[Current concepts of physiology and new orientations in the pathology of connective tissue. 3. Experimental diseases of the connective tissue].
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Biomedical subjects
Publications and source records attributed to L Lenzi.
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Urinary GAGs from patients affected with Osteogenesis Imperfecta (O.I.) type I, type II and type III - according to Sillence et al. (1979) - have been investigated. Galactosamine to glucosamine ratio resulted significantly decreased in O.I. type II and III, whereas smaller differences in the mildest type of the disease were observed. Cellulose polyacetate electrophoresis of urinary GAGs purified from some patients showed the presence of a slowly moving polysaccharide substance, which did not appear in normal subjects. Moreover some pathological fractions, mainly constituted of ChS on the basis of chemical analysis and electrophoretic behaviour, were not digested by testicular hyaluronidase and presented anomalous structures as compared with the corresponding normal ones. These results seem to indicate that in some forms of O.I. the metabolic defect(s) not only affects the synthesis or the catabolism of a particular type of collagen, but also involves the proteoglycan component of the connective tissue.
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The authors aimed to identify reference values for pyridine cross-links (HP and LP) in clinically normal populations. These cross-links are thought to be markers of bone resorption and are released by osteoclasts and eliminated in the urine. The normal values reported in the literature vary considerably. For this purpose the authors analysed groups of male and female patients, divided into age brackets, with no apparent bone metabolism disorders. Hydroxylysylpyridinoline (HP) values revealed no statistically significant differences in relation to either sex or age. Lysylpyridinoline (LP) values in subjects aged under 50 showed virtually comparable reference intervals in male and female subjects, whereas patients over 50 showed a different pattern of behaviour. In conclusion it can be said that, once reliable reference values have been identified using a urinary cross-link assay (in particular for LP), it is possible to evaluate bone resorption in postmenopausal women.