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Biomedical subjects

G Sanna

Publications and source records attributed to G Sanna.

At least 73 records · Page 4Linked to original sources

Deferoxamine-induced growth retardation in patients with thalassemia major.

In the retrospective study reported here, we compared the longitudinal growth in three groups of children with thalassemia major who received a similar transfusion program but different schedules of chelation treatment. In those patients who initiated deferoxamine (DF) administration by daily subcutaneous infusion (50 to 80 mg/kg/day) simultaneously with the beginning of transfusion (at 8 +/- 6 months), mean height at 2 to 6 years of age was significantly reduced in comparison (1) with those patients who initiated DF subcutaneous treatment after 3 years at similar doses and (2) with those who were treated intramuscularly with small doses. In the patients treated at an early stage, those with more marked stunted growth had a clinical and radiologic ricketslike syndrome associated with joint stiffness. Mineral metabolism studies in these patients showed a reduction of hair and leukocyte zinc levels and leukocyte alkaline phosphatase activity. Our findings indicate that DF administration at high doses by continuous infusion before iron overload has been established adversely affects longitudinal growth. By contrast, after 3 years of age, even large doses (in the order of 100/mg/kg/day) did not result in growth retardation. The growth retardation observed may be related to chelation of other trace elements, including zinc, in the presence of low iron burden, to the direct toxic effect of unchelated DF by interference with critical iron-dependent enzymes, or both. These results indicate that in patients with thalassemia major, DF administration should be initiated only after iron accumulation is established, namely, around 3 years of age, after 20 to 30 transfusions, which are usually associated with ferritin levels in the range of 800 to 1000 ng/ml. At this age, deferoxamine doses should be established on the basis of iron balance studies and dose response curves. Doses higher than 50 to 60 mg/kg do not adversely affect growth but produce toxic side effects on acoustic and visual pathways and therefore should not be used. Longitudinal growth monitoring of DF-treated patients is warranted.

Body Height↗

Incidence of multiple sclerosis in the town of Sassari, Sardinia, 1965 to 1985: evidence for increasing occurrence of the disease.

Prevalence studies carried out in Sardinia from 1975 suggest an increasing occurrence of MS. However, it is questionable whether this increase represents a real change in MS incidence or simply reflects longer survival. Data from 79 patients indicated the average annual incidence for the period 1965 through 1985 was 3.4 per 100,000. On December 31, 1985, the prevalence rate was 69 per 100,000. Evaluation of MS temporal trends showed a significant increase in MS incidence during the study period: values ranged around two per 100,000 in the triennial periods 1965-1967, 1968-1970, and 1971-1973, and around five in each triennium from 1977 onward.

Adolescent↗

[Role of Holter electrocardiogram in the evaluation of anti-arrhythmia therapy].

Statistical models may be useful in order to evaluate antiarrhythmic treatment and to distinguish between drug efficacy and spontaneous variability of premature ventricular depolarizations. Analysis of variance and linear regression analysis have been both proposed for documenting antiarrhythmic efficacy. Holter/exercise approach has been designed as noninvasive method of drug testing. So far only one group has reported the long-term outcome of patients managed using this approach. Patients with malignant ventricular arrhythmias in which efficacy was achieved had better chance of surviving during follow-up than did the nonresponder patients. In addition to acute drug testing, chronic trials have been undertaken. One group has reported the long-term results in 124 drug trials and the efficacy was controlled by Holter monitoring. Cumulative probability of survival at 24 months was better for responders than for nonresponders (P less than 0.001). Perspective trials with antiarrhythmic drug are being concluded, such as CAPS and TEST. The results are imminent. Additional aspects to be considered are that critical suppression of premature ventricular depolarizations by Holter does not predict long-term remission of malignant ventricular arrhythmias. Comparison of follow-up results by programmed electrical stimulation and by Holter monitoring are not similar; retrospective non randomized studies show that programmed stimulation was superior to ambulatory monitoring in predicting drug efficacy, or that clinical outcome in patients whose regimen had been found ineffective by programmed stimulation, was as good as the outcome in patients effectively controlled by Holter monitoring.(ABSTRACT TRUNCATED AT 250 WORDS)

Analysis of Variance↗

Renal vein renin in renovascular hypertension: the experience of two Italian centers.

