[A case of familial Fabry's disease in three generation].
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Cornea↗
Biomedical subjects
H Kishikawa
Publications and source records attributed to H Kishikawa.
Histidine decarboxylase measurement in brain by 14CO2 trapping.
A method for measuring histidine decarboxylase (HDC) in crude rat brain homogenates was developed by modification of existing 14CO2-trapping methods. The addition of EDTA to tissue homogenates and assay buffer reduced non-enzymatic decarboxylation, and improved assay sensitivity and reliability. Addition of polyethylene glycol (molecular weight 300, PEG300) to the homogenizing buffer increased enzyme stability, permitting storage of crude homogenates. Studies of time course, tissue dilution and blanks showed that up to 8 mg of tissue could be assayed successfully with a 3.5-hr incubation. S-alpha-Fluoromethylhistidine (FMH) and alpha-hydrazinohistidine, specific inhibitors of HDC, induced concentration-dependent reductions of enzyme activity by up to 90%, whereas inhibitors of other decarboxylases had little or no effect. Kinetic studies of the enzyme in crude homogenates yielded Km and Vmax values similar to those found previously with other HDC methods, although a poor fit was found to a single enzyme model. When determined by the new method, the distribution of HDC in seven regions of the rat brain agreed well with previous results. The method is rapid, simple to perform, and requires no specialized equipment other than a scintillation counter.
Animals↗
Tetrasomy 8 in acute myelomonocytic leukemia developing after a gastric cancer operation.
A 70 year-old-Japanese man underwent gastrectomy for early gastric cancer with preoperative hypocellular marrow. Three weeks later, acute myelomonocytic leukemia developed with tetrasomy for chromosome 8 (48,XY,+8,+8). The patient died of leukemia on the 33rd postoperative day. Whether or not the operation triggered the leukemia remains unknown.
Adenocarcinoma↗
Abnormal glucagon response to arginine and its normalization in obese hyperinsulinaemic patients with glucose intolerance: importance of insulin action on pancreatic alpha cells.
An excessive glucagon secretion to intravenous arginine infusion was found in obese hyperinsulinaemic patients with glucose intolerance. This study was designed to determine whether the glucagon hyperresponsiveness to arginine in these patients would improve by insulin infused at a high enough dose to overcome insulin resistance. By infusing high dose insulin during arginine infusion, the previously exaggerated glucagon response to arginine could be normalized. To normalize the abnormal glucagon response, insulin doses of 4.2 +/- 0.7 and 3.8 +/- 0.5 IU were required during arginine infusion in obese hyperinsulinaemic patients with impaired glucose tolerance and Type 2 (non-insulin-dependent) diabetes mellitus, respectively. This achieved plasma peak insulin levels 3 to 4 times higher than those observed in non-obese healthy subjects. Furthermore, we clarified whether or not the effect of normalizing insulin action and/or glycaemic excursions contributed to normalizing the exaggerated glucagon response to arginine in these patients. Blood glucose was clamped while high dose insulin was infused at the same levels as observed during the arginine infusion test with no insulin infusion. As a result, normalization of the exaggerated plasma glucagon response was achieved, whether hyperglycaemia existed or not. These results clearly demonstrate that, similar to non-obese hypoinsulinaemic Type 1 (insulin-dependent) and Type 2 (non-insulin-dependent) diabetic patients, the exaggerated Alpha-cell response to arginine infusion in obese hyperinsulinaemic patients with glucose intolerance is secondary to the reduction of insulin action on the pancreatic Alpha cell, and that the expression of insulin action plays an important part in normalizing these abnormalities.
Adult↗
Carcinoma in adenoma of the ampulla of Vater synchronous with cancer of the sigmoid colon.
A 58-year-old Japanese man presented with early invasive adenocarcinoma within a papillotubular adenoma of the ampulla of Vater synchronous with advanced adenocarcinoma of the sigmoid colon. The colon had two additional adenomas. This hitherto unreported concomitance in the absence of familial polyposis coli is discussed.
Adenocarcinoma↗
Latent carcinoma of the thyroid manifested by cystic supraclavicular metastasis.
In a 68-year-old Japanese woman, who had a right supraclavicular cyst containing thyroglobulin-rich fluid, metastatic adenocarcinoma was demonstrated in the cyst. A subsequent total thyroidectomy showed a papillary carcinoma measuring 11 mm in the ipsilateral lobe of the thyroid. A high thyroglobulin level in a cervical cyst may facilitate the detection of hidden primary cancer in the thyroid.
Adenocarcinoma, Papillary↗
Diabetes insipidus after traumata of two extremes in severity.
