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Biomedical subjects

R M Bernstein

Publications and source records attributed to R M Bernstein.

At least 109 records · Page 6Linked to original sources

Autoantibodies in childhood scleroderma.

The clinical presentation of scleroderma in childhood is even more varied than in adult life. This study of 19 children shows that antinuclear antibodies (ANA) detected on the HEp2 cell substrate are just as common as in the adult disorders. All seven children with diffuse scleroderma or acrosclerosis were ANA positive, as were eight of the 12 with more localised disease. Antinucleolar antibodies were particularly frequent (58% overall), but anticentromere antibody was not observed. Enzyme-linked immunosorbent assays (ELISAs) for antibodies to collagen types I-V, in contrast to adult scleroderma, gave normal results in all childhood cases.

Antibodies, Antinuclear↗

Anti-threonyl-tRNA synthetase, a second myositis-related autoantibody.

An autoantibody known as PL-7 was found in the serum of four patients with myositis and one with a systemic lupus erythematosus-like syndrome. The PL-7 antigen is an 80,000 dalton polypeptide that coprecipitates with transfer RNA. In aminoacylation reactions, PL-7 IgG inhibited the charging of tRNA with threonine but had little or no effect on charging with other amino acids. Experimental antibodies raised against purified threonyl-tRNA synthetase recognized the same 80,000 dalton polypeptide, but tRNA was not coprecipitated. We conclude that PL-7 antibody is directed at threonyl-tRNA synthetase, and that different antigenic sites are recognized by the human and experimental autoantibodies. Our findings emphasize the link between myositis and autoimmunity to tRNA-related structures.

Adult↗

Anti-Jo-1 antibody: a marker for myositis with interstitial lung disease.

An autoantibody known as anti-Jo-1 antibody is found in 25% of patients with myositis. Its prevalence in patients with both myositis and cryptogenic fibrosing alveolitis was 68% (13 out of 19 patients), compared with 7.5% in patients with myositis alone (four of 53) and 3% in patients with cryptogenic fibrosing alveolitis alone (two of 62). Anti-Jo-1 antibody may be useful in indicating patients with myositis and cryptogenic fibrosing alveolitis. Raynaud's phenomenon, the sicca syndrome, and mild arthritis are also often part of the syndrome.

Autoantibodies↗

Luxatio erecta.

Luxatio erecta or inferior dislocation of the shoulder joint is rare and is caused by a hyperabduction injury. The clinical appearance is characteristic, with the arm locked in an elevated position. Radiographically, the shaft of the humerus is directed upwards and the humeral head lies inferior to the glenoid fossa, although not in contact with it. Attention should be given to possible complication, particularly concurrent fractures, as well as injury to the brachial plexus or the axillary artery.

Adolescent↗

HLA antigens, autoantibodies and clinical subsets in scleroderma.

The HLA antigens were determined in 54 Caucasoid patients with scleroderma (ARA criteria). All were assessed for extent of skin and organ involvement, anticentromere (ACA), and scleroderma 70 (Scl 70) antibodies. The antigens DR1, DR3 and DR5 were raised in the patient group, and DR2 was lowered. Of these only the increase in DR5 was significant (chi 2 = 5.2; p = 0.02). The increased frequency of DR3 was attributable to a rise in the A1, B8, DR3 haplotype (chi 2 = 3.9; p = 0.05). Patients with non-diffuse disease showed a significant increase in the DR1 and DR5 antigens (chi 2 = 3.7, p = 0.05; chi 2 = 8.0, p = 0.01). No association was found between the HLA antigens and specific organ involvement. Anticentromere antibodies were present in 15 of the HLA-DR typed patients. Thirteen of these 15 patients were either DR1 or DR5. No relationship was found between the frequencies of HLA antigens and anti-Scl 70 antibodies.

Adult↗

Assessment of platelet function in patients with Raynaud's syndrome.

