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Biomedical subjects

J U Prause

Publications and source records attributed to J U Prause.

At least 19 recordsLinked to original sources

[Temporal arteritis without histological changes].

A patient with severe giant cell arteritis with involvement of both eyes is presented. The symptoms were partly reversible by treatment with high doses of prednison. Three months previously (having symptoms of polymyalgia rheumatica) she had started treatment with a low dosage of prednison. Biopsies of both temporal arteries showed no signs of giant cell arteritis at that time, and the results of eye examinations were normal. The importance of follow-up on patients with symptoms of polymyalgia rheumatica, even if biopsies of the temporal arteries show no histological changes, is emphasised.

Aged

[Choroid melanoma. A retrospective randomized comparative study of ruthenium irradiation vs enucleation].

A randomized retrospective study concerning survival of Ruthenium treatment and enucleation in melanoma patients has been performed. For 112 patients from Hamburg who underwent Ruthenium therapy individual match partners were selected in Copenhagen where during the same period of time enucleation was the standard procedure. Selection took place concerning patient's age and sex at the time of treatment, initial tumor volume and the time of treatment. The present status of the patients was not known during the selection process. During the observation time of 12 years there was a survival rate after irradiation of 77.9% and after enucleation of 78.6%. Cox' regression disclosed a coefficient of 0.049 with a standard aviation of 0.293 and a P-value of 0.867. All parameters were showing no statistical difference between Ruthenium treated and enucleated patients. The survival rate in males was 69.2%, female patients 87.2%. For male patients there was a 2,4-fold higher risk to develop metastasis compared with female patients. Patient's age at the time of treatment correlated significantly with the prognosis. There was an increase in mortality risk by factor 1.4 per decade.

Aged

Immunologically induced purulent anterior segment inflammation of the guinea pig eye.

A single conjunctival application of ovalbumin to inbred guinea pigs (IMM/S 209) immunized with the same antigen in Freund's complete adjuvant provoked an acute purulent inflammation of the anterior segment of the eyes with a duration of up to 1 week. Intense conjunctival injection and chemosis were followed by a purulent discharge. A corneal haze was observed regularly, and a considerable proportion of the animals developed a pronounced pannus and corneal ulcers. Tear fluid cytology revealed a rapid increase in cell concentration, from the normal level (less than 10(8)/l) to greater than 10(11)/l. Seventy to 95% of the cells were polymorphonuclear leukocytes. Histological examination revealed an acute inflammatory reaction which radiated from the conjunctival fornices to the entire anterior segments of the eyes. The process was characterized by an intense oedema, vasodilation and perivascular aggregations of polymorphonuclear leukocytes, and to a lesser extent eosinophilic granulocytes which characteristically infiltrated and penetrated the epithelial layers. Neovascularization could be observed early after challenge in the stroma of all parts of the outer eye. Ulcerations of the conjunctival and corneal epithelia were observed frequently. After a number of reiterations of the antigenic challenge, a marked infiltration with lymphocytes and basophils/mast cells was observed, and significant scarring of the conjunctival mucosa developed. In several animals, a slight, but significant co-reaction of the contra-lateral, non-challenged eye was observed.

Acute Disease

An unusual ophthalmic tumour in a 5-year-old boy.

A rare tumour in a 5-year-old boy is presented and discussed. In time and location the story had two parts (Fig. 1): 1) a conjunctival granuloma at the nasal limbus of the right eye was surgically removed. 2) a few months later a huge lesion presented in the posterior segment of the same eye. Was it an ocular tumour with extension to the orbit or an orbital process with impression or invasion of the eye? Repeated surgical biopsies have indicated nodular scleritis of the posterior eye segment as the definitive diagnosis.

Child, Preschool

Histopathological changes in exocrine glands of murine transplantation chimeras. I: The development of Sjögren's syndrome-like changes secondary to GVH induced lupus syndrome.