Two patients with post-traumatic diabetes insipidus (DI) are reported. One had suffered a fatal injury and the other a mild contusion without amnesia before DI developed. These two instances exemplify the wide spectrum of post-traumatic DI and, hence, the importance of ruling out DI even afer a mild closed-head injury.
Adult↗
A case of a postoperative aneurysm of the common hepatic artery which ruptured into the remnant stomach after a radical gastrectomy.
A rare case of an iatrogenic aneurysm of the common hepatic artery which ruptured into the remnant stomach is described herein. The patient had undergone a radical gastrectomy with regional lymphadenectomy one month previously. The iatrogenic surgical trauma to the common hepatic artery incurred during the regional lymphadenectomy with the resection of the perivascular lymphatics and neural plexuses, was considered as the main cause responsible for the postoperative aneurysmal development.
Adenocarcinoma↗
[Inhibitory effects of somatostatin analog (SMS 201-995) on pancreatic hormones in patients with malignant islet-cell carcinoma].
Acute effects of somatostatin analog (SMS 201-995) on pancreatic hormones were studied in two patients with malignant islet-cell carcinoma. Before and after subcutaneous injection of somatostatin with a doses of 50 micrograms, blood glucose (BG), serum growth hormone (hGH), C-peptide immunoreactivity (CPR), plasma immunoreactive glucagon (IRG) and gastrin were assayed, and changes in elution patterns of IRG and gastrin were also analyzed on Bio-Gel P-30 column chromatography. In Patient 1 with glucagonoma syndrome and hypergastrinemia, a prompt and remarkable decrease in plasma IRG and gastrin was observed after the injection of SMS 201-995 in association with a decrease in blood glucose, and then IRG and gastrin increased gradually. The suppressive effect continued for at least 6 hours. On gel filtration of the plasma obtained before the injection of the analog, three major peaks, greater than 20000, 9000 and 3500 molecular-weight (mol wt) fractions, were seen in IRG fraction. The decrease in plasma IRG observed at 1 hour after the injection was mainly due to a marked decrease in the 3500 molecular weight fraction. In addition, a slight decrease in the 9000 mol wt fraction was seen. At 4 hours after the injection, the 3500 mol wt peak returned to the previous level, while the 9000 mol wt peak decreased further. On the other hand, the gastrin elution pattern of plasma obtained before the injection revealed three major gastrin peaks, greater than 20000, 7000 and 5000 mol wt fraction. The changes in the gastrin elution pattern after the injection were similar to those of the IRG elution pattern. In Patient 2 with Zollinger-Ellison's syndrome, the plasma gastrin level decreased gradually for 5 hours after the injection. On gel filtration of the plasma obtained before the injection, two major gastrin peaks, 7000 and 5000 mol wt fraction, of which the large-molecular fraction was more prominent than the small-molecular fraction, were observed. After the injection, a marked decrease in the small-molecular fraction and a gradual decrease in the large-molecular fraction were observed for 4 hours, accompanied by a decrease in plasma gastrin. At 7 hours after the injection, the smaller fraction was augmented again. The serum CPR and hGH was slightly suppressed after the injection in both patients. The adverse effects of slight nausea and vomiting were noticed only in Patient 1.(ABSTRACT TRUNCATED AT 400 WORDS)
Adenoma, Islet Cell↗
Effect of alpha 2-adrenoceptor antagonist on platelet activation during insulin-induced hypoglycaemia in type 2 (non-insulin-dependent) diabetes mellitus.
The role of epinephrine in platelet activation and the effect of an alpha 2-adrenoceptor antagonist, midaglizole, during insulin-induced hypoglycaemia in Type 2 (non-insulin-dependent) diabetes mellitus were examined. The action of midaglizole as a platelet alpha 2-antagonist was confirmed by in vitro studies using platelet-rich plasma and washed platelet suspension. Hypoglycaemia was induced by a bolus injection of short-acting insulin in 24 diabetic patients. They were divided into two groups, a control group (n = 12) and an alpha 2-group (n = 12), and midaglizole was administered orally 60 min before insulin injection in the latter. Blood glucose and plasma C-peptide levels were significantly decreased (p less than 0.005) by insulin injection in both groups. Counter-regulatory hormones, including epinephrine, and arginine vasopressin were similarly increased at the hypoglycaemic nadir compared with the levels at 0 min in both groups. Plasma beta-thromboglobulin was increased at the hypoglycaemic nadir (165.5 +/- 12.6 ng/ml) compared with the level at 0 min (121.0 +/- 11.5, p less than 0.005) in the control group, whereas no significant increase was demonstrated in the alpha 2-group. These results suggest that plasma epinephrine plays an important role in platelet activation during hypoglycaemia in Type 2 diabetes mellitus, and that the platelet activation is prevented by alpha 2-adrenoceptor antagonist.
