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Biomedical subjects

V Gabutti

Publications and source records attributed to V Gabutti.

At least 37 records · Page 2Linked to original sources
PubMed1988

Hearing loss and desferrioxamine in homozygous beta-thalassemia.

The authors present the results obtained during an audiometric screening of 153 children aged 5-18 years, affected by beta-thalassemia and treated with regular blood transfusions and iron overload chelation by means of desferrioxamine. Thirty-eight percent of the patients showed a significant sensorineural hearing loss at high frequencies with recruitment. Younger patients had a greater hearing loss, indicating that cochlear damage was not due to the disease itself. Furthermore, hearing loss appeared to be correlated with the mean and peak desferrioxamine doses administered and was higher in subjects with lower iron load. Thus, the ototoxic effect seems to have been higher when a good iron chelation had been obtained. Among our patients, conductive hearing loss was not more frequent than in patients without beta thalassemia.

Adolescent↗
PubMed1985

Reversed-phase high-performance liquid chromatography of human haemoglobin chains.

A reversed-phase high-performance liquid chromatographic method for the separation of human haemoglobin chains has been devised. Using a LiChrospher 100 CH-8/2 column and a ternary eluent (acetonitrile-methanol-0.155 M NaCl, pH 2.7) improved resolution was achieved between (delta beta) Lepore, beta A, beta S, alpha, G gamma and A gamma chains within a 60-min linear gradient. The A gamma T chain can also be separated by increasing the gradient time and decreasing the flow-rate. Silanophilic interactions play an important role in the retention mechanism, and NaCl addition was necessary in order to suppress adsorption on free silanols. Increasing the methanol concentration to 10% caused a slight increase in chain retention, probably owing to solvation of the stationary phase. The recovery was 82% and the reproducibility of retention times was as good as +/- 1.5%. Quantitation of chains is likely to be possible by peak area measurement. Owing to its sensitivity, the proposed method may be useful in the diagnosis of haemoglobinopathies and in the study of haemoglobin variants.

Chromatography, High Pressure Liquid↗
PubMed1985

Growth and sexual maturation in thalassemia major.

Growth and sexual development were evaluated in 250 adolescents with beta-thalassemia major. Before transfusion hemoglobin concentration had not been less than 9.5 gm/dl in the last 5 years; desferrioxamine had been administered for 7 to 10 years, including by the subcutaneous route for 3 years. Thirty-seven percent of patients were found to be 2 SD below the mean for normal height; after age 14 years the percentage was 62% for males and 35% for females. Eighty-three percent of males and 75% of females had delayed skeletal maturation. Complete lack of pubescent changes was present in 38% of females and 67% of males aged 12 to 18 years. Only 19% of females had experienced menarche; secondary amenorrhea intervened in a third of them. A multiple regression analysis of indicators of pubertal development with age, age at first transfusion, age at splenectomy, number of transfusions, serum transaminase and ferritin, and duration and intensity of chelation therapy failed to identify the factors responsible for the variation observed in sexual maturation among patients with thalassemia.

Adolescent↗
PubMed1984

Plasma beta-2-microglobulin and fibronectin levels in beta-thalassaemia.

The plasma beta 2-microglobulin (beta 2-MG) levels in 118 children with thalassaemia were investigated. The mean level was higher than in healthy children. A significant increase of beta 2-MG was associated with hypersplenism (3.14 +/- 0.6 mg/l). The beta 2-MG levels appeared to reflect reticuloendothelial system activity but were not related to iron overload. Fibronectin levels were generally lower than in healthy adults; profound chronic fibronectin depletion was not accompanied by an increased liability to infection.

Adolescent↗
PubMed1982

Early iron overload in beta-thalassaemia major: when to start chelation therapy?

Twenty-eight children with beta-thalassaemia major aged between 11 and 48 months were given intensive transfusions. Serum iron, transferrin saturation, serum ferritin, non-transferrin iron, and subcutaneous desferrioxamine-induced urinary iron excretion were measured. The results showed that even children with a limited number of transfusions had severe iron overload as indicated, in particular, by the raised serum ferritin levels and the high excretion rates after subcutaneous infusion of desferrioxamine. The desferrioxamine test was useful, even in very young children, in assessing response to chelation therapy thus enabling such treatment to be started early to prevent harm from iron overload.

Child, Preschool↗
PubMed1982

Haemoglobin levels and blood requirement in thalassaemia.

The relationship between blood requirement and the mean level of maintained haemoglobin was examined in 392 patients with homozygous beta-thalassaemia. Pre- and post-transfusional haemoglobin levels and the amounts of blood transfused were measured during a 1-year period. No significant differences were noted in the blood requirements of patients (splenectomised or not) irrespective of the haemoglobin level. It may be supposed that if the mean haemoglobin level is high the haematopoietic activity is inhibited, and hence the bone marrow mass and total blood volume are reduced. High haemoglobin levels may thus be obtained with no increase in blood intake.

Adolescent↗
PubMed1980

Behaviour of myeloid precursors in homozygous beta thalassaemia.

Maintaining a high haemoglobin level, through a high transfusion regime, is the best method for treating thalassaemia. Not much is known about the effect of this treatment on medullary or extramedullary haemopoiesis, particularly on the extent of erythropoietic inhibition and on the behaviour of myelopoiesis. In order to analyse some aspects of the problem, we studied the myeloid stem cells (CFU-c) in the bone marrow and in the peripheral blood of children with homozygous thalassaemia, using the agar culture technique. The number of circulating CFU-c observed in 68 patients was higher than in normal subjects. This number was significantly increased after splenectomy. A positive correlation was demonstrated between the number of circulating CFU-c and the time elapsed since the last transfusion. Patients with a high Hb level displayed a marked reduction in the number of CFU-c in their peripheral blood. In 10 patients, before the beginning of transfusions, bone marrow CFU-c were lower than in normal subjects; their number increased after therapy. Most circulating CFU-c were proliferating as shown by the thymidine suicide technique.

Adolescent↗
PubMed1980

Correlation between transfusion requirement, blood volume and haemoglobin level in homozygous beta-thalassaemia.

We examined the relationship between blood volume, mean haemoglobin maintained and transfusion requirement in an extensive series (166 cases) of uniformly treated patients with homozygous beta-thalassaemia. The blood volume is increased in all the subjects and this increase appears inversely proportional to the mean haemoglobin level maintained. When this is above 12 g/dl, the blood volume is nearly normal. The transfusion requirement of patients kept between 10 and 14 g/dl is constant. Maintenance of a high haemoglobin level in thalassaemic patients inhibits erythropoiesis to a considerable degree with a reduction of the haemopoietic tissue and hence of the total blood volume.

Adolescent↗
PubMed1976

Persisting production of out-of-cycle blasts during methotrexate therapy in acute leukaemia.

Kinetics of the blast populations in two cases of acute lymphoblastic leukaemia during treatment with methotrexate have been studied in vivo. Beside a striking cytocidal effect on blasts arrested in the S phase and a synchronization and a slowing of those in the G2 phase, the most important finding was the persistence of a flux of drug-affected cells from the proliferation of the non-proliferating compartment. Some of these non-proliferating cells were able to re-enter subsequently the mitotic cycle. This fact can explain why a cell-cycle-specific drug fails to eradicate completely acute leukaemic cells in man.

Adult↗