Sjögren's syndrome (SS) is a connective tissue disease characterized by general affection of exocrine glands. The three main components of SS are: dry eyes, dry mouth, and other connective tissue disease. When only two of these, dry eyes and dry mouth, are present, the disease is designated primary SS. In the presence of the third component, most commonly SLE or RA, with one or both of the two first components the disease is designated secondary SS. In murine transplantation chimeras, we have demonstrated the development of both primary and secondary SS depending upon the mouse strains used. We transferred large numbers of viable leucocytes from homozygotic donors to heterozygotic recipients. When DBA/2 mice were used as donors, a full-developed SLE-syndrome, with autoantibodies against native DNA, nuclear antigens, and red blood cells was observed. We found immune deposits in skin ("lupus band") and kidneys, immune complex glomerulonephritis (ICGN), proteinuria, ascites, and hepatosplenomegaly. In later stages, we found a generalized dacryoadenitis. In the kidneys we found interstitial nephritis, and occasionally "half-moon" nephritis. In skin, immune deposits were demonstrated in intercellular spaces. These findings are similar to those found in patients with Sjögren's syndrome secondary to SLE. The murine transplantation chimera is therefore an experimental model for spontaneous autoimmune diseases.

Animals

Histopathological changes in exocrine glands of murine transplantation chimeras. II: Sjögren's syndrome-like exocrinopathy in mice without lupus nephritis. A model of primary Sjögren's syndrome.

Autoimmune reactions are evoked in hybrid mice after induction of a chronic graft-versus-host reaction by transfer of viable leucocytes from one of the parental strains to non-irradiated F1 recipients. We have previously demonstrated an SLE-like syndrome early in the reaction, with an additional Sjögren's syndrome-like glandular affection occurring later. In this study, we used Balb/c mice as donors and Balb/cxCBA/H-T6 F1 hybrids as recipients. We found serum autoantibodies characteristic of SLE after 9 weeks but not after 20 weeks. No clinical signs of disease were seen at any time. After prolonged studies (5 months), we found heavy inflammation in Harderian, salivary, and tear glands. All animals survived the entire length of the experiment without signs of renal failure. The pathological manifestations: lymphocytic infiltration of exocrine glands, and enlargement of lymph nodes, are similar to those seen in patients with Sjögren's syndrome. This murine transplantation chimera may be a useful experimental model for primary Sjögren's syndrome.

Animals

The normal human tear glycoprotein profile detected with lectin probes.

Tear samples were collected from 46 healthy volunteers evenly distributed according to sex and age (mean age 43.5 years). Samples were denatured in a Tris-HCl sample buffer containing 2-mercaptoethanol and SDS, and applied to a gradient SDS-polyacrylamide gel for electrophoresis. The proteinaceous material was transferred to nitrocellulose by a semi-dry blotting technique, and the glycoprotein content subsequently visualized by incubation with four lectins (WGA, PHA, PSA and SBA) and staining with avidin horseradish-peroxidase. Glycoprotein bands were generally found to be significantly less frequent in persons under the age of 30 years. Apart from this the technique gave a uniform picture of the glycoprotein profile, with only modest differences according to age and/or sex. The technique may therefore be suitable for the detection of differences in the glycoprotein composition indicative of disease.

Adolescent

Beneficial effect of sodium sucrose-sulfate on the ocular surface of patients with severe KCS in primary Sjögren's syndrome.

Sucralfate (aluminium sucrose-sulfate), a well known gastric mucosal protectant, has been tested topically on 22 patients (20 females and 2 males) suffering from primary Sjögren's syndrome. Median treatment period was 6 months (range 1-19 months). Statistically significant improvement in the ocular surface condition was found judged from the reduction in Rose-Bengal score (P less than or equal to 0.00005). The beneficial effect appeared within the first 1-4 months of treatment. No adverse side effects were encountered.

Administration, Topical

Biochemical changes in rabbit sclera following destruction of pigment epithelium.

The long-term effect of destruction of the pigment epithelium by sodium iodate on the biochemistry of rabbit sclera was studied in one group with intravenous injection of sodium iodate, and in a second group with injection of sodium iodate into the right eye. Intravenous treatment produced a non-significant increase in the uronic acid concentration. In the second group the untreated fellow eye was microscopically intact, but was shown (as the treated eye) to alter the concentration of uronic acid in different parts of the sclera. All eyes from treated animals exhibited changes in the relative content of the various glycosaminoglycans and in the content of hydroxyproline, hydroxylysine and proline. This work indicates that the pigment epithelium may play a key role in the control mechanism of the scleral connective tissue, and this again has major implications in terms of a possible medical treatment of axial myopia.

