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Biomedical subjects

G Mastella

Publications and source records attributed to G Mastella.

At least 91 records · Page 5Linked to original sources
PubMed1988

Borderline sweat test: criteria for cystic fibrosis diagnosis.

The CF diagnosis can be difficult in subjects with disease consistent symptoms and borderline values of the sweat test. This study aimed to evaluate possibly resolutory criteria. Seventy-one ill subjects with borderline sweat test values (40-70 mEq/l Cl-) aged 0.1-37.7 years (mean, 8.7; SD, 7.3) were studied and compared with 33 age-matched CF patients (Cl- over 75 mEq/l) and 25 healthy subjects. A complex CF score based on 25 variables was set: CF-specific clinical conditions, detailed analysis of sweat test (salt-free diet test included), pancreatic function, Pseudomonas infection, and others. The score ranged from 0 to a maximum of 65 and clearly distinguished the CF patients from the healthy subjects. Therefore the CF score was assumed as the best reference criterion to define the diagnosis in the borderline group: 27 of them (38%) were assigned to the CF condition and 44 (62%) to the healthy one, with a quite clear separation between the 2 subgroups. With respect to these assignments, the most discriminant variable turned out to be the sweat Cl- persisting above 40 mEq/l after 5 days of salt-free diet (mean error, 18.4%). Neither sweat Cl-/Na+ ratio nor bicarbonate duodenal output showed better discriminant power (mean error, 29% and 25%, respectively). It is concluded that in borderline situations of sweat test results a definite CF diagnosis can be achieved only by compounding many clinical and laboratory data with a preference for the sweat test after a salt-free diet period. The CF score proposal can be an effective help.

Adult↗
PubMed1987

Influence of antacid and formulation on effectiveness of pancreatic enzyme supplementation in cystic fibrosis.

A series of treatment trials, involving food balances based on determination of fat coefficient absorption, nitrogen faecal loss, and daily faecal weight, was performed in 82 patients with cystic fibrosis. Results showed that a conventional powdered pancreatic extract (Pancrex V) required a high dosage to achieve reasonable improvement in fat and nitrogen absorption (200 mg/kg body weight/day on average) and rarely restored digestion to normal. Bicarbonate (5.2 g/m2 body surface/day) slightly enhanced the enzymatic activity of the powdered extract, this being more apparent in those with more severe steatorrhoea. There was no advantage in providing the extract in microgranules protected by cellulose acetatephthalate. A product based on fungal lipase and protease (Krebsilasi) proved to be ineffective in correcting fat and protein absorption. The two recent products prepared in pH sensitive microspheres (Pancrex V microspheres and Pancreas-Prolipase) had similar advantages in digestive activity. Compared with the traditional preparations, they offered a number of practical advantages, including a smaller number of capsules (particularly Pancrex V microspheres) and improved palatability.

Antacids↗
PubMed1986

Homogeneity of cystic fibrosis in Italy.

In 12 unrelated Italian cystic fibrosis (CF) families the frequencies of four DNA polymorphisms closely linked to the CF gene on chromosome 7 were quite similar to those reported for other population samples. Among the 23 affected children from the 12 families, only one recombinant occurred between the CF gene and the met locus, thus confirming the hypothesis of genetic homogeneity of CF previously suggested by the analysis of consanguineous marriages among 624 couples of CF parents. Chi-square test of association indicates a possible linkage disequilibrium between the CF gene and the DNA polymorphism that is most informative in our sample (pmetH TaqI).

Cystic Fibrosis↗
PubMed1985

Absorption of a new semielemental diet in infants with cystic fibrosis.

A new semielemental formula made up of whey protein hydrolysate, medium-chain triglyceride as 50% of fat, and glucose polymer was studied in 21 cystic fibrosis infants to determine whether its intestinal absorption was better than that of a standard milk-based formula. Each experiment lasted 10 days, during which the patient was fed for 5 days on one formula and immediately afterwards for 5 days on the alternative formula, without any pancreatic enzyme replacement. Fat and nitrogen absorption were assessed by 3-day balance studies, stool fat was assayed by a modified Van de Kamer method, and stool nitrogen by an automatic method. Ten infants with very severely impaired digestive function (coefficient of fat absorption on normal diet less than or equal to 75%) showed a highly significant improvement in fat and nitrogen absorption, leading to a significant gain in weight when fed on semielemental diet compared with standard diet. No significant improvement was observed in fat and nitrogen absorption or in weight gain in the 11 infants with less severe malabsorption (coefficient of fat absorption on normal diet greater than 75%). These results were obtained over a short period and have to be confirmed over a prolonged period of study with pancreatic enzyme supplementation. However, they suggest that such a semielemental diet should be effective for short-term treatment in selected CF infants who have difficulty in starting to thrive adequately.

Absorption↗
PubMed1985

Plasma levels of lactoferrin in children suffering from cystic fibrosis.

Plasma levels of lactoferrin (LF) have been found to be increased in a few patients with cystic fibrosis (CF). This study was aimed at investigating plasma LF levels in children with CF (26 cases) and in controls (C) (19 cases). Plasma LF was measured by a radioimmunoassay method. Plasma LF levels were not significantly different in CF and in C, even though 10 CF patients showed LF levels above the mean + 2 SD value of the controls. Neither the duration of the disease nor the age of the controls was correlated with LF or with the exocrine pancreatic capacity. A significant relationship between the presence of an acute lung inflammation and LF levels was found. This study shows that LF is increased in CF only in the presence of an acute inflammatory state. Further studies are necessary to establish the usefulness of an LF assay as an index of the presence of an acute inflammatory process.