A retrospective analysis of renal vein renin results has been done in 96 patients with renal artery stenosis and hypertension studied in two Italian centers (Sassari and Pisa) with respect to the outcome of either surgery or percutaneous transluminal angioplasty (PTA). In all patients the renal vein renin ratio and the V-A/A ratios for the affected and unaffected kidney were calculated. Each patient underwent surgery (75) of PTA (21): 71 subjects were cured, 17 improved whereas the arterial pressure did not vary after revascularisation procedure in 8 patients. In the Pisa series all 54 patients showed a lateralisation with contralateral renin suppression and 95% of them benefitted from surgery. In the Sassari series 42 patients were submitted to PTA or surgery, not only on the basis of a positive renal vein renin study but taking into account a complete clinical evaluation: 8 of them were cured or improved in spite of negative renal vein renin criteria. In the two series, the better predictive index appeared to be the suppression of the renin secretion from the contralateral kidney while the high/low renin ratio showed a consistent amount of false-positive and false-negative results. Our retrospective study demonstrates that the renal vein test in hypertensive patients with renal artery stenosis is highly predictive of the curability of the disease, particularly when contralateral suppression of renin secretion is present. On the other hand, since patients with negative renin indexes can also take benefit from surgery of PTA, the renin parameters cannot be adopted as the sole criterion in making the decision to operate.

Adult↗

Significance and clinical usefulness of low density lipoprotein electrophoretic mobility for the evaluation of nonenzymatic glycosylation.

We evaluated low density lipoprotein (LDL) electrophoretic mobility (EM) as an index of nonenzymatic glycosylation in 88 insulin-dependent diabetic patients. Among well-controlled diabetics, 36% had increased EM and among poorly controlled patients, 63% had increased EM. This incidence difference was found to be statistically significant by the X2 test. EM can be used as a sufficiently reliable index of LDL nonenzymatic glycosylation.

Adult↗

Small hyperechoic nodules of the renal parenchyma.

Twenty-four hyperechogenic nodules ranging from 0.5 cm to 2.5 cm in diameter were observed within the renal parenchyma in 22 patients. Eighteen patients did not complain of renal symptoms, one had microhematuria, one renal colic, and two pain in the right flank. Because of the sonographic features suggesting fat-containing masses, these lesions were considered to be small angiomyolipomas. Two of our patients underwent surgery, which confirmed this histopathologic interpretation. Twelve patients had a sonographic follow-up, which lasted from 4 to 30 months. We did not observe any change in size or shape of the lesions.

Adult↗

Perinatal hepatitis B virus detection by hepatitis B virus-DNA analysis.

Maternal transmission of hepatitis B virus infection in relation to the hepatitis B e antigen/antibody system and serum hepatitis B virus-DNA were evaluated. Results indicate that hepatitis B virus-DNA analysis can identify hepatitis B serum antigen positive mothers who may transmit infection to their offspring.

Autoradiography↗

[In-hospital and long-term prognosis in acute myocardial infarction. Comparative longitudinal study of 2 patient groups].

The in-hospital mortality, the causes of death, the actuarial survival curves were compared in two subsequent groups of patients admitted to our CCU for acute myocardial infarction: the first (group A) includes 791 pts, admitted from september '67 to december '72, the second (group B) includes 542 pts admitted from january '78 to june '80. The in-hospital mortality was significantly reduced in group B (A: 186/791, 23.5%; B: 72/542, 13,3%, p less than 0.01). This could be due to a reduction of the number of deaths for cardiogenic shock (A: 71/791, 9%; B: 30/542, 5.6%; p less than 0.01) and to reduction in the mortality rate for pulmonary oedema (from 6% to 1.5%, p less than 0.01), although the frequency of pulmonary oedema was the same during the two periods (A: 205 pts, 26%; B/156 pts 29%). We did not observe any significant difference in the long-term prognosis (54 months: A 79.3%, B 71.5%). The actuarial survival curves overlapped after the 1st semester after discharge. The most frequent cause of death during follow-up was a new myocardial infarction. None in the group A and only 3% in the group B were referred to the surgeon for coronary artery bypass grafting. We conclude that, in spite of a significant reduction of the in-hospital mortality, possibly related to the evolution in diagnosis and management of the disease, the long-term survival was not improved in a non-surgically treated population with myocardial infarction.

Adult↗

Antiarrhythmic effectiveness of propafenone compared to lorajmine in ventricular arrhythmias. Controlled clinical trial.

