Clinical expression of cystic fibrosis in a large cohort of Italian siblings

Background: A clinical heterogeneity was reported in patients with Cystic Fibrosis (CF) with the same CFTR genotype and between siblings with CF. Methods: We investigated all clinical aspects in a cohort of 101 pairs of siblings with CF (including 6 triplets) followed since diagnosis. Results: Severe lung disease had a 22.2% concordance in sib-pairs, occurred early and the FEV1% at 12 years was predictive of the severity of lung disease in the adulthood. Similarly, CF liver disease occurred early (median: 15 years) and showed a concordance of 27.8% in sib-pairs suggesting a scarce contribution of genetic factors; in fact, only 2/15 patients with liver disease in discordant sib-pairs had a deficiency of alpha-1-antitrypsin (a known modifier gene of CF liver phenotype). CF related diabetes was found in 22 pairs (in 6 in both the siblings). It occurred later (median: 32.5 years) and is strongly associated with liver disease. Colonization by P. aeruginosa and nasal polyposis that required surgery had a concordance > 50% in sib-pairs and were poorly correlated to other clinical parameters. The pancreatic status was highly concordant in pairs of siblings (i.e., 95.1%) but a different pancreatic status was observed in patients with the same CFTR mutations. This suggests a close relationship of the pancreatic status with the "whole" CFTR genotype, including mutations in regulatory regions that may modulate the levels of CFTR expression. Finally, a severe course of CF was evident in a number of patients with pancreatic sufficiency. Conclusions: Physicians involved in care of patients with CF and in genetic counseling must be aware of the clinical heterogeneity of CF even in sib-pairs that, at the state of the art, is difficult to explain

Comparative study on nitrite and nitrate ions determinations

Problems have been encountered with the determination of nitrite and nitrate residues in samples of fresh mince and of matured sausages and a comparative test has been carried out between three different methods used by three different laboratories. The results have shown significant differences in the determination of nitrates and appear to suggest the usefulness of doing some more work on the subject

Low-fat mortadella: experimental formulations with some fat substitutes

An investigation has been conducted to test the feasibility of producing mortadella with a low fat content. The research has shown that it is possibile to produce mortadella with a fat content down to about 16% without using substances not normally employed for such a cooked pork product. With the help of fat substitutes, such as milk proteins, carrageenans and soya proteins, a fat content just lower than 14% has been obtained. Quality characteristics, for instance colour and flavour, were negatively affected in some cases but, overall, it appears that satisfactory formulations can be made. The determination of peroxide value, TBARS and cholesterol oxides has shown that there is no oxidation risk

Lipids oxidation and vitamin E supplementation in fresh and processed pork

Lipid oxidation products, and a few cholesterol oxides (COPs) in panicular, are considered atherogenic agents and appear to have mutagenic, carcinogeniac and citotoxic properties. Human subjects can absorb oxysterols from food sources and COPs appear to be able to replace cholesterol molecule in membraues, perturbating permeability, stability and other membrane properties. The research which has been undertaken aimed at improving the nutritional quality of pork through changes of the pigs' diets. The composition of deposit fat has been modified increasing the amount of oleic acid in the feeds and the diet has been supplemented with two levels of vitamin E

Conservazione prolungata della materia prima e processi ossidativi nel salame e nella mortadella

Sono stati studiati gli effetti che una conservazione prolungata delle materie prime può avere sui fenomeni ossidativi dei lipidi nel salame Milano e nella mortadella. A tal fine sono state acquistate le materie prime (spalle, rifilature di prosciutto, pancettoni e lardo) che sono state confezionate in fogli di polietilene e cartoni, congelate a -20'C e conservate per un periodo di 6 mesi. Con tali materie prime sono stati prodotti salami e mofadelle a 0, l, 3 e 6 mesi di conservazione. La stabilità ossidativa dei lipidi è stata valutata sulle materie prime e sui prodotti alle stesse scadenze tramite la determinazine del numero di perossidi, del TBA e degli ossidi del colesterolo. Ne è emerso un quadro sostanzialmente positivo

Osservazioni preliminari in merito ai fenomeni ossidativi nel salame e nella mortadella

Oxidation, monitored by the measure of peroxide value, the TBA test and the determiration of oholesterol oxdes, was found to be sufficiently low in general. Cholesterol oxidation products were found to differ in the two pork products. Mortadella was characterized by the presence in all samples of 5,6-alphaepoxycholesterol, while the 25-hydroxycholesterol has never been found. Only some samples of salame were found to contain cholesterol oxides and the four oxides identified were scattered among the sarnples with no relationship betweeo them

Comparative evaluation of some antioxidants in salame Milano and mortadella production

Six formulations of salame Milano and mortadella were produced, one without antioxidants, two with sodium ascorbate (0~0.5-0~1 and 0.2%), one with catechin, one with phytic acid, and one with sesamol. The products were studied for oxidation levels (peroxide value, TBARS, cholesterol oxides) and colour stability. The evaluation of the latter took place in conditions simulating commercial types of packaging (vacuum and modified atmosphere) and display. A significant difference emerged in colour stability. With the exception of salame Milano packed under modified atmosphere, in all cases salame and mortadella produced without Na ascorbate had significantly lower colour stability. Both in salame and mortadella, peroxide values and TBARS determination gave indications of a possible pro-oxidant effect of ascorbate 0.2% compared with an effective antioxidant activity of the other compounds tested. Cholesterol oxidation in salame appeared to be inhibited by sesamol and ascorbate while catechin was much less effective. In mortadella the prooxidant effect of Na ascorbate was confirmed. All lipid oxidation parameters, though, showed low oxidation values so that no quality nor health deficiencies could be envisaged with any of the formulations adopted. Lower colour stability scores, though, would create commercial problems

Lipid and cholesterol oxidation in frozen stored pork, salame Milano and mortadella

An investigation has been carried out on oxidative processes of fresh and stored frozen pork and of two pork products (salame Milan0 and mortadella). Wholesale pork cuts (shoulder, ham trimmings, belly and backfat) were checked fresh and after frozen storage (1, 3 and 6 months). The streaky bacon was studied fresh and after scalding at 80°C. Salame Milan0 and mortadella were produced with fresh and frozen raw materials and a few commercial samples were also part of the study. No real dtferences have been observed between commercial and experimentally produced salame and mortadella. Peroxide and TBARS values were low in all cases. Among the cholesterol oxides determined (7$-hydroxycholesterol, 5,6a-epoxycholesterol, 7-ketocholesterol and 25-hydroxycholesterol) those dangerous for human health were either absent or their concentration was below toxic levels. The results are discussed on the light of literature reports and Italian pork products technologies