Amino Acids

[Sociomedical aspects of primary Sjögren's syndrome].

Forty patients with primary Sjögren's syndrome were interviewed. Poor social conditions during adolescence were not predisposing factors. A significantly higher number of patients grew up in urban than in rural districts, but this difference may be because most of these patients were from Zealand. Patients over the age of 35 years had left school later than the normal population. The matrimonial status did not differ from the normal population, except that the patients had significantly fewer children. The first symptoms of disease were recorded at an age of 20-50 years by 73%. More than half of the patients employed at commencement of the disease had to give up, and received disability pensions or retired early. A number of patients found less demanding jobs. The reasons were extreme fatigue (54%), arthralgia (42%), ocular- (17%) or oral dryness (8%). Social isolation was a problem for many patients and about 50% reported psychological problems. Drug expenses were moderate, whereas dental treatment was often considered to be a major economic problem.

Adolescent

Transvitreal retinochoroidal biopsy.

Biopsies from retina and/or choroid were performed through a transvitreal approach in 14 patients during the 2-year period 1984-1986. A 20-gauge fine needle was used. The transvitreal approach was chosen because exact location of the biopsy could be documented by video recording and/or by photography. The needle was guided either by a stereotactic micromanipulator or by hand. Immediately after biopsy laser burns were placed around the biopsy hole. Light microscopical and, in selected cases, transmission electron microscopical examination of the material obtained showed malignant melanoma, choroidal haemorrhage, leukaemic infiltration of the choroid, intraretinal fibrosis in detached retina, atrophic retina after vasculitis and malignant lymphoma of the uvea. More than 2 years after biopsy, no retinal detachment due to the biopsy or any tumour-seeding has been observed. Two cases of vitreous haemorrhage cleared spontaneously. No visual impairment was related to the biopsies.

Adolescent

Conjunctival cell glycoprotein pattern of healthy persons and of patients with primary Sjögren's syndrome--light microscopical investigation using lectin probes.

Formalin-fixed biopsies of conjunctival tissue from the lower fornix of 10 healthy persons and of 10 patients with primary Sjögren's syndrome (primary SS) were examined by light microscopy. Deparaffinized, rehydrated sections of 5 microns were incubated overnight, each with one of 15 different biotinylated lectins. Bound lectins were visualized using avidin-HRP-substrate complexes. Five lectins did not bind to conjunctival cells, and further six lectins bound in an unspecific manner to conjunctival cells of both healthy persons and of primary SS patients. Jacalin lectin bound selectively to goblet cells of all specimens. Peanut agglutinin (PNA) and wheat germ agglutinin (WGA) bound significantly stronger to basal conjunctival epithelial cells of patients with primary SS. This binding pattern may be of diagnostic value.

Adult

Rose bengal score--a possible key parameter when evaluating disease level and progression in primary Sjögren's syndrome.

The rose bengal score is one of the most commonly used tests for evaluation of ocular surface epithelial damage. The test is used in most Sjögren's syndrome criteria. We examined 24 female and four male patients with primary Sjögren's syndrome (primary SS) in order to evaluate possible correlation between the various tests for keratoconjuncivitis sicca (KCS), and for possible correlations to xerostomia and p-IgG levels. Among the KCS tests a high rose bengal score appeared to be the key parameter, being correlated to low break-up time (P less than 0.01), low tear lysozyme (P less than 0.01), appearance of snake-like chromatin in conjunctival imprints (P less than 0.05), low sialometry (P greater than 0.01) and high p-IgG (P less than 0.01). We followed another group of patients with primary SS (30 females and four males) for a mean period of 53 (range 27-76) months. The patients were divided according to their initial response to systemic treatment with bromhexine. KCS parametres and p-IgG were measured repeatedly during the observation period. Patients responding to and continuously treated with bromhexine (2/3 of patients) improved significantly (P less than 0.05) in rose bengal score, but had increasing levels of p-IgG. Non-responders kept their low tear-production rate and had also increasing p-IgG levels. However, when subdivided according to p-IgG level, the group of patients with relatively low p-IgG improved in rose begal score, whereas the high p-IgG-group increased in rose bengal score. The rose bengal score appears to be a useful key parameter when evaluating disease level and progression.

