Sjörgrens

To analyze the mechanism of interstitial nephritis in patients with Sjörgren's syndrome (SS), we examined the TCR repertoire of infiltration T cells in kidney, labial salivary glands, and PBLs using a PCR. The repertoire of the TCR V beta gene on infiltrating T cells from the kidneys of SS patients was more restricted than those on infiltrating T cells in labial salivary glands and PBL. The TCR V beta 2 gene was expressed predominantly in six of seven (86%) SS patients. Junctional sequences of cDNAs encoding the V beta 2 gene on infiltrating T cells in the kidneys of five SS patients showed that some of the cells expanded clonally, indicating Ag-driven stimulation rather than superantigen-induced proliferation...

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The concentrations of immunoglobulins (Ig) A, G and M were measured in saliva using an electro-immunodiffusion technique at pH 5 with carbamylation of the antisera. Thirty-one controls and 20 patients with generalised scleroderma (10 with and 10 without Sjögren's syndrome) were studied. The three classes of immunoglobulins were in normal saliva. A concentration of IgG greater than 55 mg/l in whole saliva was associated with Sjörgren's syndrome. This is the consequence of the reduction of the rate of saliva production, as well as an increased local synthesis of IgG...

The aim of the present study was to examine the fatty acid patterns of plasma phospholipids, cholesteryl esters, triglycerides and free fatty acids in patients with Sjögren-Larsson syndrome in order to detect whether absorption or metabolism is essential fatty acids may be abnormal. The fatty acid patterns were analyzed by gas liquid chromatography. The proportions of 23 fatty acids were calculated. The parameters used for assessment of the essential fatty acid metabolic status were calculated and compared with those from a group of institutionalized mentally retarded patients and from a group of healthy controls...

A patient was observed with interstitial nephritis which resulted in renal tubular acidosis (distal type), tubular proteinuria and defective urinary concentrating ability in the absence of edema, elevated arterial blood pressure, glomerular proteinuria or abnormal urinary sediment. The presence of interstitial nephritis was established by renal biopsy which showed dense infiltrates in the interstitium, interstitial fibrosis and thickening and splitting of the pericapillary basal membranes. Immunofluorescence was non contributory...

Urinary fluoride concentrations have been measured in electric arc welders using basic electrodes. The fluoride concentration and the total welding fume concentration in air showed a linear relation with postshift urinary fluoride concentration. The measured concentrations were below internationally recommended postshift urinary fluoride concentrations believed to cause fluorosis. Biological monitoring by postshift urinary fluoride measurements is evaluated for the prediction of total welding fume exposure, when a specific basic electrode was used, by means of confidence limits and tests of validity...

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No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

A cross-sectional study of 447 laying hens (age range 0-65 weeks) and a longitudinal study of 164 similar birds showed that Campylobacter jejuni was not present in the faeces of newly hatched chicks, but that colonization arose after 5-9 weeks. A survey of 250 broilers obtained from four breeders showed that all were negative for C. jejuni before and after slaughter at the age of 5 weeks. Once C. jejuni had appeared in a flock, it rapidly spread to virtually all birds, but at the age of 42 weeks only 20-46% of birds remained colonized, possibly as a result of having developed immunity...

To investigate esophageal involvement of scleroderma in primary biliary cirrhosis, esophageal, manometry was performed in 18 patients (16 females, two males) with primary biliary cirrhosis and in a control group of 18 subjects matched by age and sex. All patients were screened for clinical manifestations of scleroderma and for the presence of Sjögren's syndrome. Four patients had scleroderma (all of them with Sjörgren's syndrome), nine had Sjögren's syndrome without scleroderma, and five had neither scleroderma nor Sjögren's syndrome...

Primary sclerosing cholangitis was diagnosed in 6 patients (4 men and 2 women), with ages ranging from 33 to 71 years. Four of them had ulcerative colitis, with an evolution from 8 to 21 years, the other two had Crohn's disease with 2 and 3 years of evolution. 66% of the patients had pain in the right upper quadrant; 50% complained of itching, 33% presented with fever, jaundice, weight loss and hepatomegaly and 16% had splenomegaly. 50% of these patients were diagnosed as having Sjörgren's syndrome. Alkaline phosphatase and gamma GT were elevated in 100% of the cases; SGOT and SGPT were slightly elevated in all cases; bilirubin was elevated only in 50% of the cases...

A 29-year-old black woman with clinical and serological diagnoses of Sjörgren's Syndrome (SS) and mixed connective tissue disease (MCTD) presented a very important muscular compromise with proximal, bilateral weakness and wasting, an abnormal electromyogram, and elevated muscle enzymes. Ultrastructural study of a muscle biopsy showed different degrees of atrophy and presence of subsarcolemmal loss of sarcomeric arrangement forming regions of contractil material with diverse electron densities. Capillaries were proliferative with distortion or occlusion of lumen and thickened basement membranes...

Autoantibodies to nuclear and intercellular antigens have been established as important diagnostic and specific markers for many of the systemic rheumatic diseases. Diseases that have demonstrated specific markers and profiles of autoantibodies include systemic lupus erythematosus, scleroderma, Sjörgren's syndrome, mixed connective tissue disease, drug-induced lupus, and dermatomyositis/polymyositis. The study of molecular biology of the various autoantigens and autoantibodies has led to a better understanding of function and tissue pathogenesis...

Five cases diagnosed as having ataxic cerebral palsy were presented with their brain imaging. Case 1, a 3-year-old-girl had been floppy since 7 months of age and began ataxic walk with spastic legs from 18 months of age. MRI revealed generalized atrophy of cerebellum (especially in anterior superior part) and slight atrophy of pons. Her mother also had ataxia with spastic legs of early onset. She and her mother were thought to have an early-onset inherited non-progressive cerebellar ataxia syndrome. Case 2, a 8-year-old-girl had ataxic walk since 17 months of age...

Findings from two patients with a reticular tapetoretinal dystrophy strongly suggested advanced stages of Sjögren's reticular dystrophy. These observations emphasize that, although initially a benign disease, advanced stages of Sjörgren's dystrophy may eventually manifest diffuse photoreceptor and retinal pigment epithelial disease.

Out of 8 cases of Sjörgren's syndrome observed by the authors between 1964 and 1974, 5 presented wit clinically as well as theoretically remarkable combinations of Sjögren's syndrome with lymphoproliferative disorders i.e. non-classifiable lymphoproliferative disease (case 1), Hodgkin's disease (case 2), reticulum cell sarcoma (case 3), and asymptomatic paraproteinemia of type IgM/K (cases 4 and 5). In case 1, manifestation of a lymphoproliferative disorder was preceded by autoimmune hemolytic anemia. In agreement with communications from several other authors, our observations confirmed the frequent occurrence of autoantibodies in patients with Sjörgren's syndrome...

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The autopsy findings of a woman with myelomatosis diagnosed half a year before her death are described. She had an IgG-lambda myeloma which initially responded to treatment. However, subsequently she developed a condition characterized by massive soft tissue involvement with increased number of plasma cells in peripheral blood. Rounded hard myeloma infiltrates were disseminated through the body, only sparing the adrenal glands and intracranial structures.