Arginine Vasopressin↗
Gastric phenotypic expression in human gallbladder cancers revealed by pepsinogen immunohistochemistry and mucin histochemistry.
Gastric phenotypic expression indicated by paradoxical concanavalin A (Con A) staining for class III mucins and the immunoperoxidase method for pepsinogen (Pg) I and Pg II was found in pyloric gland metaplasia of gallbladder epithelium. Using the same methods, the features of gallbladder cancers and their relationship to pyloric gland metaplasia in the human gallbladder epithelium were studied. Histologically, 57 gallbladder cancers were classified into 5 papillary adenocarcinomas, 29 tubular adenocarcinomas, 8 poorly differentiated adenocarcinomas, 6 signet-ring cell carcinomas, 4 mucinous adenocarcinomas, and 5 squamous cell carcinomas. In papillary and tubular adenocarcinomas, Pg I and/or Pg II staining was detected in 80% and 75.9% of cancers, respectively. Pg II staining was significantly more frequent than Pg I staining. One signetring cell carcinoma also had Pg II activity. Pyloric gland metaplasias all contained class II mucins and were further classified into complete type and incomplete type on the basis of presence or absence Pg I and/or Pg II activities. A few cancer cells with class III mucins were negative for Pg staining; conversely, a few cells with Pg I and/or Pg II had no class III mucins. Phenotypic diversity in both class II mucin reactivity and Pg activities was observed in gallbladder cancer cells with the pyloric gland cell type. By comparison, pyloric gland metaplasia varied only in Pg activities. A few Pg-positive cancers were found in the gallbladder with Pg-negative pyloric gland metaplasia. The present results clearly indicate the appearance of gastric phenotypic expression in both gallbladder epithelium and gallbladder cancers and suggest the independent induction of pyloric gland metaplasia and cancer with gastric phenotypic expression.
Adult↗
Role of alpha 2-adrenergic receptor in platelet activation during insulin-induced hypoglycemia in normal subjects.
The effects of alpha 2-adrenergic-receptor blocker mianserin on the responses of blood glucose, plasma beta-thromboglobulin (beta-TG), and various counterregulatory hormones to insulin-induced hypoglycemia were studied in nine healthy male subjects. The alpha 2-adrenoceptor-blocking action of mianserin was confirmed by its inhibitory effect on platelet activation in vitro. Mianserin was given orally 90 min before insulin injection; the same study without mianserin was performed on another day as the control study. The time courses of blood glucose and serum C-peptide (0, 20, 45, and 180 min after the insulin injection) were identical in both studies, indicating that mianserin has no effect on these parameters. However, a significant increase of beta-TG at 45 min after insulin injection was completely suppressed by the administration of mianserin (mean +/- SE, 68.5 +/- 6.0 vs. 28.8 +/- 7.6 ng/ml, n = 6, P less than .05). No significant differences were obtained between the two studies in the responses of plasma or serum catecholamines, cortisol, glucagon, growth hormone, thromboxane B2, and 6-ketoprostaglandin F1 alpha. These results suggest that epinephrine is responsible for some, if not all, of the beta-TG release from the platelets during insulin-induced hypoglycemia.
Adult↗
Intramural giant gallstone: report of a rare case.
We report a case of intramural giant gallstone. Cholangiography revealed narrowing of the neck of the gallbladder due to a hemispherical prominence. At cholecystectomy, a giant gallstone 2.1 cm in diameter which was located in the submucosa of a hemispherical prominence at the neck of the gallbladder, was found. Although the usual sizes of intramural gallstones mentioned in previous reports have ranged from 2 to 5 mm in diameter, the stone found in our case is, as far as we know, the largest intramural gallstone yet reported.
Cholangiopancreatography, Endoscopic Retrograde↗
Deoxyribonucleic acid cleavage specificity of a series of acridine- and acodazole-iron porphyrins as functional bleomycin models.
A series of metalloporphyrins linked through basic chains to certain DNA interactive groups has been synthesized. Several of these agents reproduce the characteristic properties of the antitumor glycopeptide bleomycin, including the oxygen-mediated scission of DNA in the presence of thiols, antibiobic activity under aerobic conditions, and activity against human and animal tumor models. Initial screening by scission of PM2-CCC-DNA identified six of the compounds, including those bearing acridine and acodazole intercalating groups, as the most active. The specificity of the oxygen-mediated scission of a 139 base pair HindIII/NciI restriction fragment of pBR322 by these six selected agents was then determined and compared with the action of pancreatic DNase by densitometric scans. All six of these compounds produce uniform base and sequence neutral cleavage of the restriction fragment at each base site. The six active compounds bear either of two types of intercalators, 6-chloro-2-methoxyacridine or acodazole, and with linkages to the ferric binding domain of -NH(CH2)2-, -NH(CH2)3-, -NH(CH2)4-, or -NH(CH2)3NH(CH2)3- and either porphyrin or deuteroporphyrin moieties. Comparison of the Kassoc values for binding to calf thymus DNA suggests that the enhanced binding observed with the linker -NH(CH2)3NH(CH2)3- contributes to the efficiency of sequence neutral DNA scission and may be a factor in the relative anticancer activities of these agents. The iron porphyrins give no evidence of the production of base propenals in DNA degradation, and the autoradiograms clearly indicate that a phosphate group is attached to the 5' end of the oligomer. The scission is partially suppressible by catalase and superoxide dismutase.(ABSTRACT TRUNCATED AT 250 WORDS)
Acridines↗
Hemorrhagic necrosis of pheochromocytoma associated with reversible renal artery stenosis.