Adult

Polyclonal B-cell activation is related to exocrine manifestations of primary Sjögren's syndrome.

Twenty-four female and four male patients with primary Sjögren's syndrome were examined in order to evaluate possible correlations between the exocrine disease manifestations and polyclonal B-cell activation parameters. All patients were examined three times over a 4-month period and none of them received any systemic medical treatment. Van Bijsterveld scores were negatively correlated to unstimulated whole salivary flow rate (sialometry) values (P less than 0.01) and break-up times (BUT) (P less than 0.01), while unrelated to Schirmer-1 values. Unstimulated sialometry values were, in addition, positively correlated to Schirmer-1 values (P less than 0.01), while BUT were unrelated to Schirmer-1 and unstimulated sialometry values. P-IgG levels were positively correlated to van Bijsterveld scores (P less than 0.01) and negatively correlated to break-up times (P less than 0.05), while unrelated to unstimulated sialometry and Schirmer-1 values. P-IgG levels were furthermore positively correlated to levels of P-IgM (P less than 0.05), P-IgA (P less than 0.05), serum IgM-RF (P less than 0.001) and serum ANA (P less than 0.01). Levels of P-IgM-RF were positively correlated to van Bijsterveld scores (P less than 0.01), but to none of the other exocrine manifestations. We conclude that the plasma levels of B-cell products seem to correlate positively with the degree of ocular involvement in patients with primary Sjögren's syndrome, indicating a relationship between the immunoinflammatory processes and the mucosal damage.

Adult

Sialochemistry in Sjögren's syndrome: a review.

Sjögren's syndrome (SS) is an autoimmune exocrinopathy. The salivary glands are the site of activated T- and B-lymphocytes, along with gradual parenchymal destruction, diminished flow and altered composition of the secretory product. At present, sialochemistry has achieved no significance for the evaluation of SS patient. However, the number of sialochemical publications is steadily growing. This study review current sialochemical findings in patients with SS and relate the observations to the present concept of diagnosis, pathogenesis and prognosis of SS. An ideal combination of the collection of low-stimulated pure secretion, measurements of absolute flow-rates, and biopsy from the same glands seem to be unobtainable in SS patients. But two procedures may be appropriate: stimulated parotid secretion combined with parotid biopsies, or absorbance of low-stimulated labila saliva combined with labial gland biopsy. Sufficient data on disease-specific alterations in salivary composition in SS are still lacking. However, detection of specific changes in protein synthesis or in glycosylation as well as the detection of inflammatory cell products should be possible with the use of sensitive biochemical assays.

Adult

A follow-up study of the progress of keratoconjunctivitis sicca and response to treatment in primary Sjögren's syndrome.

Thirty-four patients with Sjögren's syndrome were retrospectively examined in order to evaluate longitudinal alterations in objective ocular disease parameters and their possible relation to systemic bromhexine treatment. Twenty-three patients (68%) were initially found to respond to peroral bromhexine treatment and were subsequently treated with this agent in addition to tear substitutes. The other 11 patients (32%) were considered bromhexine non-responders and were treated with topical agents only. The bromhexine non-responders had a significantly (p less than 0.02) more reduced tear gland function, evaluated by the Schirmer-1 test, than the responder group. At the end of the follow-up period the conjunctival surface cells were significantly (p less than 0.02) more damaged in the bromhexine non-responders than in the responders. The bromhexine non-responders improved in both break-up time and van Bijsterveld score in the course of time while the responders improved in the van Bijsterveld score only. No differences as regards extraglandular disease manifestations, serological abnormalities or treatment with other systemic agents were found between the bromhexine responders and non-responders.

Adult