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Biomedical subjects

A J Barnett

Publications and source records attributed to A J Barnett.

At least 19 recordsLinked to original sources
PubMed1991

Lung function abnormalities and decline of spirometry in scleroderma: an overrated danger?

To document the prevalence and progression of pulmonary involvement in scleroderma (systemic sclerosis including the CREST syndrome), the clinical notes and lung function records of 113 cases were reviewed. Lung function was normal in 39 cases, isolated impairment of DLCO was found in 38 patients, a restrictive defect was present in 27 cases and there was evidence of airflow obstruction in 9 cases. The median duration of symptoms was 10 years. Dyspnoea and an interstitial pattern on chest X-ray were associated with impaired lung function. Death during the period of review was significantly related to initial impairment of the DLCO. Sixty-six patients (53 women and 13 men) underwent repeat spirometry at least 1 year after initial testing. The rates of change in VC and FEV1 were no more rapid than would be expected for normal subjects. There was no significant difference in rates of change between men and women or between dyspnoeic patients and those who were asymptomatic. The extent of skin involvement and the presence of interstitial fibrosis on chest X-ray were unrelated to the rate of loss of lung function. It is concluded that most scleroderma patients in this study had abnormal lung function when first tested, but overall significant worsening of spirometry was not found.

Adolescent↗
PubMed1989

The "neck sign" in scleroderma.

The "neck sign" consists of ridging and tightening of the skin of the neck on extending the head. In this study it was found to be positive in over 90% of patients with scleroderma and negative in patients with primary Raynaud's disease and in control subjects. It is a useful diagnostic test for scleroderma.

Adult↗
PubMed1989

T lymphocyte subset abnormalities and HLA antigens in scleroderma (systemic sclerosis).

Studies of T lymphocyte subsets were carried out in a group of 50 scleroderma patients, of whom 46 were also HLA phenotyped. The total lymphocyte count was depressed in 22 patients, and CD4 (helper cells) numbers were normal. CD8 (suppressor-cytotoxic) cells were reduced in 27 patients, and the CD4/CD8 number ratio increased above normal in three additional patients, resulting in 30 patients being classified as CD8-deficient. In the 46 patients HLA phenotyped, DRw8 was significantly increased in the entire patient group, but when the patients were subdivided into CD8-deficient (n = 29) and CD8-normal (n = 17), the increase in DRw8 was confined to the CD8-deficient patients. B18 was also increased in patients with limited sclerosis, while DR4 and DRw53 were significantly decreased and DR5 significantly increased in patients with more extensive skin sclerosis. These findings suggest that scleroderma is a heterogeneous condition and that this heterogeneity is reflected in different HLA profiles in patients subtyped according to their clinical profile and subpopulations of T cells.

Adult↗
PubMed1988

The diagnosis and classification of scleroderma (systemic sclerosis).

Difficulty in the diagnosis of the disease scleroderma may occur at the early stage prior to the development of obvious skin sclerosis. A presumptive diagnosis may be made if Raynaud's phenomenon is accompanied by a positive 'neck test', 'scleroderma' capillary changes in the nailfolds or antinuclear antibodies. Definitive diagnosis may have to be delayed for several years from the onset of Raynaud's phenomenon until definite characteristic skin changes are seen. Ten cases in which an earlier diagnosis of scleroderma was not substantiated are listed. The earlier incorrect diagnosis would have been avoided by use of the methods described in this paper. Various terms have been used to denote subdivisions of scleroderma. These include acrosclerosis, diffuse scleroderma and CREST. We have used the terms Type 1, Type 2 and Type 3 based on the early extent of the skin sclerosis where Type 1 (limited extent) indicates sclerodactyly only, Type 2 (moderate extent) indicates sclerosis proximal to the metacarpophalangeal joints but excluding the trunk and Type 3 (extensive) indicates diffuse skin sclerosis including the trunk. The clinical value of this simple classification is reviewed and contrasted to other classifications which appear to be poorly defined and of limited use.

Adult↗
PubMed1988

A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease.

A longitudinal study was carried out on 177 patients with scleroderma between 1953 and 1983. Patients were classified into 3 types according to the extent of skin sclerosis within one year of presentation: Type 1 with sclerodactyly only; Type 2, skin stiffness proximal to the metacarpophalangeal joints but sparing the trunk; and Type 3, diffuse skin stiffness including the trunk. Ten year survival was 71% in Type 1, 58% in Type 2 and 21% in Type 3. Quality of life was best in Type 1, intermediate in Type 2 and worst in Type 3. Nailfold capillary abnormalities did not discriminate between the types, but anticentromere antibody correlated strongly with Type 1. The simple subdivision of scleroderma based on the early extent of clinical skin involvement has been found to be useful in predicting longterm outcome in this highly variable disease.

Adult↗
PubMed1986

A highly conserved 72,000 dalton centromeric antigen reactive with autoantibodies from patients with progressive systemic sclerosis.

