Developing a hospital information strategy.
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Biomedical subjects
Publications and source records attributed to E J Acheson.
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Fifty-four adults with primary mesangial proliferative glomerulonephritis and IgM deposition were observed for a minimum of three years or until end-stage renal failure. The actuarial renal survival was 80 per cent at 5 years and 64 per cent at 10 years; multivariate analysis identified microscopic haematuria, extent of mesangial proliferation and global glomerular sclerosis as independent prognostic indicators.
Information technology is of increasing importance to the health service. Two main types of system have grown up, those in clinical departments and central administrative systems. It is important to consider their inter-relationships. A clinical system in the Renal Unit at Manchester Royal Infirmary is described, which is typical of many departmental systems. Further work is reported demonstrating an impressive commonality in the requirements of different clinical disciplines and supporting the view that departmental systems are a valuable source of accurate management data. The experience gained from designing departmental systems is reviewed. It is concluded that the participation of users is essential to the successful design and implementation of systems in the National Health Service and that departmental systems have a crucial role to play in the development of district information systems.
In a series of 60 patients with idiopathic membranous nephropathy (IMN), 8 subjects, aged 16-65 years at presentation, suffered spontaneous relapse of proteinuria after remissions of 25 months to 30 years. Renal biopsy was performed at the time of relapse in 5 cases and revealed histopathology identical to that of the original lesion. Eight courses of immunosuppressive therapy given to 6 patients did not affect either the appearance or duration of remission or relapse. No patient had a familial tendency to renal disease. Immunogenetic markers, HLA A, B and DR, did not distinguish those who relapsed from other patients with IMN. At the end of the study, 3 patients were in a second remission, one had died of myocardial infarction during relapse, 3 remained nephrotic and one had mild renal insufficiency but no proteinuria. As compared with the rest of the series the overall prognosis was not influenced by the relapses.
Over a 5-year period we have performed sequential measurements of a range of complement components in 127 patients. Each had a well-characterised glomerular lesion and there was no evidence of an underlying connective tissue disorder. In 17 patients with varied histopathology, who did not have C3 nephritic factor, there was a persisting complement defect which was present during remission in the patients we were able to study. The finding of such defects is consistent with the thesis that primary complement system abnormalities predispose to the development of glomerular lesions. Interestingly, these abnormalities did not influence the prognosis of our patients. In 12 other cases without C3 nephritic factor, complement levels were below the normal range when the glomerular lesion was active but returned to it in remission; these were secondary changes. We showed by discriminant analysis that some circulating complement component levels, assessed in relation to each other and without reference to a statistically derived 'normal' range, discriminated between histological subgroups and had prognostic significance as well. These patterns could not be distinguished until the data were stored and analysed by computer.
HLA A, B, and DR antigens were determined in a homogeneous group of patients with idiopathic membranous nephropathy. The frequency of HLA-DRW3 was significantly higher in the patients than in a control population. The frequencies of HLA B8 and B18, which are in linkage disequilibrium with DRW3, were also increased.
In 11 patients who presented with apparently idiopathic glomerular disease the antinuclear factor (ANF) was absent initially but was eventually detected during observation extending over 6 years. In 4 patients a diagnosis of systemic lupus erythematosus (SLE) has now been made and the disease treated. Of the remaining 7, 2 had conditions known to be associated with a positive ANF, and in 4, drug therapy induced the ANF. Clinical features, complement studies and measurement of anti-DNA antibody were of value in distinguishing those patients with SLE from the others.
This paper describes the detection of a paraprotein in blood or urine in 12 of 260 patients with 'idiopathic' proteinuria, most of whom presented with the nephrotic syndrome. None had myeloma at presentation and only two have developed it. Initial clinical and biochemical findings did not suggest paraprotein-associated disease, total serum globulins and individual immunoglobulin levels usually being in the normal range. In seven of the 12 cases the paraprotein was detected only after repeated analysis of serum and urine specimens over months or years. Renal histopathology varied from case to case and is described in detail; amyloid deposition did not occur in patients who excreted kappa chain Bence Jones protein and was extensive in only three. One of these eventually developed myeloma. Patients were aged 27--69 years at onset and were observed without specific therapy for up to 56 months. Glomerular filtration rate tended to decline and proteinuria persisted. All patients have now been treated by a chemotherapeutic regimen consisting of 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU), cyclophosphamide, melphalan, and prednisolone, in repeated short courses. In some patients, particularly those who had kappa Bence Jones protein, there was striking improvement. Overall survival is good, eight patients being alive 17--90 months after the onset of symptoms. The importance of repeated search for paraprotein in apparently idiopathic renal disease in adults is emphasized.
The structural changes found on light and electron microscopy study of 25 renal biopsy specimens that showed significant glomerular IgA deposition on immunofluorescence were correlated with relevant clinical data. The morphology of a wide range of glomerulopathies seeen included mesangial proliferative (60%), membranous (12%), rapidly progressive proliferative (8%), mesangio-capillary (8%), and no light microscope change (8%). Four of the 15 cases (60%) of mesangial proliferative glomerulonephritis were associated with focal segmental sclerosis and 10 with focal segmental and focal global sclerosis. In addition, 7 of the 15 cases showed capsular crescents. The clinicopathological correlations indicated that the prognosis in this group is unfavourable when focal global sclerosis and capsular crescents are present, particularly when both occur in the same biopsy specimen.
Thirty-seven percutaneous renal biopsies showing no significant abnormalities on light microscopy were studied electron optically and by immunofluorescence when available. Assessment of the pathological material was followed by analysis of the patients' clinical notes, and a clinicopathological correlation was carried out. Twenty-three patients fulfilled the clinical criteria of minimal change disease; 10 did not behave clinically as minimal change and showed immune complex deposition; two had benign recurrent haematuria; and two had myelomatosis. Our study shows that if diagnosis is based solely on the light microscope appearances of renal biopsy, diseases other than minimal change are likely to be overlooked. Accuracy of diagnosis in structural terms requires additional immunofluorescence and electron microscopic study; final clinical diagnosis also requires careful follow-up, and repeat biopsy may be necessary.