An unusual case of pheochromocytoma associated with renal artery stenosis is described. Despite the removal of bilateral adrenal pheochromocytoma, laboratory findings suggested the presence of residual pheochromocytoma and abdominal aortography revealed more pronounced stenosis of the right renal artery. Two months later, the undetected residual pheochromocytoma underwent hemorrhagic necrosis with acute cessation of catecholamine release. Thereafter, the patient's blood pressure decreased to a normal level with marked improvement in hypertensive symptoms. No remaining stenosis was demonstrated on follow up renal angiography. Our case suggests that constant local secretion of catecholamines may be responsible for the development of renal artery stenosis.
Adolescent↗
Nonfunctioning adrenocortical carcinoma in a young girl.
A 9 year old Japanese girl was admitted complaining of left hypochondrial pain and a large upper left abdominal tumor. There were no clinical or laboratory signs of hormonal abnormality. Intravenous pyelography showed marked compression and deformity of the kidney by a tumor. This tumor was excised together with the left kidney. The pathological diagnosis was adrenocortical carcinoma. Postoperatively, the child was given neither irradiation nor chemotherapy. Twenty-one months after the surgery, there was a hepatic metastasis, and she died 40 months after surgery from a combination of hepatic metastases and local tumor recurrence.
Adrenal Cortex Hormones↗
Effect of simultaneous infusion of a somatostatin analogue, insulin and glucose on serum triglycerides and blood glucose levels in man.
Nine non-diabetic, non-obese, normocholesterolemic normal male subjects with varied triglycerides levels were subjected to a simultaneous infusion test with a synthetic somatostatin analogue [des(Ala1, Gly2)-D-Trp8, D-Asn3, 14-somatostatin], insulin and glucose under ambulatory conditions. The levels of C-peptide reactivity, immunoreactive glucagon and growth hormone were reduced, and the level of immunoreactive insulin remained constant during the infusion. The blood glucose reached a constant value at 110-120 minutes (steady state blood glucose, SSBG) after the commencement of the infusion. The total cholesterol (TC) levels decreased slightly in the 30 minutes after the experiments were begun, and the triglycerides (TG) levels decreased gradually throughout the infusion period, due mainly to the reduction of very low density lipoprotein (VLDL). The most striking finding was the highly significant positive correlation (p less than 0.005, r = 0.868) between SSBG and the serum TG level prior to the infusion. These results indicate an important relationship between insulin sensitivity and serum TG level. High TG level may be regarded as one of the indices of insulin resistance.
Adult↗
Relationship between platelet phospholipase activity and plasma in ischemic heart disease.
Platelet phospholipase plays an important role in the metabolic responses of platelets to exogenous stimuli. The platelet phospholipase activity (PLA) was therefore studied in 38 patients with ischemic heart disease (IHD) and in 26 age-matched normal subjects who served as controls. The mean platelet PLA in the IHD group was 12.72 +/- 1.03 nmol/mg protein/30 sec which was significantly (p less than 0.005) higher than that of the normal controls (8.72 +/- 0.76). When they were classified into acute stage, such as unstable angina or acute myocardial infarction (AMI), and chronic stage, such as stable angina or old myocardial infarction (OMI), there was no significant difference between them. On the other hand, about two-fold activation of platelet PLA was observed in acute stage IHD, and 20-30% inhibition of it was demonstrated in chronic stage IHD following the addition of autologous plasma to washed platelet suspensions, suggesting that certain plasma factor(s) are responsible for such phenomena. In an attempt to identify these plasma factor(s), various substances such as serum albumin, high density lipoprotein, prostaglandin E1 (PGE1) and E2 (PGE2), and platelet activating factor were assessed by in vitro experiments. Only PGE1 and PGE2 revealed a significant effect on the platelet PLA. The relationship between plasma and platelet activity in terms of platelet PLA deserves attention since it varies according to the type and stage of IHD.
Angina, Unstable↗