[A case of 48,XXYY syndrome with recurrent foot ulcers and renal hypouricemia. Effective treatment of ulcer by autologous skin transplantation].
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Adult↗
Biomedical subjects
N Kono
Publications and source records attributed to N Kono.
[An autopsy case of ductuloinsular tumor].
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Adenoma, Islet Cell↗
Morphological study of lipoprotein particles in the livers of ventromedial hypothalamus lesioned rats.
Ultrastructure of the liver in ventromedial hypothalamus (VMH) lesioned rats was studied in parallel with biochemical examination. Ten weeks after the operation, VMH lesioned rats became markedly obese. Electron microscopic examination of the liver revealed lipid droplets in the cytoplasm and a high load of lipoprotein particles in the Golgi elements. Lipoprotein particles, 40-50 nm in diameter, in the Golgi elements, were scattered throughout the cytoplasm. Concentrations of triglyceride and esterified cholesterol markedly increased in the liver, while those of free cholesterol and phospholipids did not increase. Serum concentrations of triglyceride, total cholesterol and HDL-cholesterol in VMH lesioned rats were higher than control ones. These data suggest that lipoprotein synthesis was markedly increased in the livers of VMH lesioned rats.
Animals↗
Effects of the intravenous administration of magnesium sulfate on corrected serum calcium level and nephrogenous cyclic AMP excretion in normal human subjects.
The effect of intravenous magnesium sulfate infusion on corrected serum calcium level and parathyroid function assessed by determination of nephrogenous cAMP (NcAMP) excretion were studied in normal human subjects. Significant hypermagnesemia induced by the magnesium sulfate infusion for 120 minutes was accompanied by a gradual and progressive decrease in the corrected serum calcium level. NcAMP excretion fell rapidly, reaching a nadir between 60 and 120 minutes after the infusion began, and after that rose above the baseline excretion. Urinary calcium excretion gradually increased, reaching a peak between 120 and 180 minutes after the infusion began and then gradually decreased. Since magnesium was given as the sulfate, it is not clear whether these changes were attributable to magnesium or sulfate or both. As a control study, we performed intravenous sodium sulfate infusion. The sodium sulfate infusion caused slight hypocalcemia, slight hypercalciuria, and a significant increase in NcAMP excretion. These findings indicate that the hypocalcemia and the hypercalciuria caused by the magnesium sulfate infusion is mainly due to the effect of magnesium, and that the decrease in NcAMP excretion during the infusion is due to the effect of magnesium alone. We conclude that the hypocalcemia caused by the magnesium sulfate infusion is mainly due to the renal calcium loss, and that the inhibition of parathyroid function caused by hypermagnesemia may be only partially involved in the early phase of this hypocalcemia.
Adult↗
Ultrastructural and immunocytochemical aspects of lymphocytic submandibulitis in the non-obese diabetic (NOD) mouse.
The submandibular glands of female non-obese diabetic (NOD) mice (22-26 weeks of age) were studied by light and electron microscopy. Mononuclear cells consisting mostly of lymphocytes were recognized in and among the acini and secretory ducts. Some parts of the secretory ducts and mucous acini surrounded by lymphocytes showed destructive changes. In the secretory ducts lymphocytes invaded the duct epithelial lining and the duct lumen was occluded by these cells. The duct epithelial cells in such lesions were extremely distorted and tonofilament bundles running in various directions were present in the cytoplasm. Lymphocytes were in close contact with the duct epithelial cells. In the mucous acini some acinar cells, which appeared to be compressed by the infiltrating lymphocytes, showed degenerative changes. Immunocytochemical study revealed that both T- and B-lymphocytes were involved, T-lymphocytes tending to occupy the center of the infiltrate, while B-lymphocytes occupied the periphery. Although autoantibody against duct epithelial cells was identified, damage to duct epithelial cells was not correlated with the presence of this antibody. The morphological changes in the submandibular gland of the NOD mouse are very similar to those reported in the salivary gland of patients with Sjögren's syndrome.
Animals↗
Elevated antibody-dependent cell-mediated cytotoxicity and its inhibition by nicotinamide in the diabetic NOD mouse.
