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Biomedical subjects

F Schettini

Publications and source records attributed to F Schettini.

At least 37 records · Page 2Linked to original sources
PubMed1987

Coagulation contact phase factors and inhibitors in beta-thalassemia major children.

Selected hemostatic parameters of 23 children affected by beta-thalassemia major were studied and compared to an age- and sex-matched group. Plasma prekallikrein level was reduced in all patients, splenectomized or not. In splenectomized patients, platelet count and in vitro platelet aggregability were significantly increased and Protein C was slightly increased. The activated partial thromboplastin time was prolonged and the normotest reduced. Finally, a reduction in the plasma levels of fibrinogen and of vitamin K-dependent proteins, including the antithrombotic Protein C, was observed in nonsplenectomized patients. Our data indicate that the hemostatic system in patients with thalassemia major may be altered. The relationship between these laboratory changes and clinical manifestations remains to be established.

Adolescent↗
PubMed1987

Neonatal diabetes mellitus: evaluation of pancreatic beta-cell function in two cases.

Two cases of neonatal diabetes mellitus, a transient form and a permanent form, are described. Comparing their clinical presentations and courses, we exclude the possibility of an early differential diagnosis based on clinical or laboratory data. We hypothesize that only repeated dynamic evaluations of pancreatic beta-cell function could be useful to differentiate the two forms.

Blood Glucose↗
PubMed1987

Lymphadenopathy syndrome and HIV infection in multitransfused beta-thalassemia child.

Lymphoadenopathic Syndrome (LAS), diagnosed also with histologycal studies, is described in a polytransfused 11-year old boy affected by beta-thalassemia major. After three days of the admission he suffered a serious acute diarrhea by Aeromonas hydrophila, an opportunistic enteric bacteria demonstrated, up to date, in immunocompromised patients and never in AIDS patients. It is important to remark that beta-thalassemic patients are at high risk from HIV infections, either for the chronic need of transfusions and for the impairment of the immunological functions.

AIDS-Related Complex↗
PubMed1986

Cytochrome b and FAD content in polymorphonuclear leucocytes in a family with X-linked chronic granulomatous disease.

Chronic granulomatous disease (CGD), an immunodeficiency syndrome characterized by extreme susceptibility to bacterial infections, is due to a defect of the respiratory burst in human phagocytes. NADPH oxidase, the enzyme that catalyzes the reduction of oxygen and the release of oxidative radicals, was studied in polymorphonuclear leucocytes (PMNs) in a family affected by an x-linked inheritance form at high penetrance of the disease. The contents of cytochrome b, suggested as the terminal component of the oxidase electron transport chain, and FAD, the hypothetical proximal component of the chain, were determined in patients and in carriers. Cytochrome b showed the typical behaviour of x-linked CGD: total absence in patients, intermediate values in carriers. FAD content evaluated on plasma membranes was less decreased than cytochrome b. Carriers also showed a decrease of this flavoprotein. Cytochrome b and FAD contents were compared to NBT test and superoxide production: a clear correlation was observed for the cytochrome b, but FAD plasma membrane evaluation could also be an interesting tool for the metabolic characterization of the disease in patients and in carriers.

Adult↗
PubMed1985

Post-natal development of protein C in full-term newborns.

The purpose of this study was to determine the concentration of Protein C in the blood of full-term healthy newborns. The levels of Protein C, evaluated by electroimmunoassay, were low in the first 5 days of life and lower than the critical adult thrombotic level. The antigenic activity increased progressively from the 2nd week of life and the adult values were reached after the 6th month. The reduction of Protein C levels may impair the ability of the newborn to control consumptive disorders, thus exposing the infants to the risk of thrombotic conditions in neonatal age.

Age Factors↗
PubMed1984

Endocrine involvement in children with beta-thalassaemia major. Transverse and longitudinal studies. I. Pituitary-thyroidal axis function and its correlation with serum ferritin levels.

