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Biomedical subjects

H Nishio

Publications and source records attributed to H Nishio.

At least 235 records · Page 13Linked to original sources

A case of Sjögren's syndrome with valvular diseases.

The case of a 61 year old woman with Sjögren's syndrome with aortic and mitral stenosis is reported. She suffered from rheumatic fever at a young age. Physical and echocardiographic examinations showed findings of mitral and aortic valve stenosis. In addition, she had experienced xerostomia, a gritty sensation in the eyes and Raynaud's phenomenon. Blood examination showed hypergammaglobulinemia, positive rheumatoid factor, antinuclear and anti-Ro (SS-A) antibodies. The diagnosis of Sjögren's syndrome was confirmed by sialography and biopsy of the labial salivary gland. The combination of valvular disease and Sjögren's syndrome is rare and the etiological correlation is discussed.

Antibodies, Antinuclear↗

Adrenoleukodystrophy without adrenal insufficiency and its magnetic resonance imaging.

Fatty acids of plasma and erythrocyte membrane sphingomyelin were determined by gas chromatography-mass spectrometry in adrenoleukodystrophy (ALD) without adrenal insufficiency. Mass chromatogram tracing with the ion at m/z 143 [(CH2)6COOH3]+ showed increases of saturated very long chain fatty acids in plasma and erythrocyte membrane sphingomyelin in ALD. The C26:0/C22:0 ratios in plasma were 0.121, 0.057 and 0.007 in cases 1 and 2, and a control subject, respectively. The C26:0/C22:0 ratios in erythrocyte membrane sphingomyelin were 0.386, 0.211 and 0.093 in cases 1 and 2 and the control subject, respectively. The demyelinating process of ALD was clearly observed in both the inversion recovery 2100/500 and spin echo 2100/80 scans on magnetic resonance imaging. The magnetic resonance image in case 1 revealed widespread demyelinated lesions, involving almost the entire cerebrum and cerebellum, at 4 years after the onset, while that in case 2 revealed demyelinated lesions mainly limited to parieto-occipital areas at 1 year after the onset.

Adolescent↗

Transient hyperphenylalaninaemia with a high neopterin to biopterin ratio in urine.

A case of transient hyperphenylalaninaemia with a maturational delay of dihydropteridine synthesis is described. With the Guthrie test, the patient showed a blood phenylalanine level of 38 mg dl-1, which had fallen to a normal value without a phenylalanine restricted diet by 3 months of age. The neopterin level and the neopterin to biopterin ratio in the patient's urine were very high at 19 days of age. The blood phenylalanine level did not decrease when tetrahydrobiopterin (2.5 mg kg-1) was administered at 19 days of age, while administration of tetrahydrobiopterin (7.5 mg kg-1) at 20 days of age had decreased the blood phenylalanine level to 50% of the preloading level after 24 h. The oral phenylalanine loading test showed the pattern of classic phenylketonuria (PKU) at 15 days of age, but it showed the normal pattern at 1 year 8 months of age.

Alcohol Oxidoreductases↗

Analysis of fatty acids and sphingosines from urinary sulfatides in a patient with metachromatic leukodystrophy by gas chromatography-mass spectrometry.

The urinary sulfatides in metachromatic leukodystrophy (MLD) were analyzed by gas chromatography-mass spectrometry. Fatty acids and long chain bases were obtained after methanolysis. C22:0 and C22h:0 were major components of the fatty acids distributed in the urinary sulfatides in MLD while they were only minor components of the fatty acids in the brain sulfatides in a control subject. These results were in accordance with the report of Philippart et al. It was suggested that the urinary sulfatides originated not in the brain but in other organs. The mass spectra of the trimethylsilyl derivatives of the hydroxy fatty acid methyl esters always showed peaks at m/z (M-15-28)+ and (M-59)+, indicating that the hydroxy group was on carbon 2. Two kinds of long chain base were identified: C18-sphingosine and 3-O-methyl-C18-sphingosine. The latter compound may be a by-product formed on methanolysis.

Brain Chemistry↗

Determination of pipecolic acid in rat brain areas by high-performance liquid chromatography of dansyl derivatives with fluorimetric detection.

A method for the determination of pipecolic acid in the rat brain is reported. The identification and quantification of pipecolic acid is accomplished with reverse-phase high-performance liquid chromatography including precolumn dansylation procedure by using nipecotic acid, a regio-isomer of pipecolic acid, as an internal standard. The lower limit of quantification for the method is in the picomole range. Higher concentration of pipecolic acid in the rat brain regions were found in cerebellum, medulla oblongata, hypothalamus, and midbrain than the other regions. The method establishes the usefulness of dansyl chloride for the simple and sensitive detection of pipecolic acid, and is easily adapted to routine analysis of pipecolic acid in the rat brain regions.

Animals↗

Accumulation and metabolism of pipecolic acid in the developing brain of the mouse.