Platelet function was studied in 11 patients with Raynaud's syndrome and 11 healthy controls. Platelets obtained from patients with Raynaud's syndrome were significantly more responsive to adrenaline, produced more thromboxane A2, and were resistant to prostaglandin inhibitors (prostacyclin and prostaglandin E1) of platelet aggregation. Platelets from control subjects and patients with Raynaud's syndrome were more resistant to prostaglandin inhibitors when reactions were carried out at 27 degrees C rather than at 37 degrees C. Patients with Raynaud's syndrome also had significantly increased plasma concentrations of beta-thromboglobulin, fibrinogen, and circulating platelet aggregates. In an attempt to elicit local platelet responses, the forearms of control subjects and patients with Raynaud's syndrome were cooled in water tanks and platelet function tests performed before and after cooling. No significant difference in the results was observed. The potential role of platelets in the pathogenesis of Raynaud's syndrome is discussed.

Adult↗

A comparative trial of a controlled-release formulation of ketoprofen ('Oruvail') and a conventional capsule formulation of ketoprofen ('Orudis') in patients with osteoarthritis of the hip.

An open study was carried out in 46 patients with osteoarthritis of the hip to compare the efficacy and tolerance of treatment with ketoprofen given either as 100 mg capsules twice daily or as 2 capsules of 100 mg ketoprofen in a controlled-release formulation given once daily. The results of subjective and objective assessments before and during 3-months' treatment in the 48 patients who completed the trial showed both treatments produced improvement in all parameters, except for the time taken for inactivity stiffness to develop, and there was no significant difference between treatments in terms of efficacy. The controlled-release preparation, however, was significantly better tolerated than the ordinary capsule form. Minor haematological and biochemical changes during treatment were noted but these were not of clinical importance. Six patients, 2 receiving the controlled-release and 4 receiving the ordinary formulation of ketoprofen, were withdrawn because of lack of efficacy or unacceptable side-effects.

Adult↗

Cellular protein and RNA antigens in autoimmune disease.

Antibodies directed against soluble cellular antigens are a distinctive feature of systemic autoimmune disease. We have examined 22 autoantibodies in sera from 1111 patients and present the disease associations together with a biochemical analysis of the antigens. The data emphasize the clinical specificity of the antibodies and the restricted number of cellular components that commonly elicit an immune response. In several instances, serological relationships between antibodies mirror biochemical relationships between the corresponding antigens. The antigens are mainly proteins and are often present in complexes with additional protein or nucleic acid molecules. In myositis the antibodies react chiefly with cytoplasmic antigens such as aminoacyl-tRNA synthetases, in contrast to the mainly antinuclear response in SLE. It is argued that both environmental stimuli and genetic factors govern autoantibody specificity, and that molecular characterization of the cellular antigens may yield clues to the aetiology of the disease and of the concomitant, specific autoimmune response.

Antibody Specificity↗

Immunological studies in 'primary' idiopathic pulmonary hypertension.

It has been suggested by several authors that so called 'primary' pulmonary hypertension may have an 'autoimmune' basis because of the clinical association of this condition with Raynaud's phenomenon, polyarthritis and its not infrequent association with other conditions affecting connective tissue, such as progressive systemic sclerosis (PSS) and systemic lupus erythematosus (SLE), often in the absence of pulmonary parenchymal disease. In addition, serum protein abnormalities, positive antinuclear factors and the occasional positive rheumatoid flocculation tests or false/positive Wasserman reaction have also been noted. Data are presented on 9 patients with this condition, in whom not only was there a high frequency of antinuclear factor present (ANA) but in addition, a higher than average incidence of antibodies to smooth muscle (SMA). Screening for antibodies to DNA, extractable nuclear antigens, anticentromere antibodies and antibodies to cardiolipin were uniformly negative. The significance of these negative results are briefly discussed.

Antibodies, Antinuclear↗

Diversity of autoantibodies in primary biliary cirrhosis and chronic active hepatitis.