A randomized double-blind cross-over trial was carried out in 20 patients suffering from ventricular ectopic contractions (VECs), in order to evaluate the effectiveness of a new antiarrhythmic drug, Propafenone (P), versus Lorajmine (L). The patients were given 900 mg/day P and 600 mg/day L in three divided doses for 3 days. Every patient was evaluated with four 24-hour ambulatory ECGs, the first at the beginning of the trial, the second after the administration of the first drug, the third after the administration of the second drug and the last after wash-out. With P, the average fall in VECs was 70.6 +/- 10.8% with a clinically significant individual response in 14 of the 20 patients (VECs reduction greater than or equal to 75%, VECs mean decrease: 93.5 +/- 2.1%). With L, VECs decreased by 37.4 +/- 14.2% whilst VECs reduction was larger than 75% only in 50% patients (VECs mean decrease 85.8 +/- 3.1%). Lastly, after therapy interruption, VECs globally increased by +5.4 +/- 14.4%. Propafenone is a considerably active drug against ventricular arrhythmias. It proved to be more effective than Lorajmine under our experimental conditions.

Adolescent↗

Methyl-prednisolone treatment of serum HBsAg negative chronic active hepatitis occurring in transfusion-dependent thalassemia major.

We report the results of a two-year non randomized prednisolone trial carried out in 18 thalassemia major patients with chronic active hepatitis and in 16 controls. We found a beneficial effect on the biochemical remission rate and on the extent of liver inflammation with no significant side effects and no overt reactivation of possible latent HBV infection at three-year follow-up. However, a more prolonged longitudinal study is necessary in order to evaluate whether steroid treatment can impede the evolution to cirrhosis without determining long-term consequences, depending on virus-host interactions such as liver cell carcinoma.

Blood Transfusion↗

Effect of subcutaneous desferrioxamine on iron balance in young thalassemia major patients.

In this study we evaluated the effect of 1-year, subcutaneous desferrioxamine daily infusion on iron balance in 23 thalassemia major children, aged 8-74 months, with transfusional iron accumulation less than 3.8 g at the outset of the trial. Statistical analysis showed a significant correlation between transfusional iron accumulation and percentage of transfusional iron eliminated. Confidence limits analysis of these data indicated that over 3.5 g of transfusional iron load, 95% of the patients may achieve balance. The appropriate time to begin desferrioxamine infusion, therefore, seems to be after about 30 transfusions of packed red blood cells when iron accumulation of approximately 3 g is reached. As we found a statistically significant correlation between transfusional iron eliminated and serum ferritin levels, the evaluation of serum ferritin alone can also predict when balance can be obtained.

Age Factors↗

[Malignant ventricular hyperkinetic arrhythmias. Classificational criteria: review of Lown's classification. Polycentric study].

Although Lown grading system of ventricular premature complexes (VPCs) has important conceptual shortcomings and has shown severe limitations in either cross-sectional or longitudinal studies, this classification is still the most well-known and used method to characterize the arrhythmologic ventricular pattern of the individual patient. We have looked at a modification of VPCs Lown grading scheme to correct the defects and to make the use of the classification more accurate. Our proposal is based on maintenance of 5 classes--similar to those of Lown original classification--to which we have assigned a score from 0 to 60. We have also introduced many sub-classes, each with a definite score, to better characterize the type of arrhythmic event. The sum of the score of the highest class and the score of every subclass furnishes the final score. This score is a direct index of the importance of the ventricular arrhythmias and seems to be useful for statistical evaluation of the anti-arrhythmic therapy and for follow-up studies, as confirmed by preliminary clinical results obtained through the use of this new VPCs classification.

Arrhythmias, Cardiac↗

Iron chelation in transfusion-dependent thalassemia with chronic hepatitis.

In this study maximum urinary iron elimination with continuous desferrioxamine subcutaneous infusion was obtained in thalassemia major patients with chronic persistent or active hepatitis with lower doses (60 mg/kg) than those necessary in patients without hepatitis (80 mg/kg). Since dose-response curves were highly variable the treatment schedule should be tailored to the individual needs of each patient. Both groups may achieve iron balance but chronic hepatitis patients have more frequently a net urinary iron excretion. In patients with chronic hepatitis no correlation was found between serum ferritin levels or serum ferritin/aspartate aminotransferase ratios and transfusional iron overload while serum ferritin/aspartate aminotransferase ratios were seen to be correlated with liver iron stores.

Adolescent↗