Lingual osseous choristoma. Report of 2 cases.
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Biomedical subjects
Publications and source records attributed to T Yamamura.
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A case of malignant fibrous histiocytoma (MFH) developing in a burn scar is presented. The patient was burnt at 3 years of age and 25 years later, a rapidly growing tumour developed in the burn scar. MFH was diagnosed by light and electron microscopy and by an immunoperoxidase method. This complication of a burn scar has not been previously reported.
The components of the thready material in skin lesions of pseudoxanthoma elasticum, recessive Type I, were analysed. Immunoelectron microscopic observation revealed the presence of fibrinogen in the thready material. Part of the fine fibrils in the thready material was sensitive to collagenase. The thready material was also stained by ruthenium red. These results suggest that the thready material is composed at least of fibrinogen, collagenous protein and glycoprotein.
A 26-year-old woman developed a verrucous papule on the dorsum of the right hand during the treatment of housewive's hand dermatitis. Histological and ultrastructural study showed a typical appearance of Pagetoid reticulosis (Woringer-Kolopp disease).
We analyzed the heterogeneity of apo E in very low density lipoprotein from 58 hyperlipidemic subjects with or without atherosclerosis, 69 patients with ischemic heart disease, and 100 apparently healthy individuals. Apo E gene frequencies in the group of healthy individuals were comparable with those in German and American populations. The distribution of six common apo E phenotypes in the groups of hyperlipidemia and ischemic heart disease was similar to that in the healthy group. In addition to the three major isoforms of apolipoprotein E (apo E-4, E-3, and E-2) and the new one (apo E-5) which was recently found in this laboratory, we have discovered an additional series of components, which showed themselves as at least three bands on an isoelectric focusing gel in the region of E-VII through E-V, in four patients with hyperlipidemia and atherosclerosis. The new series of apo E components, named apo E-Suita, was identical with the ordinary apo E in its interaction with heparin-Sepharose gel and with anti-apo E antibody. The most basic component of apo E-Suita (E-VII) was the unsialylated form and other components (E-VI and E-V), the sialylated forms. Family studies revealed that apo E-Suita was determined by inheritance of a new apo E allele located at the same locus as the hitherto known apo E components. Apo E-5 and apo E-Suita isoproteins had isoelectric points more basic than apo E-3, the parent type, by two and four units of charge, respectively. While the apo E-Suita isoprotein had the same molecular weight as ordinary major apo E isoproteins, the molecular weight of the apo E-5 isoprotein was approximately 1,500-2,000 lower than the other apo E isoproteins by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. The incidence of abnormal apo E components, including apo E-5 and apo E-Suita, was high among patients with hyperlipidemia and ischemic heart disease (7:127), while we could not find such components among 100 healthy individuals. Moreover, five of seven patients with the abnormal apo E had overt atherosclerotic disease. The findings suggest that these kinds of apolipoprotein mutation are closely related to the development of atherosclerosis.
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Changes of coagulation and fibrinolysis with regard to the difference among histopathological features of cancerous tissue and postoperative alteration of hematological disorders were studied in 95 patients with gastric cancer. Platelet, fibrinogen, platelet aggregation and FDP were increased and plasminogen, alpha 2-macroglobulin and antithrombin III were decreased as the stage of the cancer progressed. Increase of these hematological disorders with the advance of cancer was observed regardless of histopathological characteristics. Although degree of hematological derangement were increased with the advance of medullary type of cancer, these findings were not observed in scirrhous cancer. Furthermore, fibrin deposition around cancerous tissue was not observed in scirrhous cancer but in medullary cancer. After complete resection of cancerous tissue was performed, hematological disorders were normalized. On the other hand, platelet, fibrinogen, plasminogen, alpha 2-macroglobulin and antithrombin III were decreased and FDP was increased markedly after incomplete resection of cancer. These results suggested that hematological disorders in the advanced stage of gastric cancer, especially medullary cancer, were inclining to the level of DIC and patients in case of incomplete resection of cancer might be on the verge of DIC with residual cancerous tissue.
Combined drug therapy with cholestyramine and compactin was found to be extremely effective against heterozygous cases of familial hypercholesterolemia. With a single drug regimen, compactin at a dosage of 15 mg/day produced a cholesterol reduction of 23% (70 mg/dl) in cases of combined hyperlipidemia, while twice the dosage (30 mg/day) was needed to produce a comparable effect with heterozygous familial hypercholesterolemia. When compaction was combined with cholestyramine (in a 4 g dose three times a day), the cholesterol-lowering effect of compactin was strongly improved with heterozygous familial hypercholesterolemia; half the dosage of compactin was enough to produce the additive effect compared with the effect produced by each single drug regimen.
