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Biomedical subjects

A Giunta

Publications and source records attributed to A Giunta.

At least 19 recordsLinked to original sources

Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with cystic fibrosis and associated liver disease.

We have previously documented that ursodeoxycholic acid exerts a beneficial effect on liver function and bile acid metabolism in patients with cystic fibrosis. We hypothesized that the mechanism of action may be related in part to the choleretic properties of the administered bile acid. We therefore compared hepatobiliary scintigraphic images obtained before and 1 yr after initiation of ursodeoxycholic acid therapy to document an improvement in bile flow in 13 patients with cystic fibrosis and hepatobiliary involvement. Before therapy, hepatobiliary scintigraphy documented biliary stasis with retention of the isotope in intrahepatic and extrahepatic bile ducts in nine patients; during therapy, duct dilatation decreased substantially in eight patients, with decreased intrahepatic retention and more rapid biliary outflow of the tracer. The time of appearance of isotope in the intestine decreased (from a mean of 36.9 +/- 17.8 min to 18.8 +/- 9.0 min; p less than 0.01) in all patients in whom it had been abnormal, and the half-time of hepatic washout decreased from a mean of 35 +/- 20.7 min to 26 +/- 15.6 min (p less than 0.05). During ursodeoxycholic acid administration enrichment of bile was achieved, with the mean ursodeoxycholic acid percent composition increasing from 5.8% +/- 2.9% to 35.7% +/- 8.5%. Ursodeoxycholic acid became the predominant bile acid in serum. Liver function improved in all 10 of the patients with abnormal values at baseline. We conclude that hepatobiliary scintigraphy is of value in monitoring the therapeutic responses of cystic fibrosis patients with liver disease to ursodeoxycholic acid therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Ursodeoxycholic acid therapy in cystic fibrosis-associated liver disease: a dose-response study.

Previous studies from our groups have demonstrated improvements in biochemical markers of liver function when cystic fibrosis patients with associated liver disease were administered oral ursodeoxycholic acid. The magnitude of the response was somewhat less than that found when comparable doses (10 to 15 mg/kg body wt/day) of ursodeoxycholic acid are given to other liver disease patients; this may be explained by the bile acid malabsorption that is characteristic of the disease. For this reason a dose-response study was carried out in nine cystic fibrosis patients with liver disease to establish whether improved efficacy could be obtained with higher doses. Ursodeoxycholic acid in doses of 5, 10 and 15 mg/kg body wt/day was given orally for consecutive 2-mo periods in a replicated Latin-square design. After this, all patients received 20 mg/kg body wt/day. Liver function, individual serum bile acids and biliary bile acid composition were determined at entry and at the end of each treatment period. Our data demonstrate that the magnitude of the biochemical improvement in serum liver enzymes was significantly greater with higher doses of ursodeoxycholic acid; at 20 mg/kg body wt/day it was similar to that reported for patients with other liver diseases administered lower doses. Biliary ursodeoxycholic acid enrichment increased with increasing doses, attaining 42% +/- 6% of the total biliary bile acids with the highest dose. Fasting serum ursodeoxycholic acid concentrations increased during ursodeoxycholic acid administration but were variable and correlated poorly with the dose of ursodeoxycholic acid administered, whereas no correlation was found between serum ursodeoxycholic acid concentration and the proportion of ursodeoxycholic acid in bile.(ABSTRACT TRUNCATED AT 250 WORDS)

Bile Acids and Salts

Treatment of hydrocele: randomised prospective study of simple aspiration and sclerotherapy with tetracycline.

Following reports in the literature on the efficacy of tetracycline sclerotherapy in the treatment of hydroceles, we carried out a randomised prospective study of 45 patients (50 hydroceles), comparing simple evacuation with tetracycline sclerotherapy. The results revealed no statistically significant difference in the percentage of success between the 2 groups, but complications were more common in the patients treated with tetracycline. The good results reported by other authors in non-randomised studies are, therefore, not confirmed.

Adolescent

Left ventricular diastolic function with epirubicin therapy: an echodoppler study.

Thirty patients undergoing epirubicin therapy for primary lung cancer were studied by echocardiography and Doppler echocardiography. 2 D ejection fraction (EF) and Doppler left ventricular filling parameters (peak E, peak A, E/A ratio) were calculated before and after the completion of therapy. No differences in the mean values of these parameters were observed. However, 6 out of 30 patients (20%) showed left ventricular filling abnormalities; in 2 of them a slight reduction of EF was also noted. These abnormalities seem to be dose related. A longer term prospective study will be required to evaluate whether these findings are irreversible and to establish the clinical implications of our observations.

Adult

Evaluation of cardiac structures and function in systemic sclerosis by Doppler echocardiography.