Antibody-dependent cell-mediated cytotoxicity (ADCC) by splenic mononuclear cells was measured in female non-obese diabetic (NOD) mice and age-matched ICR mice. No significant difference in ADCC activities was observed between the two groups when all the NOD mice were pre-diabetic. ADCC activities in diabetic NOD mice were significantly higher than those in age-matched ICR mice (P less than 0.001). Nicotinamide, known to prevent the diabetes of the NOD mouse, strongly inhibited ADCC by the mononuclear cells from diabetic NOD mice. Kinetic studies revealed that the inhibition was non-competitive.
Animals↗
Cytoplasmic hyalins resembling Mallory's alcoholic hyalins in pulmonary carcinoma cells.
Two cases with Mallory body (MB)-like cytoplasmic hyalins in cells, which were quite uncommon as MB-having cells, were presented. The cases were 61-and 59-year-old males. MB-like hyalins were found in large cell carcinoma cells of giant cell type of the lung. The cells containing MB-like hyalins frequently showed a hydropic swelling of the cytoplasm with pyknotic, or wrinkled nuclei. Histochemical properties of the hyalins were just similar to those of hepatic MBs in alcoholic liver diseases. Electromicroscopically, the hyalins were composed of granular, or homogeneous electron dense materials with the peripheral meshwork of randomly oriented fibrils measuring about 100A in diameter. These were stained with anticytokeratin antibodies, suggesting the relateness of the hyalins to intermediate-sized filaments of the cytokeratin type.
Carcinoma↗
Renal and prostatic malakoplakia associated with submassive hepatic necrosis. A case report with immunocytochemical, ultrastructural and X-ray analytical observations.
An autopsy case of malakoplakia involving the kidney and prostate was reported. The case was a 58-year-old Japanese male with submassive hepatic necrosis of three and half months' duration. He received 20 to 40 mg of prednisolone daily during the course of the disease (total 2,700 mg). Malakoplakic lesions were incidentally found in the right renal parenchyma and prostate at autopsy. The lesions were characterized microscopically by an accumulation of macrophages with PAS-positive intracytoplasmic granules and with intracytoplasmic inclusions of owl's eye appearance called Michaelis-Gutmann bodies and, electronmicroscopically, by numerous phagolysosomes with varying numbers of bacteria. Immunocytochemical stain revealed that the bodies were positive for lysozyme, indicating that the Michaelis-Gutmann bodies are of lysosomal origin. No calcium, phosphorous, and iron were demonstrated in them by X-ray microanalysis, suggesting that they are of immature form in which a mineralization does not take place as yet. Malakoplakia has not been previously described in association with submassive hepatic necrosis. Administration of a large amount of steroid for the treatment of submassive hepatic necrosis was suspected to be implicated in the development of the malakoplakia in the present case.
Electron Probe Microanalysis↗
Pulmonary cytoplasmic hyalin resembling Mallory's alcoholic hyalin in the liver.
Sixty-three consecutive autopsy cases of interstitial fibrosis of the lung, 6 cases of organizing pneumonia, 14 of pneumocystis pneumonia, and 20 of acute bacterial pneumonia complicating as a terminal illness listed in our Department of Pathology during a period from 1978 to 1983 were surveyed for Mallory body-like cytoplasmic hyalins in the alveolar cells. We found the hyalins in 10 of 63 cases (15.9%) with interstitial fibrosis of the lung and one of 6 cases with organizing pneumonia. Seven of the former 10 had an associated malignancy; 3 esophageal cancers, 2 lung cancers, and 2 leukemias. Five of the seven patients received an irradiation for treatment of their malignancies, subsequently developed interstitial fibrosis of the lung. Among the remaining 3 of the 10, one showed diffuse interstitial fibrosis associated with rheumatoid arthritis and two had an idiopathic type of diffuse pulmonary fibrosis. There was only one case in which the pulmonary hyalins were found in the absence of extensive interstitial fibrosis within small organizing foci of peribronchial and subpleural location. Pulmonary hyalins showed the same conventional staining properties and ultrastructural features as Mallory's alcoholic hyalins found in the liver, but did not reveal a simultaneous association with the hepatic hyalins. Pulmonary hyalins frequently stained positively with monoclonal anti-cytokeratin antibodies, more strongly at their periphery. Pulmonary hyalins were considered to be a non-specific reaction of alveolar cells to injuries, mostly in association with the pulmonary fibrosis of any etiology but not the hepatic hyalins.
