Biomedical subjects
Publications and source records attributed to B B Scott.
Although there are many causes and associations of erythema nodosum, it remains a useful sign often giving a clue to the diagnosis of a puzzling illness. It is therefore important for clinicians to be aware of all the possible causes and associations. The following report is of a patient in whom typical erythema nodosum developed during the course of severe salmonella gastroenteritis.
Twenty-five jaundiced patients presenting to a district general hospital were investigated by both ultrasonography (performed by a medical physicist) and hepatobiliary scintigraphy using 99Tcm-HIDA. Hepatobiliary scintigraphy was completely unable to distinguish medical from surgical jaundice. However, with mild jaundice--bilirubin < 100 mumol/l--the presence or absence of gall-bladder activity fairly accurately predicted the presence or absence of gall-bladder disease. Ultrasonography had three technical failures. Of the remaining 22 patients, medical jaundice was correctly predicted in nine of ten patients and surgical jaundice in eight of 12 patients. Erroneous results were only obtained from patients with mild jaundice, the accuracy being 100% in the 15 patients with serum bilirubin > 60 mumol/l. In a district general hospital ultrasonography is recommended as the investigation of first choice in jaundice, perhaps complemented by hepatobiliary scintigraphy when the jaundice is mild.
A patient is reported with hypobetalipoproteinaemia and clinical features resembling the Bassen-Kornzweig syndrome (abetalipoproteinaemia) more completely than previously described. This supports a link between hypobetalipoproteinaemia and abetalipoproteinaemia and it is suggested that the Bassen-Kornzweig syndrome has a wide spectrum with serum betalipoprotein ranging from absent to normal. It is likely that there are different genetic entities with similar end results.
A case is reported of a former woman astronaut candidate who withstood the rigors of the preliminary physical examinations for this position. Some years later, she sustained a subarachnoid hemorrhage from an arteriovenous malformation in the right parieto-occipital area, which was successfully excised. Postoperatively, she had a marked visual deficit, from which she completely recovered within 3 months. The development of psychomotor seizures 5 months later was due to probable scarring in the right parieto-occipital region of the cerebral cortex, the interpretive area for orientation of body image in space, which had been supplied by the clipped right anterior and posterior cerebral arteries feeding the arteriovenous anomaly. These seizures have been well controlled on anticonvulsants. A lesion in the temporoparieto-occipital region due to a hemorrhage from a ruptured arteriovenous anomaly, resulting in the disabling symptoms of disorientation or loss of body image due to impairment of the interpretive cortex, could be devastating to the pilot and a mission. This case raises the question of an automatic use of the CT brain scan in screening potential space candidates, and even the consideration of a percutaneous femoral four-vessel arteriogram in all, or possibly selected, candidates. Most neurosurgeons and neuroradiologists probably would consider the risks of the latter procedure too great to justify its use for fear of permanent complications to the space candidate.
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An arteriovenous fistula which shunted arterial blood from the circle of Willis to the internal jugular vein was created in ten monkeys. Continued patency of the shunt was confirmed angiographically. Cerebral blood flow measurements were then obtained with the shunt both open and occluded. Flow was also determined in response to elevations of systemic arterial pressure and raised arterial carbon dioxide tensions (PaCO2), again with the shunt both patent and occluded. In addition, flow through the shunt alone was measured in response to elevations of systemic arterial pressure and PaCO2. The results suggest that flow through our arteriovenous shunt is minimally autoregulated to changes in systemic arterial pressure and is only slightly responsive to changes in PaCO2.
Jejunal immunofluorescence studies have shown that there is a significantly increased incidence of extracellular IgA and complement in the basement membrane zone and lamina propria of untreated adult coeliac patients compared to coeliac patients on a gluten-free diet. IgG was also demonstrated with complement, particularly in the untreated patients. These findings, taken together with those of other studies, suggest that a local antibody-antigen reaction involving IgA and complement may be responsible for the ongoing mucosal damage in untreated coeliac disease as well as for the acute damage following gluten challenge of the treated patient. Furthermore, consequent upon this damage there may be a secondary IgG antibody response, possibly reticulin antibody, contributing to the mucosal damage.
