Biomedical subjects
Publications and source records attributed to J U Prause.
The four most frequently used criteria for definition of Sjögren's syndrome--the Copenhagen, the Japanese, the Greek and the Californian criteria--are critically evaluated. The article focuses on the similarities and the dissimilarities as well as the advantages and the disadvantages between the various sets of criteria.
Sjögren's syndrome (SS) is defined by the presence of at least two out of three clinical features: keratoconjunctivitis sicca (KCS), xerostomia and another connective tissue disease (CTD). Various criteria have been used to diagnose the oral component of SS (xerostomia) including the presence of at least two out of the following three: lowered unstimulated whole saliva secretion rate, abnormal salivary scintigraphy and sialadenitis in a labial salivary gland biopsy (LSG) ("Copenhagen criteria"), or the solitary criterion of a focus score (FS) value greater than 1 in a LSG biopsy. We compared the application of these two diagnostic criteria on 52 patients suspected of having SS, and evaluated the interexaminer variation of LSG biopsy FS determination. Compared to the Copenhagen criteria, using FS as the only criterion for the oral component of SS would change the diagnosis in 7 (adding 3 and deleting 4 diagnoses of SS) of 52 patients suspected of having the disease. The FS value may be affected by the size of biopsy and interpretation of pathological changes. The overall interexaminer agreement on FS was 90%.
Hybrids of New Zealand black and New Zealand white mice were used in an animal model for Sjögren's syndrome. The animals were treated with bromhexine (Bisolvon), ambroxol (Mucosolvan), or placebo from their 20th week of life for 10-17 weeks. The parotic glands were examined in a masked fashion by light and transmission electron microscopy after treatment. Significant inhibition of pathological changes in the parotic glands was observed by both methods in hybrids receiving 60 mg/kg bromhexine. Other types of treatment had no effect. In addition, the animals receiving the high dosage of bromhexine had a significantly higher survival rate than other hybrids.
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Seventy-five patients in two randomly selected groups (N = 40 plus 35) with primary Sjögren's syndrome were tested for Lewis, A1A2BO, secretor, MNSs, P, Duffy, Kell, Lutheran and rhesus blood group antigens. The results were compared with the frequencies in a control group and in the general population. The Lewis blood group frequency differed (p less than 0.05) from that of the general population, due mainly to an increased Le(a-b-) frequency. Similar immuno-haematological findings have not been reported earlier in patients with rheumatological diseases. If confirmed by other centres, the results may be of importance for our understanding of the immunological mechanisms of chronic inflammatory connective tissue diseases.
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Thirty-six patients with primary Sjögren's syndrome participated in a randomised double-blind, cross-over, 3-week, study to compare the effect of Efamol (1500 mg X 2) with that of placebo. Efamol contains 9% of the prostaglandin-E1 precursor gamma-linolenic acid, which is presumed to occur in reduced levels in Sjögren's syndrome. Efamol treatment improved the Schirmer-I-test (P less than 0.03) while values of break-up time,-van Bijsterveld score, corneasensitivity, tear-lysozyme and nuclear chromatin in conjunctival epithelial cells did not reach the statistical 0.05 level.
Thirty-six patients with primary Sjögren's syndrome were randomized to Mucosolvan (60 mg X 2 daily) or placebo, in a double-blind cross-over study. Each period of treatment was 3 weeks with 1 week wash-out in between. None of the objective ophthalmological tests (Schirmer-1-test, break-up time, van Bijsterveld score, cornea sensitivity, appearance of nuclear chromatin in conjunctival epithelial cells, tear lysozyme) improved during the investigation period.
Bowman's membrane, seen as a homogeneous layer in light microscopy, is demonstrated by transmission electron microscopy as a layer with fibrils in random distribution, for which reason the term used at present is Bowman's layer. Frozen resin cracking demonstrates that the fibrils of the layer run in certain patterns and lie in 3 or 4 strata in the central areas of the cornea. In the periphery, stratification is scarcely demonstrable. The fibrils consist of collagen inseparable by histochemical methods from the collagen in the stroma, but the diameter is only half or two-thirds of the diameter of the stromal fibrils. The authors discuss the possibility that artifacts are responsible for the findings. However, an explanation of the results may be that Bowman's layer is a condensation of the superficial layers of the stroma resulting from the development of the layer. The fibrils are presumed to break in different ways, but apparently in layers in which the breaking strengths are identical.