The presence of autoantibodies in the serum of 110 patients with primary biliary cirrhosis (PBC), 50 with HBsAg negative chronic active hepatitis (HBsAg- CAH) and 30 with HBsAg positive chronic active hepatitis (HBsAg+ CAH) was assessed using two methods: indirect immunofluorescence on cells grown in tissue culture (HEp-2 cell line) or standard mouse tissue sections, and counter immunoelectrophoresis (CIE) with soluble tissue extracts. Anti-nuclear antibodies (ANA) were found in 38% of sera from patients with PBC using HEp-2 cells compared with 10% using mouse tissue. A variety of staining patterns were detected including a pattern of multiple nuclear dots. In contrast, ANA was detected in 70% of sera from patients with HBsAg- CAH and 27% with HBsAg+ CAH. Using CIE four distinct antibody antigen systems were detected: Ro (SS-A), La (SS-B) and two new systems, designated XH and XR, reacting with extracts of human spleen and rabbit thymus, respectively. Correlation of the presence of antibody with clinical conditions confirmed the close association between anti-centromere antibody and sclerodactyly in patients with PBC and indicated an association between 'multiple nuclear dot' staining and the sicca syndrome in PBC. No association was found between the presence of either Ro or La antibody and the sicca syndrome in patients with PBC.

Antibodies, Antinuclear↗

Measurement of antibody to poly (adenosine diphosphate-ribose): its diagnostic value in systemic lupus erythematosus.

Poly (ADP-ribose) and dsDNA binding activity have been measured in sera from 61 patients with systemic lupus erythematosus (SLE) and 188 control sera from 20 normal individuals, 144 patients with clinically similar diseases and 24 patients with drug-induced anti-nuclear antibodies (ANA). Elevated poly (ADP-ribose) binding was not observed with normal sera. Five of 144 samples from diseases entering the differential diagnosis of SLE gave raised poly (ADP-ribose) binding compared with 12 in the 125I-dsDNA binding. Only two of these false positive samples gave elevated binding in the 14C-dsDNA assay. The apparent high specificity of the poly(ADP-ribose) assay was not observed with samples containing drug-induced ANA where 62% had elevated binding values. The frequency with which the poly(ADP-ribose) assay was positive with SLE sera (sensitivity) was lower than either of the dsDNA assays. This low sensitivity and the high rate of false positives in patients with drug-induced ANA limit the value of the poly(ADP-ribose) assay as a diagnostic test for SLE. However the restriction of poly(ADP-ribose) antibody to SLE and patients with drug-induced ANA together with the known role of poly(ADP-ribose) in DNA excision repair suggest that the antibody may be of fundamental significance.

Adult↗

Genetic susceptibility to scleroderma-like syndrome induced by vinyl chloride.

Vinyl chloride (VC) monomer can induce a scleroderma-like syndrome in a proportion of workers exposed to it during production of polyvinyl chloride. As part of a 5-year follow-up study HLA A, B, and DR antigens and anti-centromere and anti-scleroderma-70 antibodies were determined in 44 such workers. 21 of these had severe and 23 mild forms of vinyl-chloride disease. 50 patients with "classical" scleroderma and 148 healthy hospital workers acted as controls. 11 of the 21 patients classified as having severe VC disease were DR3 positive, and 8 of these had both B8 and DR3 antigens. None of the 23 patients with mild disease carried either antigen. The HLA-antigen frequencies in VC disease mirrored those found in scleroderma (raised DR5 frequency and increased linkage disequilibrium between B8 and DR3). There were, however, significant differences in the frequency of autoantibodies in the two conditions.

Autoantibodies↗

The relationship between developments in self-and peer perception during adolescence.

The hypothesis that self-perceptions would be more advanced developmentally than peer perceptions was assessed. The sample consisted of 80 males between 15 and 18 years of age. A structured interview was employed to obtain descriptions of self and a best friend. These data were coded into systems defining the constructs of differentiation, abstraction, and integration. The study's hypothesis was supported. A developmental model was presented to integrate the data on self and social cognition.

Adolescent↗