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Collagen diseases and the biosynthesis of collagen.

Increasing knowledge concerning the molecular structure of collagen and the steps involved in its biosynthesis provides the basis for a new look at the collagen diseases. Some, like the Ehlers-Danlos syndrome, are now known to be the expression of primary molecular defects; others, such as scurvy and scleroderma, appear to be secondary to some process that disrupts normal controls over collagen synthesis or deposition.

Amino Acid Sequence

[Pulmonary lesions in systemic connective tissue diseases with immune disorders (collagen diseases)].

The data from the literature and the authors' own studies on changes in the lungs in systemic diseases of the connective tissue with immune disorders: systemic lupus erythematosus, progressive systemic sclerosis, rheumatoid arthritis, and periarteriitis nodosum are presented. Changes in the lungs in the above diseases have some common features: damage of the microcirculatory bed, increased vascular permeability, impregnation with plasma of alveolar septae and vessel walls, cellular reactions, and septo-alveolar sclerosis. Specific features of each of the diseases under study were demonstrated. The time course of morphological changes in the lungs was followed in relation to the severity, duration, and form of the disease.

Antigen-Antibody Complex

[Studies on the secretion of gonadotropins in patients with collagen diseases].

It is well known that majority of patients with collagen diseases are women and that collagen diseases take turn for the worse or the better when the secretion of sex hormones changes greatly at menarche, pregnancy, delivery or menopause. These facts suggest that sex hormones are involved in the pathophysiology of collagen diseases. In the present study, the responses of plasma luteinizing hormone (LH) and follicle stimulating hormone (FSH) levels following the intravenous injection of 100 mug luteinizing hormone-releasing hormone (LR-RH) were investigated in 34 patients with systemic lupus erythematosus (SLE) and in 15 patients with rheumatoid arthritis (RA). The results obtained were as follows: 1) The magnitude of plasma LH response to LH-RH in 29 mature female patients with SLE was significantly greater than that in normal subjects. 2) On the other hand, the magnitude of plasma FSH response to LH-RH in patients with SLE was comparable to that in control subjects. 3) In patients with RA who have normal menstrual cycles, the magnitude of increase in plasma LH and FSH levels after the injection of LH-RH was the almost same as that in normal subjects. Increased responses in plasma LH and FSH levels to LH-RH were observed in 7 patients with RA who were 3 menopausal females and 4 aged males. These findings suggest that the secretion of LH, but not FSH, in response to LH-RH might augment in patients with SLE. On the other hand, in RA patients the function of the pituitary-gonadal axis might maintain within normal limits. For that reason, I guess the following possibilities: 1) In patients with SLE the pathological changes of the disease reached to the ovary and ovarial function was slightly suppressed and then a hypersecretion of LH was observed, 2) the hypothalamus was attacked with the disease and then an unknown mechanism caused the hypersecretion of LH.

Adolescent

[Cerebral angiography in collagen disease and arteritis of different aetiology (author's transl)].

The cerebral angiograms of 11 patients suffering from collagen disease are presented. Panarteriitis nodosa was diagnosed in 4 cases, Lupus erythematodes in 2 cases. With 5 patients immunovasculitis with cerebral affection was found, which was, however, not to be classified in detail. More or less characteristic features are to be expected in the angiogram; they might harden the suspicion of collagen disease, although they are not likely to prove its diagnosis. An interpretation of the radiological findings should--in addition to the morphology--primarily take into account the distribution type of the vessel wall lesions. Clinically as well as by means of angiography it is difficult to differentiate between collagen disease and cerebral arteriitis of different aetiology; this applies particularly to the alterations in cases of embolic circumscribed encephalitis in sepsis lenta. The diagnostic value of angiography in cases of collagen disease with cerebral affection is discussed, the criteria of cerebral arteriitis of different aetiology are dealth with.

Adolescent

[Clinico-radiological and functional aspects of respiratory syndromes caused by collagen diseases].

The clinical and radiological features in 100 patients with collagen diseases (rheumatoid arthritis, lupus, sclerodermia, dermatomyositis, and panarteritis nodosa) were compared with respiratory performance. 56 patients were drawn from the series of Pende et Al. and 44 from a personal series. The results are set out in tables and graphs. It was found that lung lesions due to collagen disease have no special clinical and radiological features. Respiratory performance is that of a restrictive syndrome that gradually progresses from A.R. to E.S., S. and P.M., accompanied by obstruction of the large airways, as shown by hyperinsufflation in sclerodermia and reduced specific conductance in rheumatoid arthritis.

Adult

[Morphological changes in the gingiva in collagen disease, uncomplicated or complicated by odontogenic infection].

Biopsy specimens of the gum of patients with systemic lupus erythematodes, rheumatoid arthritis, and systemic sclerodermia, complicated or not with chronic odontogenous infection, and of 10 those of practically healthy persons with chronic odontogenous infection were studied. In patients with collagenous diseases outside the odontogenous infection foci there were observed considerable lesions of the microcirculatory bed vessels, largely of inflammatory nature.--productive vasculitis of the epithelium and connective tissue of the mucosa proper of the gum. The immunopathological character of the above-mentioned lesions in some cases was confirmed by the immunofluorescent method of investigation. The observed changes in vessels and the connective tissue of the gum in collagenous diseases reflected a locally generalized vasopathy of immune character and systemic disorganization of the connective tissue. Studies of gum biopsy materials in patients with collagenous diseases taken from chronic odontogenous infection foci revealed inflammatory changes in the gum tissues much more serious than in practically healthy people in analogous foci od odontogenous infection. Lesions of the gum in patients with collagenous diseases, not complicated with odontogenous infection, represented a background which aggravated the effect of this infection on the gum.

Adolescent