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Two siblings with marked reduction of plasma high density lipoprotein (HDL) were found in a Japanese family. Their plasma cholesterol levels were very low (30-60 mg/dl), especially in the HDL fraction (0-1 mg/dl). The concentration of apolipoprotein (Apo) A-I in their plasma was 2-3 mg/dl and that of Apo A-II was 1.5-2.0 mg/dl, determined by means of a single radial immunodiffusion technique. An ultracentrifugally separated HDL fraction contained two different populations of lipoprotein particles, as shown by electron microscopy; a small particle with a diameter of 50-70 A and a relatively large particle at 200 A. Plasma lecithin: cholesterol acyltransferase activity was substantially retained in both cases. Hepatosplenomegaly was present and liver biopsy revealed lipid deposition in reticuloendothelial cells, although the tonsils were apparently normal. No severe atherosclerotic lesions were noticed. The results from these two cases were consistent with the characteristic features of homozygotes of familial HDL deficiency (Tangier disease). HDL cholesterol levels were relatively low in the parents and two children from one patient, which is consistent with the heterozygote state. Two other cases in the kindred were also found to have relatively low HDL cholesterol levels, besides these 4 cases of obligate heterozygotes. Apo A-I and Apo A-II levels in the plasma of the obligate heterozygotes, however, were within the normal range. Plasma low density lipoprotein in the patients moved faster in polyacrylamide gel electrophoresis than those of normal subjects, as did those in the heterozygotes.
The plasma pancreatic polypeptide response to a meal was compared in 6 healthy controls and 30 patients with gastric cancer who had undergone either subtotal gastrectomy or total gastrectomy with radical lymph node dissection including sympathectomy. Twelve patients were reconstructed with Billroth I, 9 patients with Billroth II, 6 patients with a double tract, and 3 patients with Roux-en-Y. Ten patients with a gastric ulcer who had undergone Billroth I gastrectomy including pyloric ring preservation also were examined. Impaired pancreatic polypeptide secretion was noted only in Billroth II and Roux-en-Y patients, where the duodenum is not affected by the passage of meals. Billroth I and double tract patients, in contrast, and an enhanced pancreatic polypeptide secretion. However, in BI patients with pyloric ring preservation the PP response to a meal was almost normal. These findings suggest an important role of the duodenum in the entero-PP axis in man.
In order to investigate the mechanism of GIP secretion and the role of endogenous GIP in the enteroinsular axis in duodenal ulcer patients, we have compared plasma GIP, insulin, and blood glucose responses to oral glucose ingestion in 10 duodenal ulcer patients, 5 patients with total gastrectomy, and 20 normal subjects. The mean basal level of plasma GIP in totally gastrectomized patients was significantly higher than in normal subjects, while in duodenal ulcer patients the value was not different from that of controls. Plasma GIP and insulin responses to oral glucose loading were significantly higher than normal in both groups. The degree of exaggerated plasma GIP and insulin secretions was more prominent and earlier in totally gastrectomized patients than in duodenal ulcer patients, and was positively correlated with the blood glucose increase during glucose ingestion. On the other hand, no significant change in GIP secretion during insulin-induced hypoglycaemia was observed in normal subjects, duodenal ulcer patients, or patients with selective proximal vagotomy. These findings indicate that the exaggerated GIP response to oral glucose in duodenal ulcer patients may be due not to increased vagal tone, but to more rapid incoming load. We found also that the hypersecretion of GIP induced by glucose ingestion in patients with duodenal ulcer and total gastrectomy may be responsible for the hyperfunction of the enteroinsular axis in these patients.
Adenosine-5'-triphosphate (ATP) produced the rapid relaxation followed by the contraction in the gallbladder strips from guinea pig. The contractile response to ATP was antagonized with theophylline, but not with apamin and atropine. The dose-response curve for ATP-induced contraction was shifted to the right by theophylline. The relaxation induced by ATP was blocked by apamin, but not by theophylline, phentolamine and propranolol. The dose-response curve for ATP-induced relaxation was shifted to the right and downwards by apamin. These results indicate that two kinds of receptors for ATP exist in the guinea pig gallbladder and the theophylline-sensitive receptors may be involved in the contractile response and the apamin-sensitive receptors in relaxation.
Exogenously applied adenosine 5'-triphosphate (ATP) caused a biphasic response; a quick relaxation followed by a slow contraction. This response was not affected by atropine, phentolamine, propranolol and tetrodotoxin. Theophylline, quinidine and IBMX reduced ATP-induced contraction and had no effect on ATP-induced relaxation. On the other hand, apamin reduced ATP-induced relaxation and had no effect on ATP-induced contraction. Indomethacin remarkedly reduced ATP-induced contraction without affecting the relaxation. It was suggested that there were two types of receptors (apamin sensitive and apamin resistant) in the guinea-pig gallbladder.