Adult↗
Histological and ultrastructural examination of the intrahepatic biliary tree in primary sclerosing cholangitis.
Intrahepatic bile ducts were examined histologically and ultrastructurally in wedge-biopsied liver specimens from three patients with primary sclerosing cholangitis. Bile ducts with periductal concentric fibrosis, which is a characteristic finding in primary sclerosing cholangitis, revealed ultrastructurally finger-like projections or fine undulations of the basal free surfaces with markedly duplicated basal lamina. The lamina was collared by a layer of elongated fibroblasts and thickened bundles of collagen fibers outwards. These changes were consistently found in all sizes of ducts examined, and might be related to progressive periductal fibrosis. Serial section observations showed that some severely affected ducts actually disappeared when accompanying severe periductal fibrosis. It would therefore appear that progressive periductal fibrosis may interrupt fluid and nutrient exchange between the bile duct epithelia and peribiliary capillary plexus, followed by obliteration of the biliary lumina. Although the bile ducts showed segmentally periductal lymphocytic infiltration and, ultrastructurally, point contacts between infiltrating lymphocytes and biliary epithelial cells were observed occasionally, the exact role of infiltrating lymphocytes in the pathogenesis of primary sclerosing cholangitis remains unclear.
Adult↗
Renal hypouricaemia in a patient with 48,XXYY syndrome.
Studies on hypouricaemia observed in a patient with 48,XXYY syndrome revealed an abnormality in renal urate handling. His renal urate clearance was abnormally increased. Inosine administration and provocative tests using probenecid and pyrazinamide identified an isolated renal tubular abnormality with increased urate secretion. Since the serum urate in his brother with a normal sex chromosome constitution was also low, the association of renal hypouricaemia and 48,XXYY syndrome in this patient is probably coincidental. Although the brother was not investigated, these siblings may be a previously unreported case of familial hypouricaemia due to isolated renal hypersecretion.
Adult↗
Acute renal failure in McArdle's disease.
This is a report of a case of acute renal failure associated with McArdle's disease. A 45-year-old man had acute renal failure that required dialysis. His case was subsequently diagnosed as rhabdomyolysis secondary to McArdle's disease, a primary myopathy due to myophosphorylase deficiency. History-taking revealed nothing suspected to be responsible for precipitating the renal failure.
Acute Kidney Injury↗
Increased plasma uric acid after exercise in muscle phosphofructokinase deficiency.
Type VII glycogenosis (muscle phosphofructokinase deficiency) is attended by hyperuricemia and hyperuricosuria. In one patient, we found that exercise on a bicycle ergometer increased plasma uric acid, inosine, and hypoxanthine levels. Forearm exercise also markedly increased venous inosine, hypoxanthine, and ammonia in the exercising arm of two patients. Exaggerated release of precursors for uric acid synthesis from exercising muscle may be related to the hyperuricemia.
Adult↗
Treatment of Kearns-Sayre syndrome with coenzyme Q10.
We studied the metabolism of coenzyme Q10 (CoQ) and the effects of CoQ therapy in five patients with Kearns-Sayre syndrome (KSS). Although the mitochondrial fraction was increased in muscles from KSS patients, CoQ content was slightly low. CoQ synthesis was normal in fibroblasts from KSS patients. Administration of 120 to 150 mg/d of CoQ improved abnormal metabolism of pyruvate and NADH oxidation in skeletal muscle. CoQ therapy decreased CSF protein concentration and CSF lactate/pyruvate ratio. ECG abnormalities and neurologic symptoms also improved.
Adult↗
Demonstration of islet cell surface antibodies in sera of New Zealand black mice and inhibitory effect on insulin release.
