-6-phosphate dehydrogenase (G6PD)-deficient children during and after the hemolytic crisis due to fava bean ingestion. A statistically significant reduction of sheep-red-cell (SRC)-rosetting lymphocytes occurred in all patients during the hemolytic crisis, whereas B lymphocytes were not affected. In order to establish the possible relationship between serum changes occurring during hemolysis and reduction of SRC-rosetting lymphocytes, the effect in vitro of varying concentrations of bilirubin and hemoglobin on the rosette formation capacity of lymphocytes was studied. Both substances produced a statistically significant reduction of SRC-rosetting lymphocytes both in normal and deficient subjects. This effect showed a direct relationship with the concentrations of bilirubin and hemoglobin used and was more pronounced on lymphocytes from G6PD-deficient children. These data suggested that the reduction of SRC-rosetting lymphocytes observed during acute hemolysis of favism can be related to a modifying effect of bilirubin and hemoglobin.

Bilirubin↗

Transient increase of fetal haemoglobin in kala-azar.

Haemoglobin F (HbF) levels were significantly increased in 40 children with kala-azar in comparison with controls (3.36 +/- 2.32 versus 0.90 +/- 1.8; P < 0.01). The HbF was heterogeneously distributed among the red blood cells. The glycine residue of peptides gamma CN3 were within the normal umbilical cord blood range, and the haemoglobin synthesis in vitro was balanced. After recovery from kala-azar the HbF fell within the normal range. These results suggest that increased production of HbF is associated with accelerated erythropoiesis due to temporary marrow stress.

Child↗

Lymphocyte subpopulations in anaphylactoid purpura.

The immunoglobulins, complement components C3 and C4, lymphocyte subpopulations, and K-cell activity were studied in 13 children with anaphylactoid purpura and in 12 children of the same ages who acted as controls. The children with anaphylactoid purpura had significantly lower T-cell counts, greater K-cell activity and IgM values, and lower C3 levels than the controls.

Child↗

K cell activity in acute lymphoblastic leukaemia of childhood.

Lymphocyte subpopulations were studied in 28 children with acute lymphoblastic leukaemia at different stages of the disease. T and B lymphocytes, present in low percentages at diagnosis, increased towards during remission in most patients. All patients off therapy had normal values of T and B lymphocytes. K cell activity, which was increased during remission in several patients, was normal in all patients off therapy. Further prospective studies will establish whether determination of K cell activity is an indicator of the stage of the disease.

Antibody-Dependent Cell Cytotoxicity↗

Glucose-6-phosphate dehydrogenase deficiency in Sicily. Incidence, biochemical characteristics and clinical implications.

This report deals with the incidence, type and clinical implications of G6PD deficiency in Sicily. Of 3347 male subjects examined, 56 were deficient in G6PD. They were distributed throughout the island. The G6PD levels in RBC were almost zero; in leukocytes, platelets and saliva they were found to be 26%, 18% and 16%, respectively, of controls. The Michaelis constant for NADP and G6PD was lower than for controls. Conversely, the utilization of the analogous Ga16P and 2dG6P was higher. The thermostability of the enzyme was lower and the pH optima (6.5 and 9.5) were different from the controls. An identical electrophoretic pattern was found both in normal and deficient subjects. This pattern is superimposable on that described as Mediterranean variant. The analysis among 270 subjects admitted to our Clinic with hemolysis due to G6PD deficiency demonstrated that the most frequent disease is favism, followed by neonatal jaundice, while hemolysis due to drugs is very rare. Ingestion of fresh fava beans was the most frequent cause of favism, but cases occurred after breast feeding and inhalation of pollen.

Adolescent↗

Lymphocyte changes in beta-thalassaemia major.

Lymphocyte subpopulations were studied in 20 hypertransfused patients with beta-thalassaemia major, some of whom had been splenectomised. B-lymphocytes were normal but T-lymphocytes were decreased in all patients. The T-cell count was lower in the splenectomised patients than in the nonsplenectomised ones. In the former, the active rosette-forming lymphocytes were also diminished, but the difference was not significant. In all patients the percentage of null cells was greater and the activity of K-cells increased compared with controls.

Adolescent↗