Adolescent↗
PubMed1985

Incidence in Italy, genetic heterogeneity, and segregation analysis of cystic fibrosis.

Taking advantage of the availability of an archive of consanguineous marriages that gives accurate estimates of consanguinity in Italy, it has been possible to calculate the increase of first- and second-cousin marriages among 624 couples of cystic fibrosis (CF) parents over the general population. From these estimates, the incidence of CF in Italy has been found to correspond approximately to 1/2,000. In turn, the same data have been used to test the hypothesis of genetic heterogeneity of CF, recently proposed, which is based on the presence of two distinct genetic disorders having similar frequencies. If such a hypothesis were true, the number of first-cousin marriages among CF parents should be significantly higher than that observed in our present study. Finally, the segregation analysis of 624 CF sibships has yielded under multiple selection a segregation ratio of 0.252, confirming the recessive mode of inheritance.

Consanguinity↗
PubMed1984

BT-Paba test in the diagnosis of pancreatic exocrine insufficiency in cystic fibrosis: urinary and serum determinations compared.

Urinary recovery and serum determination of Paba were carried out in 48 control children (C) and 53 paediatric patients with cystic fibrosis (CF) divided into three classes by age. Ninety and 120 min after the ingestion of 15 mg/kg of BT-Paba and of a standard meal, serum Paba was determined. In the same subjects the percentage Paba recovery was measured in the urine collected during an 8 h period after the same administration of BT-Paba. Correlation between urinary and serum Paba values was higher in the older children in respect to the 0-2-year-old infants. A urinary Paba test was less sensitive and specific than a serum Paba test in the evaluation of exocrine pancreatic function. The best discrimination between C and children with CF, using the maximal value of serum Paba at 90 or 120 min (peak), was obtained in the younger infants (0-2 years old). BT-Paba test with serum Paba peak determination is recommended as a substitute for the classical urinary Paba test in the evaluation of exocrine pancreatic function in paediatric patients, especially in the younger infants.

4-Aminobenzoic Acid↗
PubMed1984

Serum and sputum concentrations of azlocillin, cefoperazone and ceftazidime in patients with cystic fibrosis.

Single-dose pharmacokinetics of azlocillin, cefoperazone and ceftazidime were studied in 17 patients with cystic fibrosis (CF). All patients had broncho-pulmonary infections caused by Pseudomonas aeruginosa. Three groups of five, six, and six patients were treated with azlocillin, cefoperazone, or ceftazidime, respectively. The size of the single dose was 133 mg/kg for azlocillin, 66.7 mg/kg for ceftazidime and 66.7 mg/kg for cefoperazone. The clearance values for the three antibiotics calculated from the single-dose data were, on the average, higher than the values previously reported for normal subjects. After the first dose, the patients received a repeated-dose treatment with the same antibiotic. During the first 5 days of therapy, a complement postural drainage of sputum was obtained four times a day for each patient. Cefoperazone could be measured in 47 (39.2%) of the 120 sputum samples assayed while ceftazidime was shown to be present in all 120 sputum samples examined. Azlocillin was not detected in any of the 100 sputum samples assayed.

Adolescent↗
PubMed1983

Alternative antibiotics for the treatment of Pseudomonas infections in cystic fibrosis.

We have investigated the effectiveness of seven new beta-lactam antibiotics, azlocillin, piperacillin, ceftazidime, cefsulodin, cefoperazone, latamoxef (moxalactam), and cefotaxime, against acute pulmonary exacerbations caused by Pseudomonas aeruginosa in cystic fibrosis. Three hundred and fifty-five strains of Ps aeruginosa isolated from 310 sputum cultures (190 cystic fibrosis patients) were tested for susceptibility to the drugs by determination of minimal inhibitory concentrations (MIC). The highest activity was shown by ceftazidime (6% resistant strains) followed by cefsulodin and piperacillin (15 and 16% resistant strains); very low activity was found for cefotaxime and latamoxef (moxalactam). Ceftazidime was the most active drug against 32 pseudomonas isolates that were resistant to both carbenicillin and aminoglycosides (78% susceptible). A randomized, double-blind trial of azlocillin, piperacillin, ceftazidime, cefsulodin or cefoperazone was performed in 111 cystic fibrosis patients with predominant and susceptible pseudomonas in their sputum. Results were evaluated by a clinical, radiological and bacteriological scoring system: the best results were obtained with ceftazidime, followed by cefsulodin and piperacillin. However, pseudomonas was eradicated in only 22 (23%) of the cases with the most active drugs and persisted or reappeared in all the cases 1 to 3 months later. Ceftazidime always eradicated Staph. aureus and Haemophilus influenzae associated with pseudomonas. Similar eradication occurred nearly always with cefsulodin but rarely with the other drugs. No serious drug reaction occurred but a later fever and rash with piperacillin, transient diarrhoea with cefoperazone, vomiting with cefsulodin, and very frequent eosinophilia with ceftazidime should be mentioned. These five drugs offer, in varying degree, alternatives to traditional anti pseudomonas antibiotics in cystic fibrosis pulmonary infections, but they should be used only against well-proven resistant strains. Ceftazidime is best and cefotaxime and latamoxef (moxalactam) least useful.

Adolescent↗