Myopathy in hypokalaemic periodic paralysis: reversal with acetazolamide.
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Biomedical subjects
Publications and source records attributed to A J Thompson.
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Immunofluorescent assessment of hormone receptors in melanocytic tumours is quite feasible without loss of diagnostic material, in contrast to the impracticability of quantitative biochemical assays. Using this method, oestrogen receptors were demonstrated in 4/6, and progesterone receptors in 3/5 patients with metastatic melanoma. Receptors were not found in 3 patients with primary cutaneous melanomas of the superficial spreading type. Progesterone receptors were present in the junctional component of a naevus from one healthy person.
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Circulating immune complexes (CIC) were detected in 43.6% of 78 patients with primary IgA nephropathy by the solid-phase Clq radioimmunoassay. The IC were intermediate (9 to 17S) in size and contained IgA, IgG, and less commonly IgM. CIC were often present intermittently, correlating with episodes of macroscopic hematuria. Elevated serum IgA concentrations (38.7%) did not correlate with the detection of CIC. Similar findings were observed in sera samples from patients with Henoch Schonlein purpura and in IgA glomerulonephritis associated with alcoholic cirrhosis and/or portal systemic shunts. The factors responsible for the mesangial localization of the IC are not clear, but elevations in serum antibody titers to respiratory pathogens (mycoplasma pneumoniae, herpes virus, influenza), gut flora (E. coli 07), and bovine serum albumin suggest that common exogenous antigens may be involved in the pathogenesis. Primary defects in either mucosal antigen exclusion or reticuloendothelial IC sequestration are proposed to account for these findings.
Direct immunofluorescent tests for IgA deposits were done on biopsies of normal appearing skin from 49 patients with proven renal disease and 7 healthy individuals, in order to evaluate the method in the diagnosis of IgA nephropathy. Many of the 28 patients with IgA nephropathy had high levels of IgA deposition, often accompanied by notable deposits of IgM, Clq and fibrin, and less frequently by C3 and IgG, in small vessels of the superficial dermis. However, some of the 21 patients with other renal diseases had heavy deposits of IgA, limiting the usefulness of the test as a diagnostic aid.
Kappa light chains were demonstrated by immunofluorescence microscopy in the renal basement membranes of 6 of 10 patients with multiple myeloma subjected to kidney biopsy. Nodular glomerulosclerosis was present in 2 of these patients, but the remainder showed only minor light microscopic abnormalities. Deposition of kappa light chains in renal basement membranes may be a frequent cause of kidney disease in multiple myeloma. This deposition may be detected with electron microscopy by the demonstration of granular dense transformation of basement membranes. Occasionally there is systemic vascular deposition of kappa light chains which may be difficult to distinguish from amyloid but lacks a fibrillar character. The tissue effects of light chains in multiple myeloma seem likely to be more extensive than their deposition in renal tubules. There appears to be a greater tendency for kappa light chains to accumulate in renal and systemic basement membranes, but the mechanisms governing this deposition are unknown.
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Serial circulating immune complex (IC) determinations were performed in 24 patients with infective endocarditis (IE) using the solid phase Clq, solid phase conglutinin and 3.5% polyethylene glycol precipitation assays. Circulating IC were detected in 67% of IE patients at presentation, but in only 7% of valve lesion controls. Serial determinations produced a 75% prevalence of IC in IE. The presence of circulating IC correlated with "subacute" disease, the presence of tissue deposits of immunoglobulin and/or complement components and with certain extravalvular manifestations (immune complex type glomerulonephritis cutaneous vasculitis and musculoskeletal manifestations). Effective therapy was associated with a fall in circulating IC levels, an effect which was well demonstrated by 3 patients in whom IC rapidly fell to zero following artificial valve replacement. The results support a role for circulating IC in the pathogenesis of this disorder, and suggest that serial IC determinations are useful in following clinical progress, particularly in culture negative endocarditis.