The effect upon the tear film of a new tear substitute, Dacriosol, was compared to a non-viscous solution, saline, an to a highly viscous solution, Isopto Plain, in 15 normal healthy subjects and in 15 patients with mild to moderate keratoconjunctivitis sicca. No differences between the test solutions could be measured 3 h after last medication regarding: Schirmer's test, break-up time, rose bengal staining, corneal sensitivity and tear content of lysozyme. The test persons could not discriminate subjectively between the effect of the test solutions. The investigation parameters show an equal effect of Dacriosol, Isopto Plain and saline both in normal healthy subjects and in patients with keratoconjunctivitis sicca.
In a randomized clinical study, 32 patients with Sjögrens syndrome (SS) were given placebo/bromhexine (Bisolvon) 48 mg daily for 3 weeks. Various ophthalmological and oral variables as well as different proteins in tear fluid and saliva were registered. Bromhexine stimulated the tear secretion significantly, presumably without changing the protein composition of the tear fluid. In saliva the IgM concentration in contrast to other proteins decreased in patients with high pre-treatment values. Bromhexine may be of valuable help in the treatment of some patients with SS.
A new syndrome consisting of bilateral diffuse malignant uveal melanoma and simultaneous occurrence of another systemic malignant neoplasm was described in 6 patients in 1982. The present study reports a seventh case. Our patient was younger than the patients previously reported. He had large cell carcinoma of the right lung. The bilateral intraocular neoplasm was characterized as a diffuse malignant uveal melanoma of the mixed cell type by light microscopy and by transmission electron microscopy. All parts of the tumor contained S-100 protein. In addition to the two primary neoplasms, the patient had a combination of Ota's and Ito's naevi on the left side.
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Using a micro-electroimmune assay and a 'non-reflex stimulating' method to collect normal tear fluid, the concentration of the serum proteins albumin (S-A), alfa-1-antitrypsin (alpha 1A), alfa-1-antichymotrypsin (alpha 1X) and alfa-2-macroglobulin (alpha 1M) was determined in the tear fluid of 53 normal persons. In addition, the tear fluid was tested for the content of polymorphonuclear leucocyte neutral collagenolytic protease (PML-c-ase). PML-c-ase and alpha 1X could not be detected in any tear samples. The concentrations of S-A, alpha 1A and alpha 1M were constant over time, and showed no variation with sex, age or between the right and left eyes. There was no correlation between the concentration levels of the detected proteins in the tear fluid and correlation between tear fluid levels and corresponding serum concentration levels of the proteins could not be shown.
Using a micro-electroimmune assay, the concentration of polymorphonuclear leucocyte neutral collagenolytic protease, serum-albumin, alpha-1-antitrypsin and alpha-2-macroglobulin were measured in the tear fluid from 18 eyes of 16 patients with severe, melting corneal ulcers. By this method, PML-c-ase was the first time detected in tear fluid. All proteins normalized during healing of the ulcer. The findings are indicative of an important role for PML-proteases in the degradation of the corneal stroma, and of a regulative effect of the serum antiproteases leaking into the tear fluid from inflamed conjunctival vessels.
Four patients with central melting perforating corneal ulcers were treated by n-butylcyano acrylate gluing of the ulcer region. The glue facilitated healing of the eyes, and when the glue scaled off weeks later the corneas were left with stable leucomas. The glue membrane appears to prevent tear-borne polymorphonuclear leucocytes (PML) from reaching the ulcer region and to diminish activation of PML, with the consequent dramatic fall in tear fluid polymorphonuclear leucocyte neutral collagenolytic protease (PML-c-ase). After gluing, the tear fluid content of serum antiproteases returns to normal levels within days. This might indicate a decreased leakage of proteins from conjunctival vessels.
A study of isoamylase in serum from 53 patients with Sjögren's syndrome demonstrated that the levels of both pancreatic type and salivary type amylases remain within reference value limits in most cases. However, abnormal low or high values for pancreatic type amylases were seen more frequently than in the control groups. The intra-individual variation in concentration levels of both isoenzymes among patients with Sjögren's syndrome were significantly greater than in the control groups. These observations are interpreted as signs of subclinical lesions of the pancreas and the salivary glands occurring during the course of the disease. Sjögren's syndrome should be borne in mind and sought in cases of unexplained hypo- or hyperisoamylasemias.