In newborn mice, following i.p. injections of D,L-[3H]PA (pipecolic acid, 28 micrograms/kg), accumulation of radioactivity continues to increase up to 24 h. In adults, radioactivity peaks at 5 min and remains approximately constant up to 5 h, and then declines slowly to 24 h. Fifteen-day-old mice follow the newborn pattern, while 30-day-old mice show the same trend as the adult. Radioactivity in plasma shows essentially the same pattern of accumulation in adult and newborn animals with some quantitative differences. Secretion of radioactivity in the urine is significantly higher in the adult than in the newborn during the interval between 10 min and 5 h. Accumulation of radioactivity at 24 h in the newborn brain shows a preferential localization to the olfactory bulb, the anterior telencephalon and the diencephalon. Two hours after the i.p. injection, approximately 70% of the radioactivity recovered in brain is due to PA. This percentage increases to 75% and 87% at 5 and 24 h respectively. Alpha-aminoadipic acid (alpha-Aaa) a major metabolite of PA was identified in brain extracts at 5 h. The maximal formation of alpha-Aaa in relation to PA occurs approximately at 5 h. No other brain metabolites of PA could be identified with this chromatographic system. The present results show that access of PA to the brain is easier in the newborn mouse than in the adult. In addition, our results demonstrate, for the first time, the presence of PA metabolism in the newborn mouse.

2-Aminoadipic Acid↗

Effect of concanavalin A on 3H-5-hydroxytryptamine uptake in rabbit blood platelets: interaction with adenylate cyclase activity.

Concanavalin A (Con A) was shown to have inhibitory effect on platelet adenylate cyclase as well as 5-hydroxytryptamine (5HT) uptake. These effects of Con A were antagonized by alpha-methyl-D-mannoside, a specific inhibitor of Con A binding to glycoprotein. The effect of Con A on adenylate cyclase was partial inhibition, and Con A had no effect on prostaglandin E1 stimulated activity, indicating the adenylate cyclase which is thought to be involved in 5HT uptake might be a minor component. The same result as Con A was demonstrated in the inhibitory effect of N-ethylmaleimide (NEM) on this activity. Impermeable sulfhydryl blocking reagents and iodoacetamide had no effect on 5HT uptake. It might be necessary for the sulfhydryl blocking reagent to pass through the cell membrane in order to exert its inhibitory effect on 5HT uptake. Furthermore, the inhibitory effect of Con A on 5HT uptake was antagonized by sulfhydryl reducing reagents and adenosine. It is postulated that a NEM sensitive, sub-membrane contractile protein system might mediate the effect of Con A, and Con A might inhibit platelet 5HT uptake by affecting the adenylate cyclase system through some possible transmembrane regulatory mechanism.

Adenosine↗

[Cellular changes in malignant pleural effusion induced by intrapleural injection of BCG-CWS].

The intrapleural injection of BCG cell wall skeleton (BCG-CWS) is thought to be beneficial in the treatment of malignant pleural effusion. The study of cancer cells in pleural effusion after the intrapleural injectin of CWS showed that nuclear damage to cancer cells was slight and delayed compared to findings obtained after the intrapleural injection of Mitomycin C. After the intrapleural injection of CWS, the ratio of granulocytes in pleural effusion was rapidly elevated. This suggests that CWS induces an inflammatory response in the pleural cavity. After the inflammatory reaction, adhesive pleuritis was induced, the pleural space was reduced, leading to the disappearance of effusion. On the other hand, after the intrapleural injection of CWS, no elevation of the ratio of macrophages, and no increase of macrophage and cancer cell adherence were observed. This suggests that the immunopotentiating effect of CWS is not high.

Adenocarcinoma↗

Accumulation, elimination, release and metabolism of pipecolic acid in the mouse brain following intraventricular injection.

Following i.c.v. (intracerebral ventricular) injections of D,L-[3H]pipecolic acid (PA), it is reabsorbed from the ventricles and redistributed to various brain regions. The highest accumulation is found in three brain regions ipsilateral to the injection site, hippocampus, neocortex, striatum, and in the diencephalon. Following preloading in vivo, the radioactivity is released from hippocampus slices in the perfusion medium after depolarization induced by high K+. During perfusion with a Ca++ free medium containing EGTA, a significant reduction of release is observed. The radioactivity of D,L-[3H]PA in the brain shows a more rapid phase of decrease from 0 to 2 hours and a slower phase from 2 to 5 hours. At 5 hours, only 28% radioactivity, represented mainly by PA, is left in the brain. Kidney secretion represents the major route of elimination of the injected PA. The presence of alpha-aminoadipic acid both in brain and urine was observed. Probenecid (200 mg/kg) significantly increases the accumulation of i.c.v. injected D,L-[3H]PA in brain and kidney. The presence of a regional accumulation of PA in certain brain regions, its metabolism in brain, its enhanced retention following probenecid administration and its Ca++ dependent release following high K+ stimulation, all constitute indirect evidence for a neuronal localization of this brain endogenous aminoacid.

Animals↗

Brain uptake of pipecolic acid, amino acids, amines following intracarotid injection in the mouse.

The uptake of pipecolic acid by the mouse brain was compared to that of several amino acids and amines, following an injection of a double-labeled mixture into the carotid artery. In general, BUI (brain uptake index) values were lower in the mouse than those previously reported in the rat. The only exception was proline. Lysine, a precursor of pipecolic acid biosynthesis in brain, showed a higher BUI than pipecolic acid. The BUI of D,L-[3H]pipecolic acid was found to be 3.39 (at 0.114 mM). This was saturable between a concentration of 0.114 and 3.44 mM. Kinetic analysis suggests the presence of two kinds of transport systems. Substances structurally related to pipecolic acid, such as nipecotic acid, isonipecotic acid, L-proline, and piperidine show a significant inhibitory effect. Amont the amino acids tested, only GABA showed an inhibitory effect. Data are reported which, when considered with other findings (5), present evidence that pipecolic acid is (1) synthesized both in vitro and in vivo in the mouse brain, (2) actively transported in vivo into the brain, and (3) taken up in vitro by synaptosomal preparations.

Amines↗