Ventricular diastolic filling was investigated in a series of 51 consecutive patients with systemic sclerosis by means of Doppler echocardiography. Peak flow velocity in early (peak E) and late (peak A) diastole, E/A ratio, slope of the early diastolic flow velocity and isovolumic relaxation period were calculated. Nine out of the 51 patients showed abnormalities of ventricular filling dynamics in the absence of left ventricular systolic dysfunction at rest and after provocation. The abnormal diastolic filling pattern in these patients was detected in spite of the absence of systemic hypertension, left ventricular hypertrophy or other clinically evident myocardial disease. These diastolic abnormalities might represent an isolated evidence of the underlying myocardial fibrosis not yet clinically apparent.

Adult

Nomogram for penile biothesiometry.

Three hundred and fifty men, aged between 17 and 80 years, with normal sexual anamnesis and without any neurological pathology, underwent finger (index) and penile biothesiometry using a biothesiometer. Tabular and graphical nomograms were obtained according to age and instrumental values. The use of the above-mentioned nomograms is an effective aid in detecting each individual case.

Adolescent

Sudden appearance of echocardiographic changes in a case of acute massive pulmonary embolism.

We report a case of a 68-years-old woman who developed during hospitalization a massive pulmonary embolism. Since the patient had undergone routine cardiac examination, comparison between the echo features immediately preceding and following the event allowed to detail the time interval required for the occurrence of the anatomical and functional changes of the heart chambers.

Acute Disease

Frequency of the delta F508 mutation in a sample of 175 Italian cystic fibrosis patients.

A sample of 175 Italian cystic fibrosis patients has been analysed for the presence of the delta F508 mutation. The frequency of this mutation among 137 patients with pancreatic insufficiency is equal to 57%; in 23 patients with pancreatic sufficiency it is 26%. A high proportion of the unknown mutations is associated with the same rare haplotype found in association with delta F508, suggesting that at least another mutation occurred on a chromosome characterized by the same haplotype.

Chromosome Deletion

Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis.

The hydrophilic bile acid ursodeoxycholic acid (UDCA) has recently been shown to improve indexes of liver function in adult patients with various liver diseases. The clinical and biochemical responses to UDCA administration (10 to 15 mg/kg body weight per day) were therefore investigated in nine patients with cystic fibrosis and evidence of liver disease. All patients were receiving pancreatic enzymes and taurine supplementation. Liver function tests were done and serum bile acid concentrations and biliary bile acid composition were determined before and during UDCA therapy; fat balance studies and fecal bile acid excretion were carried out before and 6 months after UDCA treatment. After 2 months of bile acid therapy, biliary bile acid composition was enriched in UDCA from approximately 5% before treatment to 25%, at the expense of cholic and chenodeoxycholic acids, thus making the pool more hydrophilic. This enrichment is lower than that reported for adults with chronic liver diseases. Serum concentrations of UDCA increased significantly but variably. UDCA became the predominant fecal bile acid excreted (12% to 67%), indicating a variable absorption of the administered bile acid. Liver function improved in all patients after 2 to 6 months of therapy, although the degree of improvement (aspartate aminotransferase, -34%; alanine aminotransferase, -41%; gamma-glutamyltranspeptidase, -41% alkaline phosphatase, -19%) was lower than that observed in adults with chronic liver diseases. Mean coefficient of fat absorption and growth rate were, on average, unaffected by UDCA therapy, although an improvement was noted for three patients with greater severity of steatorrhea. The study indicates that UDCA can be used safely in this patient population but that higher doses of UDCA may be of greater benefit in the treatment of the liver disease associated with cystic fibrosis.

Adolescent

[Cost-benefit relationship between endoscopic resection (TURP) and open surgery (Millen) in prostatic hypertrophy ].

Evaluation of the cost/benefit relationship between TURP (50 cases) and Millin (30 cases). The factors considered are as follows: average post-operative hospitalization, average catheterisation time, average operation time, cost of materials, number of operators employed, number of transfused blood units. The post-operative hospitalization relating to the two kinds of operation varies considerably with a difference of 5.15 days less for the TURP, while the average catheterisation time doesn't seem to be affected. Blood transfusion was necessary in the 43.3% (0.75 unit/patient) of cases for the Millin compared to the 14% (0.26 unit/patient) for the TURP. Evaluation of economical advantages between TURP and Millin shows a relationship of 1:2.3 in favour for of TURP.

Aged

Prevention of cystic fibrosis in Italian families by DNA studies.

We performed a feasibility study of prenatal diagnosis in the Italian population by DNA analysis utilizing the probes: metD, metH, J3.11, KM.19 and XV-2c. With these probes, 118 out of 126 families (93.7%) were fully informative. We also tested part of the families with new additional tightly linked probes, E.9 and W3D1.4. With all the probes feasibility was complete in 44 out of 45 families (97.8%). In particular with a set of only 3 tightly linked probes, KM.19 + XV-2c + W3D1.4, 71% of the families were fully informative. We carried out 8 prenatal diagnoses, 4 of which with the polymerase chain reaction (PCR) for KM.19. These data show that these probes are highly informative and can be used for feasibility studies and prenatal diagnosis of CF in the Italian population.