In view of data suggesting that deficiency of gamma glutamyl transferase (GGT) may be the primary abnormality in the small-intestinal mucosa of patients with coeliac disease, this enzyme was measured in mucosal biopsies from coeliac patients at different stages of treatment. Activity was only slightly reduced in only one of nine well-treated patients, suggesting that deficiency of this enzyme has no primary role in the aetiology of coeliac disease.
In a routine adult gastroenterological practice, seventeen (15%) of 123 patients had small-intestinal biopsies which were neither normal nor were completely flat as found in classical coeliac disease. Some of these had a possible cause for the mild mucosal abnormality, but in six there was no other apparent cause and all four of these who were treated with a gluten-free diet responded morphologically and symptomatically. It is concluded that at least these four patients have coeliac disease in a mild form and that the classical flat biopsy is not a prerequisite for the diagnosis of coeliac disease.
In a study to determine the most suitable method of expressing activity of jejunal mucosal enzymes, the effect of variation of thickness and protein concentration of biopsies was assessed. Despite wide variation of both these factors, we found no advantage in expressing enzyme activity by reference to either surface area or protein content. Expression of activity by reference to weight of biopsy gave the lowest variance for each of the enzymes studied and, since it is also the easiest measurement to make, it would appear to be the preferred means of expressing jejunal mucosal enzyme activity.
The frequencies of histocompatibility antigens (HL-A) have been determined in patients with chronic active hepatitis, cryptogenic cirrhosis, and alcoholic cirrhosis, and were compared with those in a control group of 900 healthy subjects. No significant differences were found and the previously reported increased frequency of HL-A8 in chronic active hepatitis has not been confirmed. The discrepancy from some previous reports could not be explained by different histological, immunological, or biochemical features. However, in view of the variation in incidence of a number of features of the disease from series to series, it seems possible that our findings may reflect differences in the selection of patients or real differences in the disease entity seen by different workers.
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Serum milk, egg, and gluten antibody titres were measured in ninety men with acute myocardial infarction and compared with those of thirty-six age-matched male controls. None of the antibody titres was higher in the patients with myocardial infarction, nor was there a significant correlation between antibody titres and the Norris prognostic index or death before hospital discharge. The results do not support the suggestion that immunological mechanisms are involved in the pathogenesis of coronary heart-disease and atherosclerosis.
A diagnosis of acute, cervical, spontaneous spinal epidural hematoma was made in two patients by combining clinical signs and myelographic findings. If surgical decompression can be accomplished early, the results are favorable.
The incidence of histocompatibility (HLA) antigens in 100 men with myocardial infarction was compared with the incidence in 500 healthy controls. No significant difference was found, providing no support for either a genetic or an immunological basis for myocardial infarction.
The possession of the histocompatibility antigen HL-A8 has been correlated with serum levels of antibodies to gluten and a variety of other dietary, viral, and bacterial antigens and autoantigens. The only significant correlation with HL-A8 was of raised levels of gluten antibody. Thus it seems that HL-A8 may be associated with an enhanced immune response specifically to gluten, and not with a generally enhanced immunological responsiveness as has been suggested.
Using diagnostic criteria which are currently accepted as most reliable we have found that 19% (9/47) of patients with dermatitis herpetiformis (DH) have no evidence of coeliac disease. The incidence of HL-A8 in the DH patients was 78%, which is considerably greater than that in healthy controls and no different from that reported in coeliac disease. Furthermore, the incidence of HL-A8 was just as much increased in those DH patients without evidence of coeliac disease suggesting that HL-A8 is associated with DH per se--that is, regardless of its association with coeliac disease.
Fourteen coeliac patients on a gluten free diet (GFD) and 10 on a normal diet were studied by lymphocyte transformation in response to PHA to assess the integrity of cell-mediated immunity (CMI). Transformation was depressed in the majority taking a normal diet, with improvement after a GFD. In some patients the depression may have been due to a serum factor, as transformation was more nearly normal when the lymphocytes were cultured in pooled AB serum than in their own serum. There was no correlation between transformation and nutritional deficiencies. Mantoux tests were performed in some of these and other coeliac patients and there was a very significant reduction in the incidence of positive tests compared with controls. These findings provide evidence of depressed CMI in coeliac patients taking a normal diet with improvement on a GFD and may be of relevance to the high risk of malignancy in coeliac disease, further strengthening the case for a strict GFD.