Thyroid function was investigated by a TRH test in 24 clinically prepubertal children, 3-15 years old with beta-thalassaemia major; in 7 of them the test was repeated once and in 2 twice at intervals of at least 12 months. Basal T4, T3, TBG and TSH levels and the TSH levels during a TRH test were determined and correlated with age and serum ferritin levels. Basal serum T4, T3 and TBG levels were lower and serum TSH levels were higher during the test and in the basal state in thalassaemia major children than in control children. These results show a compensated sub-clinical primary hypothyroidism. The transversal study did not show any significant correlation between the hormonal parameters studied and chronological age or serum ferritin levels. In contrast, the longitudinal study showed a significant correlation between pituitary-thyroidal axis function and siderosis (positive correlations between the variations of TSH levels as delta, peak, 30 and 45 min values and the variations of serum ferritin levels). The thyroid impairment seems not to be correlated with serum ferritin levels in the transversal study because of the presence of an individual different sensitivity of the gland to the iron overload. The ferritin dependence of this impairment is shown only by longitudinal studies where individual differences in sensitivity of the gland are absent.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1984

[Longitudinal echocardiographic evaluation in children with thalassemia major].

We report the results of a longitudinal echocardiographic survey on 15 children with thalassemia major, who had received multiple transfusions. The average interval between the two examinations was 2.9 +/- 0.7 years. At the second examination the mean hemoglobin (Hb) level for the group was higher because of the increased number of transfusions. During the same period of time the chelating treatment with desferrioxamine was administered subcutaneously instead of intramuscularly. The following echocardiographic parameters have been measured: left ventricular dimension, diastolic (LVDd); left ventricular dimension, systolic (LVDs); septal thickness, diastolic (STd); free wall, diastolic (FWd); left atrial dimension (LAD); aortic root (AoR); fractional shortening (FA); velocity of circumferential shortening (VCF). At the first examination 6 patients had abnormal values of LVDd, STd and AoR; 10 subjects had increased LAD; the mean Hb levels of the patients with abnormal STd and LAD was significantly lower than in normals (p less than 0.05 and less than 0.01 respectively). At the second examination 8 patients had abnormal LVDd values and 7 had increased LAD. Only the patients with increased LVDd had lower Hb values. Patients with increased LAD had received more transfusions. The following functional parameters were found to be decreased at the second study: FA (29 +/- 3.3 vs 32.6 +/- 7; p less than 0.05); VCF (1.16 +/- 0.25 vs 0.99 +/- 0.14; p less than 0.05). From the results it appears that the change of treatment improved some parameters (STd, AoR, LAD), but was unable to arrest the deterioration of the LV contractile efficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1981

Delayed decrease in serum ferritin in polytransfused children with thalassemia major after continuous subcutaneous infusions of desferrioxamine.

A longitudinal study has been conducted on serum ferritin concentrations in children with thalassemia major treated with desferrioxamine (DFO), both intramuscularly and by continuous subcutaneous infusion, in order to evaluate the time interval after which iron chelation becomes effective. In a first group of 19 children, treated intramuscularly with DFO at a dose of 20 mg/kg for 20 days a month, ferritin levels were followed for 739 days and a progressive increase with a significant linear correlation with time was seen. The daily increase was calculated to be 8.53 +/- 1.95 ng/ml. In the second group of 12 children treated with DFO by continuous subcutaneous infusion (greater than or equal to 20 mg/kg/day for 6 days a week), the increase in ferritin was markedly lower, the mean daily value being 4.77 +/- 3.30 ng/ml. Only after the first 360 days of treatment did the serum ferritin decrease, by 1.95 +/- 3.32 ng/ml/day, in spite of a mean daily iron contribution from blood transfusions of 7.09 +/- 2.69 mg/day. These changes in serum ferritin led us to conclude that one can only expect to obtain a decrease in the body's iron overload after more than a year of treatment with subcutaneous DFO.

Blood Transfusion↗
PubMed1980

Post-natal development of factor IX.

Post-natal development of clotting activity and of antigen level of Factor IX was evaluated in 111 healthy, breastfed, newborn infants, aged 1-30 days. Of these, 80 had received at birth 2 mg of vitamin K1 orally. Factor IX clotting activity was determined by one-stage assay and antigen level by electroimmunoassay. On the 1st day both antigen level and clotting activity were low and the ratio was 1.01. There was a significant postnatal increase of the two activities of Factor IX during the first three days of life; thereafter both remained constant. No statistical difference inFactor IX activity was found with oral administration of vitamin K1 after the birth. During the first month of life both clotting activity and antigen level of Factor IX were low as compared to adult values. There was no correlation with age. The Factor IX protein of newborns did not show molecular heterogeneity by crossed-immunoelectrophoresis.

Antigens↗