Cystic Fibrosis

Effects of taurine and ursodeoxycholic acid on liver function tests in patients with cystic fibrosis.

In 9 CF patients with clinical and biochemical evidence of liver disease, taurine (30 mg/kg/day) was administered one month before and during the successive treatment with ursodeoxycholic acid (10-15 mg/kg/day). Standard liver function tests were determined before and after each period of treatment. Taurine administration produced only inconsistent changes of liver function tests from baseline, whereas after the addition of ursodeoxycholic acid a substantial improvement in all abnormal indices was observed. The effects of longer period of treatment are currently investigated, with purpose of establishing their clinical impact and their relationship with changes in bile acid metabolism.

Bile Acids and Salts

Survival in cystic fibrosis: preliminary report from an Italian multicenter study.

An Italian multicenter study has been carried out in order to obtain data on survival and prognostic factors in CF. We report data from four CF Centers: Milan, Neaples, Cagliari, Rome, Anagraphic data, mode of presentation and of diagnosis were collected, using CFDB database, from 536 CF pts. 39 pts had meconium ileus (7.3%); 269 (50%) were diagnosed before 12 months of age. 27 pts were lost to follow-up (5%); and 65 pts died (12%). Statistical analysis was performed by Kaplan-Meier survival curves compared by Logrank test.

Adolescent

Nasal polyposis in cystic fibrosis treated by beclomethasone dipropionate.

Nasal polyposis appears frequently in C. F. patients. Our study intends to evaluate the efficacy of topical beclomethasone dipropionate (BMD) when administered in a position designed to increase exposure of nasal and paranasal sinuses mucosa to the drug. 14 patients were analysed: the size of the polyps was assessed by rhinoscopy and rhinomanometry at the beginning and at the end of the therapy. Our findings show that nasal polyps can be successfully treated medically in a significant number of C. F. patients.

Administration, Intranasal

[Mitomycin C in the topical treatment of superficial neoplasms of the bladder].

From January 1982 to October 1987, 100 patients with multifocal superficial bladder cancer received intravesical MMC at the Institute of Urology, University of Padova. Seventy-three patients had papillary multifocal superficial bladder carcinoma (stages Ta-T1): treatment was divided into therapeutic (18 cases), and prophylactic (55 cases). 27 patients had carcinoma in situ. 39 patients affected by neoplasia relapsed to previous intravesical ADM treatment. All patients received 40 mg. intravesical MMC weekly for 8 consecutive weeks, repeating the cycle in cases of relapse. Complete response rates at 12 months was 53%, 50 to 67% for each group, respectively. Recurrence rate was lower compared to that before treatment; progression rate was also lower compared to a group who received only endoscopic treatment. In patients who relapsed with ADM treatment, the percentage of complete response was 69%. We underscore the efficacy of MMC in lowering relapse and progression in multifocal superficial bladder cancer, which represents the best indication for this kind of treatment.

Administration, Intravesical

Echo-Doppler evaluation of diastolic filling in differently induced left ventricular hypertrophy.

The study was performed in order to assess whether left ventricular hypertrophy (LVH) (exercise or pressure-overload induced but of a similar extent) may differently affect LV diastolic function. The authors compared LV filling echo-Doppler indexes of 16 male trained rowers (mean left ventricular mass index (LVMI) = 162 +/- 18 g/m2) with those of 34 male patients with secondary LVH, subdivided into two groups according to the extent of LVH (group I, n = 20, with a mean LVMI = 159 + 20 g/m2, and group II, n = 14, with a mean LVMI = 221 +/- 23 g/m2). No abnormalities of LV diastolic filling were observed in the athletes; while in patients with secondary LVH of group I an impairment of the echo-Doppler indexes was detected. A more severe involvement of the diastolic properties was observed in patients with LVH of group II.

Adolescent

Doppler echocardiographic assessment of left ventricular diastolic function in patients with primary lung cancer treated with doxorubicin.

Eighteen patients were examined by Doppler echocardiography before and after treatment with doxorubicin (DXR). Early left ventricular filling rate decreased from 66 +/- 15 to 51 +/- 10 cm/sec whereas late filling rate increased from 48 +/- 12 to 61 +/- 9 cm/sec (p less than 0.05) with inversion of the E/A ratio suggesting an impairment of left ventricular diastolic function; conversely no significant changes were found in systolic function indexes. These results suggest that follow-up of patients treated with DXR must include left ventricular diastolic function studies. Doppler echocardiography can be successfully used to detect the presence of diastolic abnormalities.

Adult