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Biomedical subjects

A Fischer

Publications and source records attributed to A Fischer.

At least 811 records · Page 45Linked to original sources
PubMed1980

Haemoglobin Lepore Boston-Washington in Sicily: clinical, haematological, and biosynthetic studies.

In the south-east of Sicily 23 children from 14 unrelated families have been diagnosed as suffering from haemoglobin Lepore. Such a high incidence shows that Sicily is an important focus of haemoglobin Lepore. The results of haematological and biosynthetic studies in 18 carriers of Hb Lepore and in five double heterozygotes for Hb Lepore and beta-thalassaemia are presented. In the carriers the haematological and biosynthetic data are compared with carriers of beta-thalassaemia, while the five double heterozygotes are compared with beta o- and beta +-thalassaemia major subjects. In the carriers of Hb Lepore no synthesis of delta beta-chains was observed in peripheral blood cells; in fact we found a peak in the bone marrow. Double heterozygotes with circulating nucleated red cells showed delta beta-chain synthesis in peripheral blood.

Bone Marrow↗
PubMed1980

Hematological and serological aspects of Mediterranean kala-azar in infancy and childhood.

Some hematological aspects of Mediterranean kala-azar were studied with radioisotopical methods. The results showed that pancytopenia is due to increased destruction of circulating elements in the spleen, less in the liver, and not to a defect in production. The serological alterations are characterized by an increase of immunoglobulins, and in particular of IgG, IgA and IgM, which is recorded not only in the acute phase of the disease but also many years after recovery. An hypothesis to explain the persistent alterations of serum proteins is suggested.

Autoantibodies↗
PubMed1979

Alpha thalassaemia in Sicily: haematological and biosynthetic studies.

Eight Sicilian patients with Hb H disease and their families have been studied. The standard haematological tests and the alpha/beta chain synthesis ratios showed significantly different results in the patients with Hb H disease as compared with alpha thalassaemia carriers, except for Hb A2 values. There was no significant difference in the mean RBC, MCV, Hb A2, Hb A1 and Hb F of alpha thalassaemia carriers compared with normal controls. On the contrary significant difference was found between the mean alpha/beta chain synthesis ratio of alpha thalassaemia carriers and that of the normal controls; however, the extensive overlapping of alpha/beta values between these two conditions make this parameter insufficiently discriminant. No correlation was found between MCV, MCH, RBC and alpha/beta chain synthesis ratio in patients with alpha thalassaemia trait, suggesting that the ratio cannot be used to distinguish between carriers of a mild gene ('silent' carrier) and carriers of the more severe alpha thalassaemia gene. A possible genetic model for alpha thalassaemia in Sicily is presented.

Adolescent↗
PubMed1979

Beta-carotene in erythropoietic protoporphyria: 5 years' experience.

36 patients from 19 families with erythropoietic protoporphyria were treated for about 5 years during the summer months with beta-carotene alone or beta-carotene plus canthaxanthin in daily doses of 50-200 mg. The effect of the treatment was evaluated clinically on the basis of information provided by the patients regarding the period of time they could stay in the sun. 18 patients became completely free of symptoms, 16 patients improved to some extent, and 2 patients noted a slight effect. Apart from the carotenaemia, no side-effects were recorded.

Adolescent↗
PubMed1979

Viral hepatitis B and Wiskott-Aldrich syndrome.

A 4-year-old boy affected by Wiskott-Aldrich syndrome had overt viral hepatitis B after repetitive blood transfusions. He was given immune serum containing HBs antibodies, with only transient improvement. The HBsAg titer decreased immediately after each administration of immune serum, but 2 days later it was higher than before. This effect could be explained by the presence of HBs antigen-HBs antibody complexes in the immune serum, not detected by the current testing procedure.

Child, Preschool↗
PubMed1978

Thalassaemia of intermediate severity resulting from the interaction between alpha- and beta-thalassaemia.

A Sicilian family is described in which the alpha-thalassaemia gene is interacting in several members with beta-thalassaemia resulting in a balanced alpha/beta chain production ratio. In one patient, affected by homozygous beta-thalassaemia, the presence of alpha-thalassaemia resulted in a less severe clinical expression of the disease, less marked imbalance in the alpha/non-alpha ratio, and a lower level of HbF. Further studies of haemoglobin synthesis are needed to clarify the complex genetic picture that results from the interaction of different forms of thalassaemia.