An autoantibody reactive with a 72,000 dalton centromeric antigen was detected by immunoblotting with the use of a nuclear enriched HeLa cell preparation in 42 of 77 patients with progressive systemic sclerosis (PSS). Reactivity with the 72,000 dalton polypeptide was associated with anti-centromere autoantibodies (ACA) detected by immunofluorescence (IF), and the antigen was highly conserved, being present in both human cells and Leishmania tropica. Thirty-five (83%) of the 42 sera reactive with the 72,000 dalton polypeptide also reacted with a 19,500 dalton polypeptide, and antibodies eluted from both the 72,000 dalton and the 19,500 dalton polypeptides reacted with the centromere when retested by IF on intact HEp2 cells, demonstrating that both polypeptides are antigenic components of the centromere. Only one of the 42 sera had precipitating antibodies to the Scl-70 antigen detected by counterimmunoelectrophoresis, indicating that the 72,000 dalton polypeptide was not related to the previously described Scl-70 antigen. The other 35 of the 77 sera tested were negative for ACA, although all had ANA, with the main patterns of IF being fine speckling of the nucleus (18 sera) and homogeneous or speckled staining of the nucleolus (17 sera). Anti-Scl-70 antibodies were detected in 17 of these 35 patients, 15 (88%) of whom reacted with an 89,000 dalton polypeptide, one with a 140,000 dalton polypeptide, and one with a 74,000 dalton polypeptide. Ten of the 15 sera reacting with the 89,000 dalton polypeptide also reacted with a 74,000 dalton polypeptide, and 2-D gel analysis suggested a relationship between the two molecules. Clinically defined types of scleroderma tended to associate with antibodies to particular molecular antigenic specificities. Thirty-seven (88%) of the 42 patients reactive with the 72,000 dalton polypeptide had sclerodactyly and features of the CREST syndrome, whereas patients reactive with the 89,000 dalton polypeptide and with Scl-70 tended to have more extensive cutaneous and visceral involvement.

Antigen-Antibody Complex↗
PubMed1986

Identification and characterization of mitochondria autoantigens in progressive systemic sclerosis: identity with the 72,000 dalton autoantigen in primary biliary cirrhosis.

Sera from eight out of 62 (14.5%) patients with progressive systemic sclerosis (PSS) reacted by immunoblotting with a 72,000 dalton antigen and one, a patient with concomitant primary biliary cirrhosis (PBC), reacted with the 72,000 dalton and a 47,000 dalton antigen. Reactivity with these antigens was not seen with any of 111 control sera. The antigens with minor variations in m.w. were present in a variety of cultured cells and tissue homogenates from different species. Subcellular fractionation studies localized the antigens to the mitochondria. Of 19 sera from patients with other diseases selected for immunofluorescence staining for anti-mitochondria autoantibody, nine reacted with the 72,000 dalton antigen, seven reacted with both the 72,000 and 47,000 dalton antigens, and three reacted with the 47,000 dalton antigen. These results show that serum reactivity with the 72,000 dalton and 47,000 dalton mitochondria autoantigens is found with some patients with PSS. Because mitochondria autoantibodies that are reactive with the 72,000 dalton and 47,000 dalton polypeptides are also found in patients with PBC, the present finding provides additional support for the association of PSS with PBC. Prior absorption of rat liver homogenate with PBC sera removed PSS serum reactivity with a 63,000 dalton antigen, the equivalent 72,000 dalton antigen in rodents, and vice versa, showing that both PBC and PSS sera recognize the same antigen.

Animals↗
PubMed1986

Scl-95/100: doublet of endothelial marker autoantigens in progressive systemic sclerosis.

Thirteen out of 60 sera (22%) from patients with progressive systemic sclerosis reacted by immunoblotting with a doublet of 95 kD and 100 kD proteins in endothelial cells of human and bovine origin. Reactivity with the doublet was not seen in any of 125 control sera. The endothelial doublet was localized to the nucleus by immunoblot reactivity with nuclear subcellular fractions but not with mitochondrial, microsomal or soluble subcellular fractions. In HEp-2 cells and in HeLa cells, positive sera reacted with the 100 kD antigen, but not with the 95 kD antigen. Identical immunoblot reactivity was obtained with a standard reference serum containing anti-scl-70 activity by immunodiffusion. All positive sera also reacted with cell nuclei by indirect immunofluorescence and 10 gave anti-scl-70 reactivity by immunodiffusion. These observations suggest that the 95 kD/100 kD doublet may be a larger form of the Scl-70 autoantigen.

Adult↗
PubMed1979

Ocular involvement in scleroderma.

Thirty-eight patients with scleroderma (progressive systemic sclerosis) without renal failure were subjected to detailed clinical ophthalmic assessment. Abnormalities were frequent. However, many of these, including lens opacities, vitreous frosting, and arteriosclerotic changes, were considered to be age-related, and there were various incidental changes. Posterior subcapsular lens opacities in one patient were probably corticosteroid-induced. Changes related to scleroderma included eyelid abnormalities (stiffness or tightness in 11, telangiectasia in 8), deficient tear secretion (14 cases), and conjunctival abnormalities (injection 19 cases, vascular sludging 27 cases). Iris light reflux (6 cases) was possibly related to scleroderma. The changes related to scleroderma occurred in the 3 types classified according to the extent of skin involvement.