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The radiological features of three cases of pulmonary actinomycosis in children are presented. Two of the cases had the classical roentgen features of chronic consolidation with overlying rib periostitis. The third had non specific consolidation but demonstrated extension through the diaphragm. Two of our cases were mentally subnormal children in institutional care. Their pulmonary pathology is almost certainly attributal to poor dental hygiene, a constant problem in this group of children. Hence, although pulmonary actinomycosis is rare in the general populus, we suggest it might be suspected more often in the subnormal group when chronic consolidation is present.
The solid phase Clq radioimmunoassay was used to detect immune complexes in sera from patients with systemic lupus erythematosus (14/25), rheumatoid arthritis (4/5), vasculitis (5/15), infective endocarditis (2/2), acute rheumatic fever (2/3), pre-eclamptic toxaemia (0/14), lung cancer (3/7), glomerulonephritis (26/98) and renal transplant patients (0/5). The best correlation with disease activity was seen in systemic lupus erythematosus and infective endocarditis where serial immune complex determinations were clearly of value in monitoring therapy. The findings in primary glomerulonephritis indicate only a limited usefulness of the assay in that serum immune complexes were detected in a minority (22/73) of patients with glomerular immune deposits. In particular the data do not support a role for Clq fixing immune complexes in the pathogenesis of membranous glomerulonephritis or in pre-eclamptic toxaemia.
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Primary lymphoma of the colon is rare and comprises less than 1% of large bowel malignancies. Secondary colonic involvement in patients with advanced lymphoma is not uncommon and is frequently undetected. The radiographic findings of 34 patients with colonic lymphoma were reviewed. Thirteen patients had primary tumors, while the remaining 21 had colonic involvement secondary to systemic disease. The radiologic features were classified as mucosal nodularity, endo-exoenteric mass, intraluminal mass, mural infiltration, and mesenteric invasion. One patient with diffuse mucosal nodularity developed acute colonic dilatation, while two other patients had pneumatosis coli secondary to local tumor depositions. Lymphoma is now a well-described complication of chronic ulcerative colitis, and a further case is described here. Although colonic lymphoma is a relatively rare tumor, the spectrum of radiologic changes reviewed here may enable a more accurate radiologic diagnosis.
Two patients with poorly differentiated lymphoma who developed pneumatosis coli are described. In one case, the intramural gas was associated with diffuse nodular submucosal deposits of tumour. An annular stricture due to lymphoma was the underlying aetiology of the pneumatosis coli in the second case. The pathogenesis of pneumatosis coli in the context of malignant lymphoma is discussed.
To study the association of plasma cortisol and coronary atherosclerosis, we elected 71 male outpatients who had coronary angiography as part of their evaluation at our facility. Forty-eight percent of the angiograms showed no evidence of coronary artery disease (CAD), 20% showed mild CAD, and 32% showed moderate to severe CAD. We found significant correlations between elevated serial morning plasma cortisols and moderate to severe coronary atherosclerosis. Using the odds ratio, we compared plasma cortisol to the major risk factors for coronary artery disease. Plasma cortisol was second only to serum cholesterol as a discriminator in our patient population between diseased and non-diseased patients. We found a significant correlation between plasma cortisol and cholesterol, blood pressure, and smoking- the three cardinal risk factors for CAD. The highest degree of correlation was found between cortisol and cholesterol. The possible significance of the association of cortisol and the major risk factors for CAD is discussed.
Cardiac catheterization was used to evaluate 298 asymptomatic, apparently healthy aircrewmen with electrocardiographic abnormalities. These men were identified from annual electrocardiograms and exercise tests used to screen for latent heart disease. Data from 27 additional symptomatic aircrewmen who underwent cardiac catheterization because of mild probable angina pectoris are also included. The men were grouped according to major reason for cardiac catheterization. The order of groups by increasing prevalence of coronary artery disease was as follows: abnormal treadmill test (labile lead only), supraventricular tachycardia, right bundle branch block, left bundle branch block, abnormal treadmill test, ventricular irritability, probable infarct and angina. Approximately 60 percent of the men were completely free of angiographic coronary artery disease. Risk factors and other possible causes for the electrocardiographic abnormalities are discussed. The electrocardiographic abnormalities studied have a poorer predictive value for coronary artery disease in asymptomatic apparently healthy men than in a hospital or clinic population.