Biomedical subjects
Publications and source records attributed to S K Price.
Heterotopic thyroid tissue, presumably due to an embryological developmental abnormality, was discovered incidentally within the right atrial wall of an elderly woman who underwent coronary arterial aortic saphenous vein bypass grafting. As far as we are aware, this is only the third patient with thyroid heterotopia of the heart to be documented.
A 20-yr-old black male was admitted with a 5-month history of profound weight loss and diarrhea. Appetite and dietary intake had been remarkably well preserved up until the week before admission. The severity of his depletion was evidenced by a body weight of only 38% of standard, multiple electrolyte deficiencies, and reduced metabolic expenditure, protein turnover, protein synthesis, and pancreatic function. Immunological defects included diminished lymphocyte numbers, lymphocyte transformation, gamma-globulin concentration, and cell-mediated immunity. A diagnosis of alpha-heavy chain disease (alpha-HCD) was made on endoscopic duodenal biopsy and serology--lymphoma being excluded by scanning and laparotomy. Treatment consisted initially of intravenous nutrition (because of the extreme malnutrition, severe diarrhea, and malabsorption of fluid, electrolytes, carbohydrates, and fat) and oral tetracycline. Response was dramatic, with a doubling of body weight within 6 wk, and resolution of malabsorption. He was discharged on a normal diet and long-term oral tetracycline (250 mg/day), and at 1-yr follow-up, nutritional status and gut function were normal despite persistence of duodenal mucosal abnormalities and markers of alpha-HCD and bacterial overgrowth. These results suggest that the malabsorption initially identified in this patient was not due simply to the mucosal abnormalities that characterize alpha-HCD, but was more a consequence of the superimposition of nutrient maldigestion and absorption resulting from the extreme state of protein deficiency and its effects on gut and pancreatic function.
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Macroscopic 'TNM' staging was performed during 78 consecutive operations for gastric carcinoma and compared with subsequent pathological staging. Surgical assessment was correct for tumour (T) in 60 per cent when depth of invasion was assessed, for nodes (N) in 61 per cent, for liver metastases (M) in 92 per cent but for all aspects in only 21 per cent. Curability (conservatively defined as T1-3, N0-1, M0) was correct in 8 of 18 patients thus assessed at surgery and incurability was pathologically correct in 58 of 60 patients. Despite inaccurate surgical staging, no patient was denied a resection although 10 patients had unduly radical procedures for their stage and 2 had inappropriately conservative procedures for their stage (but without evidence of residual disease). Staging errors did not jeopardize conventional surgical management substantially and use of intra-operative microscopic sampling of nodes would have improved surgical treatment only minimally.
Analysis of DNA from the mucosal tissue of three patients with immunoproliferative small intestinal disease (IPSID) and alpha chain disease, two of whom had early stage disease responsive to antibiotics, showed monoclonal heavy and light chain gene rearrangements in all cases. These findings suggest that IPSID is neoplastic even in its early stages, but that the neoplastic cells respond to normal stimuli. Monoclonal lymphoid populations could not be detected in circulating lymphocytes from these patients, which raises the possibility that the circulatory pathways of lymphocytes derived from human gut associated lymphoid tissue may not necessarily parallel those in experimental animals.
Case reports of three teenage patients with nonfamilial diffuse juvenile polyposis are presented to illustrate the considerable morbidity and significant growth retardation that may occur. Although generally considered to have no malignant potential, histologic findings revealed features ranging from adenomatous change to severe dysplasia. It is suggested that a conservative approach may not always be appropriate in this condition.
Two cases of papillary and cystic neoplasm of the pancreas (PCN) occurring in 17 and 21 year old women are reported with ultrastructural and immunohistochemical findings. A review of the English literature shows that although potentially malignant, PCN, which occurs mainly in young women, is amenable to surgical cure. The significance of large PAS positive and alpha-1-antitrypsin positive tumour granules; the lack of specificity of alpha-1-antitrypsin for pancreatic acinar cells; the possibility of acinar differentiation of PCN; and its separation from pancreaticoblastoma are discussed.
Four cases of Mediterranean lymphoma, in two of which serum alpha chains had been identified, were investigated with immunohistochemical techniques. In addition to alpha chains of alpha 1, subclass, monotypic light chains were identified in infiltrating tumour cells in all four patients and in plasma cells in two. The pattern of staining was in keeping with loss of light chain occurring with plasma cell differentiation and strongly suggested that the plasma cells and tumour cells shared a common clonal origin. In two patients concentrations of dendritic reticulum cells were identified in the tumour. These results support the suggestion that Mediterranean lymphoma is a tumour of follicle centre cells which undergoes plasma cell differentiation as a result of exposure to lumenal antigen.
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A 25-year-old refrigeration technician died 85 days after accidental massive anhydrous ammonia inhalation; the case history and autopsy findings are described. The latter included bronchiectasis and obliterative bronchiolitis, but interstitial fibrosis and emphysema were conspicuously absent. The roles of dosage, infection, and healing are discussed.