Islet cell surface antibodies (ICSAs) in sera of New Zealand Black (NZB) and New Zealand White (NZW) mice were detected by the indirect immunofluorescence method with cultured Balb/c mouse islet cells as antigens. Circulating ICSAs appeared in NZB mice from age 20 wk; at 30 wk, 73% of male mice and 88% of female mice had detectable ICSAs. The ICSAs were significantly absorbed with mouse islet cells but hardly absorbed with spleen cells or liver powder. The ICSAs also bound with islet cells of ICR mice, Sprague-Dawley rats, and NZB mice. NZB mice showed glucose intolerance especially at ages 10 and 30 wk. Although plasma glucose levels tended to be higher in NZB mice with strongly positive ICSAs, pancreatic insulin content was not reduced, and insulitis was rarely observed in the pancreases. On the other hand, 30-wk-old NZW mice had normal or mildly impaired glucose tolerance and only weak, if any, ICSAs. The ICSA-positive serum of NZB mice significantly suppressed glucose-induced insulin release by cultured islet cells. The ICSAs may be responsible, at least in part, for glucose intolerance in NZB mice after age 20 wk through the inhibitory effect on insulin secretion.
Age Factors↗
Excess purine degradation in exercising muscles of patients with glycogen storage disease types V and VII.
To investigate purine catabolism in exercising muscles of patients with muscle glycogen storage disease, we performed ischemic forearm exercise tests and quantitated metabolites appearing in cubital venous blood. Two patients with glycogen storage disease type V and three with glycogen storage disease type VII participated in this study. Basal lactate concentrations lowered in every patient with glycogen storage disease type V or type VII. Two patients with glycogen storage disease type VII, who had markedly elevated concentrations of serum uric acid (14.3 and 11.9 mg/dl, respectively), showed high basal concentrations of ammonia (118 and 79 mumol/liter, respectively; 23 +/- 4 mumol/liter in healthy controls) and of hypoxanthine (23.4 and 20.4 mumol/liter, respectively; 2.0 +/- 0.4 mumol/liter in healthy controls). Other patients showed near normal measurements of these metabolites. After forearm exercise, ammonia, inosine, and hypoxanthine levels increased greatly in every patient studied, in contrast with the lack of increase in lactate levels. The incremental area under the concentration curves for venous ammonia was 13-fold greater in the glycogen storage disease group than in controls (1,120 +/- 182 vs. 83 +/- 26 mumol X min/liter). The incremental areas of inosine and hypoxanthine were also greater in the glycogen storage disease group (29.2 +/- 7.2 vs. 0.4 +/- 0.1 and 134.6 +/- 23.1 vs. 14.9 +/- 3.2 mumol X min/liter, respectively). The incremental areas of ammonia in controls and in glycogen storage disease patients strongly correlated with those of hypoxanthine (r = 0.984, n = 11, P less than 0.005). These findings indicated that excess purine degradation occurred in the exercising muscles of patients with glycogen storage disease types V and VII, and suggested that the ATP pool in the exercising muscles may be deranged because of defective glycogenolysis or glycolysis.
Adult↗
Improvement of abnormal pyruvate metabolism and cardiac conduction defect with coenzyme Q10 in Kearns-Sayre syndrome.
In a patient with Kearns-Sayre syndrome, concentration of coenzyme Q10, a component of the mitochondrial electron transport system, was decreased in serum and in the mitochondrial fraction of skeletal muscle. Serum concentrations of lactate and pyruvate were abnormally high, especially after exercise or oral glucose loading. Levels of folic acid in plasma and CSF were decreased. ECG showed a first-degree atrioventricular block. After administration of coenzyme Q10 60 to 120 mg daily for 3 months, serum levels of lactate and pyruvate became normal, with improvement of atrioventricular block and ocular movements.
Adult↗
Nicotinamide prevents lymphocytic infiltration in submandibular glands but not the appearance of anti-salivary duct antibodies in non-obese diabetic (NOD) mice.
Previously we have shown that nicotinamide prevents spontaneously occurring diabetes associated with insulitis in non-obese diabetic (NOD) mice. In this study we injected nicotinamide (0.5 mg/g) or saline (0.01 ml/g) into female NOD mice daily during a period between 4 and 16 weeks of age. At the end of the treatment, periductal and perivascular lymphocytic infiltration in submandibular glands was observed in 91% of saline-injected control mice and 36% of nicotinamide-injected mice (P less than 0.01). No significant difference was observed in the prevalence of anti-salivary duct antibodies or antinuclear antibodies between the nicotinamide group and the saline group. Nicotinamide may alter cell-mediated, but not humoral, immunity to salivary gland cells, resulting in the prevention of submandibulitis.
Animals↗