Child↗
PubMed1978

beta-Thalassemia in Sicily: hematological and biosynthetic studies.

The degree of imbalance in beta(0)-Th and beta(+)-Th as well as the frequency of the two forms in Sicilian beta-thalassemic subjects have been studied. The hemoglobin synthesis in Rietti-Greppi-Micheli disease (RGMD) and in the beta-thalassemia trait has also been studied. In an unselected thalassemic population, about 30% have been found to be beta(0)-Th. Both groups of beta(0)-Th and beta(+)-Th showed severe imbalance with alpha/non-alpha ratio of 4.22 +/- 1.88 (SD) and 3.46 +/- 1.36, respectively. This difference was not statistically significant. In RGMD the alpha/non-alpha ratio was 2.12 +/- 0.36 while in the beta thalassemia trait it was 1.76 +/- 0.35.

Child↗
PubMed1978

Specific inhibition of in vitro Candida-induced lymphocyte proliferation by polysaccharidic antigens present in the serum of patients with chronic mucocutaneous candidiasis.

A specific inhibitory activity of in vitro proliferative responses of normal human lymphocytes to Candida metabolic antigen was found in the serum of 6 out of 23 children with chronic mucocutaneous candidiasis. In each of the six patients, the presence of an inhibitory activity was associated with Candida-specific cellular defects, characterized by a negative-skin test and a lack of in vitro lymphocyte proliferation. The presence of a circulating inhibitor was detected during relapses of the disease and disappeared under antifungal therapy. This inhibitory effect was not associated with any toxicity on tested lymphocytes. The factor was shown to be nondialysable, thermostable, nonprecipitable with ammonium sulfate and absorbable on anti-Candida antibodies or concanavalin A-coupled agarose columns. Altogether, these results suggest that the inhibitory factor is not an immunoglobulin, but rather a polysaccharidic antigen of Candida albicans. An inhibition of Candida-induced proliferative response of normal human lymphocytes was also obtained by addition of polysacharide antigens or purified mannans from C. albicans to cultures. Candida polysaccharidic antigens appeared, therefore, to be involved in specific depression of cellular functions observed in chronic candidiasis.

Adolescent↗
PubMed1978

Leukokinetic studies in Mediterranean kala azar.

Two patients with acute Kala Azar were studied with DF32P (diisopropylfluorophosphate) and three patients with 51Cr (chromate) in an attempt to delineate the mechanism producing neutropenia in this disease. The granulocyte life span was found to be reduced in all the patients with exception of one who was studied during Glucantim treatment. The surface radioactivity counts showed that the reduced granulocyte life span was due to pooling and probable destruction of granulocytes in the spleen and to a lesser degree in the liver. Bone marrow neutrophil reserve, evaluated by the response to the intravenous hydrocortisone hemisuccinate, was found to be markedly reduced in all patients. An enlarged marginal granulocyte pool indicated also that the neutropenia may be due to altered intravascular granulocyte distribution.

Agranulocytosis↗
PubMed1977

Effect of splanchnicotomy on the renal excretion of uric acid in anaesthetized dogs.

The renal excretion of uric acid was examined during acute i.v. urate (Ua) loading on unilaterally splanchnicotomized ("renal denervation") anaesthetized mongrel dogs. Glomerular filtration rate (GFR) in general was not different in innervated and denervated kidneys, whereas urine flow (V) and urinary excretion of sodium (UNaV) on the splanchnicotomized side were significantly increased at any plasma concentration of Ua. The excretion (UUaV) and tubular transport (TUa) of urate calculated for unit GFR were considerably increased and depressed, respectively, at normal plasma Ua level and during minor urate loading (plasma concentration up to 4.7 mg%). Above this plasma level, i.e. up to 24.6 mg%, no difference in net urate reabsorption between intact and sympathectomized organs was found. It is suggested that both reabsorption and secretion of Ua in denervated kidneys are diminished.

Anesthesia↗