Clinical experience with 125 patients with abdominal tuberculosis (46 of whom had intestinal tuberculosis, 8 mesenteric tuberculous lymphadenitis and 71 the peritoneal form) is presented. The diagnosis, especially in the intestinal cases, was seldom considered and a wide variety of conditions was simulated. Ileocaecal tuberculosis accounted for only 11 (24%) of the intestinal cases, while 17 patients (37%) had more distal involvement. A number of patients with diffuse colonic tuberculosis were seen. Chest radiographs showed that 51% of the 125 patients had active tuberculosis, but were completely normal in 38% of cases. Tuberculin skin testing was negative in 45% of cases. The pathogenesis, pathological features, diagnostic difficulties and complications are discussed, and the importance of considering the diagnosis is stressed.
Etomidate (Hypnomidate; Janssen) 1,25% in sterile water was given rectally on 100 occasions to 50 male Long-Evans rats in doses ranging from 4 mg/kg to 12 mg/kg. The onset and duration of ataxia and hypnosis (i.e. loss of righting ability) were recorded. Ataxia was observed in all rats, even at the lowest dose levels. The lowest hypnotic dose was 6 mg/kg, when 2 out of 5 rats lost their righting ability. In all 50 rats given 8 mg/kg or more hypnosis occurred within 4 minutes (range 2-4 minutes, average 3,3 minutes), from which they recovered within an average of 10,4 minutes. The duration of hypnosis and time to full recovery from ataxia were dose-dependent. The rectal hypnotic dose of etomidate in rats has been found to be approximately ten times the documented intravenous dose. No mortality was recorded despite the rectal administration of ten times the intravenous lethal dose (LD50) for rats. Histological examination of the rectal and colonic mucosa showed that etomidate 1,25% in sterile water (pH 3,5) caused no significant mucosal change, but undiluted etomidate 12,5% (pH 1,8) caused haemorrhagic necrosis. We conclude that rectal etomidate 1,25% in sterile water is an efficient, predictable, evanescent and safe method of inducing anaesthesia in rats and warrants further investigation in clinical anaesthesia in children.
Patients with inflammatory bowel disease and serum alkaline phosphatase persistently raised to more than twice the normal level were investigated to assess the frequency of primary sclerosing cholangitis (PSC) in the Gastro-intestinal Clinic from 1975 to 1981. Twelve patients had a persistently raised alkaline phosphatase level of hepatic origin, 9 out of 250 with ulcerative colitis and 3 out of 164 with Crohn's disease. PSC was demonstrated in 8(3%) of the patients with ulcerative colitis, and carcinoma of the pancreas in the remaining 1. Three of the patients with PSC also had gallstones. The colitis antedated the biliary symptoms and signs in all but 1 patient. There was no correlation between the duration, extent and activity of the colitis and the development and outcome of the liver involvement. Investigations in the 3 patients with Crohn's disease revealed the presence of PSC in 2 (1,2%) and chronic active hepatitis in the 3rd. Of the 2 with PSC, one had cholelithiasis and has had recurrent episodes of cholangitis. The other has had only mild symptoms.
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Prostaglandin E was measured by radioimmunoassay in endoscopic biopsy specimens obtained from 7 normal healthy volunteers, 28 patients with a benign gastric ulcer, and 10 with a malignant gastric ulcer. Biopsy specimens were taken from the antral and body mucosa and from the ulcer edge in patients. The median value in normal antrum specimens was 5.15 (4.44-8.40) ng PGE/mg protein which was more than the body with 3.50 (1.86-9.28), p less than 0.05. In the 28 patients with benign gastric ulcer the ulcer edge contained 2.33 (0.48-7.67) ng PGE/mg protein which was more than the antrum with 1.06 (0.30-4.17) or the body with 0.97 (0.20-7.67), p less than 0.01 respectively. The antral and body levels in patients with gastric ulcers were lower than those found in the normal volunteers, p less than 0.01. There was no difference between the levels found in patients with malignant as opposed to benign gastric ulcer. In patients with gastric ulcer lower levels of PGE were found in body mucosa with atrophic gastritis 0.83 (0.53-2.66) ng PGE/mg protein as compared with histologically normal body mucosa 2.55 (0.63-7.67), p less than 0.05. These results suggest that mucosal PGE levels are reduced in patients with gastric ulcer disease and that this may be due to the presence of atrophic gastritis.
A case of renal malakoplakia associated with prednisone therapy is reported; clinical recovery had taken place 6 months later, after nephrectomy and cessation of corticosteroid administration.
The changes of myospherulosis were observed in masses from the buttocks of three patients. Ultrastructural examination of parent bodies and spherules from two patients revealed some spherules to contain dense bodies and filaments. Spherules had a double electron dense wall: the outer layer had a thickness corresponding to that of a cell membrane. Incubation of packed red blood cells with tetracycline ointment produced a similar electron dense deposit on the inner aspect of the cell membrane of the erythrocytes. No filaments or parent bodies were seen. Our findings support the hypothesis that myospherulosis represents altered red blood cells. The filaments seen in our patients may represent polymers of haemoglobin. It is suggested that parent bodies may be derived from histiocytes.