Adult↗
PubMed1978

Scleroderma (progressive systemic sclerosis): progress and course based on a personal series of 118 cases.

A follow-up study has been made of a personal series of 118 cases of patients with scleroderma (progressive systemic sclerosis) followed for periods up to 25 years. These were classified according to the early distribution of skin changes as Type 1 (skin changes in fingers only), 48 cases; Type 2 (skin changes beyond the fingers but mainly in extremities), 47 cases; and Type 3 (diffuse), 19 cases, and atypical, four cases. Eighty per cent of the total series have survived five years, and 68% have survived 10 years. Survival is related to the type of scleroderma, being much better for Types 1 and 2 (acrosclerotic) than for Type 3 (diffuse). Actual compared with expected survival rate was better for younger than for older patients and for females than for males. Although the disease type gives an indication of survival in general, the caution must be used in the individual cases, as the course of the disease is very variable even in patients of the same type, and is subject to remission and relapse.

Adolescent↗
PubMed1978

Labetalol compared with prindolol plus hydraliazine in the treatment of hypertension: a double-blind cross-over study.

The hypotensive action of labetalol, a new drug with alpha-adrenoceptor and beta-adrenoceptor blocking action, was compared with that of a combination of prindolol and hydrallazine. Fourteen patients with mild to moderately severe hypertension completed a double-blind cross-over study with treatment periods of eight weeks. Both treatments were effective and, in the doses which were used, produced clinically significant lowering of blood pressure (reduction of mean blood pressure by more than 10 mm Hg) both in clinic and in home blood pressures in 12 of the 14 patients. The action of labetalol (600 mg twice a day) was approximately equivalent to that of prindolol (15 mg twice a day) plus hydrallazine (50 mg three times a day). There was a tendency for the second treatment (irrespective of nature) to be more effective than the first. However, when the results for each treatment were combined, the fall in the blood pressure due to labetalol was practically identical with that due to prindolol and hydrallazine. There were no toxic effects from either treatment and side effects were mild. Labetalol is a useful hypotensive drug with effect similar to that of a combination of a beta-adrenoceptor blocking drug and a vasodilator.

Adult↗
PubMed1978

Arterial reconstruction extending below the popliteal bifurcation.

Results are reported for 172 anastomoses below the popliteal artery in patients almost exclusively suffering from severe distal ischaemia with an in-hospital mortality of 7.6 per cent. The patency rate in survivors was 83 per cent on discharge and 39 per cent at 3 years. There were 37 major amputations following 172 operations and 15 following 47 operations on diabetics. As most of the patients would have lost their limbs if arterial repair was not undertaken this constitutes a considerable salvage rate. Diabetes mellitus does not appear to influence the results infavourably, so patients suffering from this disease should be considered for operation.

Aged↗
PubMed1978

Some observations on the immunological status in scleroderma (progressive systemic sclerosis).

A variety of tests were performed to determine the immune status of a group of patients with scleroderma (progressive systemic sclerosis). Sera of 11 of 35 patients showed autoantibodies by an immunofluorescent technique. There was no significant difference between the C3 and C4 components of complement in 12 patients tested and laboratory controls. IgM levels were raised in 24 of 34 sera. Four of ten patients showed some deficiency of phagocytic function in the leucocyte iodination test. B cell and T cell numbers were normal in the eight patients tested. Reactivity to mitogens was usually normal in ten patients tested with a deficient response to one of the three mitogens in two patients. In skin reactivity tests in 23 patients, only one was anergic to all of the four antigens tested. It is concluded that although some immune abnormalities are common in scleroderma there is no consistent abnormality in any of the tests used. This does not exclude the possibility that immune factors may be involved in systemic manifestations of the disease, or that there may be some subtle specific abnormality not revealed by the tests performed.

Adult↗
PubMed1977

The systemic involvement in scleroderma.

A survey was made of the systemic involvement in 38 patients with scleroderma: 33 with the acrosclerotic form (Type 1, 18, Type 2, 15) and five with the diffuse form. The study comprised inquiry about symptoms, physical examination, and the laboratory tests, such as radiological examination of chest and hands, barium swallow and meal X-ray examination, electrocardiography, pulmonary function tests, haematology tests, examination for autoantibodies, and a battery of biochemical tests. Evidence of some systemic involvement (that is, in addition to skin) was almost universal. Similar disturbances occurred both in the acrosclerotic and in diffuse forms. The most common clinical involvement was that of the joints and gastrointestinal tract. The most common confirmatory signs were a positive "neck test" (tethering of the skin of the root of the neck and upper part of the chest on extending the head) and telangiectasia. The most common abnormalities in test results were those found in X-ray films of the hands (about 80%), and in pulmonary function, and barium swallow and meal X-ray studies (each about 70%). The most frequent abnormalities in the biochemical scan were increased levels of immunoglobulin M (IgM), and decreased creatine clearance.

Adult↗