[A case of generalized komuragaeri disease (Satoyoshi disease) treated with glucocorticoid].

Generalized komuragaeri disease (Satoyoshi disease) is a rare disorder of unknown etiology, characterized by painful muscle spasms, alopecia, diarrhea and various endocrine disorders. We administered glucocorticoid to a girl with this disease, resulting in a marked improvement of all clinical features. The patient was a 15-year-old girl.

Ultrasonography and magnetic resonance imaging in Leigh disease.

An infant with Leigh disease, who was the younger sister of a similarly affected infant, had been examined before the onset of the disease. Ultrasonography revealed hyperechoic lesions in the putamen and caudate nucleus during the preclinical stage. At onset, these changes extended into the cerebral cortex and medulla.

[A study on tolerance to denopamine, oral beta 1-agonist].

To investigate whether effects of denopamine become weaker or not. Initial single dose (10 mg) effects, single dose effects after long-term treatment (30 mg per day, about 40 days), and chronic effects were studied by Doppler and echocardiography in 23 patients with heart failure (NYHA II-III). 1) Chronic effects: Blood pressure and heart rates didn't change significantly whether they were measured before or after long-term treatment.

Expression of cellular retinoic acid binding protein in the developing nervous system of mouse embryo.

The expression of cellular retinoic acid binding protein, CRABP, in developing mouse embryos was immunohistochemically analyzed. Differentiating young neurons and neuronal fibers in the myelencephalon, metencephalon, mesencephalon and spinal cord in 10.5- and 12.

[Clinical electrophysiological effects of nicorandil on the conduction system in humans].

The electrophysiological effects of nicorandil on the conduction system in humans were examined. Twelve patients with sick sinus syndrome (SSS), 3 patients with AV block and 3 subjects with normal conduction system were studied. The clinical electrophysiological effects were examined before, and 5 min after intravenous administration of nicorandil 2 mg over 3 min.

Two-dimensional mapping by the high-performance liquid chromatography of oligosaccharides released from glycosphingolipids by endoglycoceramidase.

A two-dimensional sugar mapping method has been developed by which sensitive, reproducible, and simple analysis can be carried out on the structures and compositions of oligosaccharides released from glycosphingolipids by endoglycoceramidase. The oligosaccharides were labeled quantitatively with an ultraviolet-absorbing compound, p-aminobenzoic acid ethyl ester (ABEE). The ABEE-oligosaccharides were separated first on an amide-silica column and then on a C4-silica column by high-performance liquid chromatography.

Genetic relationship among the musk shrews, Suncus murinus Insectivora, inhabiting islands and the continent based on mitochondrial DNA types.

Cleavage patterns of mitochondrial DNA (mtDNA) by restriction endonucleases were examined in musk shrews collected at six trapping sites on two Japanese and two Indonesian islands, on Sri Lanka, situated close to the Indian subcontinent, and on the mainland of East Bengal in Bangladesh. No variation of mtDNAs was found among the Japanese and Indonesian shrews, despite their geographical isolation by the sera. In contrast, at least six mtDNA types were present in the Sri Lanka and the Bangladesh populations (three types for each), and these two populations seemed to be differentiated to the extent, which could be compared to the "mice-intersubspecific" differences.

[Effects of verapamil and disopyramide phosphate on the termination, reinduction and long-term prevention of paroxysmal supraventricular tachycardia].

The efficacy of verapamil and disopyramide phosphate for the termination and prevention of paroxysmal supraventricular tachycardia (PSVT) were studied electrophysiologically in 32 patients with inducible sustained PSVT (17 patients received verapamil, 15 patients received disopyramide). Twelve patients had atrioventricular nodal tachycardia, 7 had concealed and 13 had overt Wolff-Parkinson-White syndrome. Intravenous verapamil (0.

[Hemodynamic responses to sublingual nifedipine during exercise and recovery in congestive heart failure].

To elucidate the hemodynamic responses to sublingual nifedipine administration during exercise and recovery in congestive heart failure, 16 patients with dilated cardiomyopathy (DCM) and 11 controls were studied using supine bicycle exercise testing. The cardiac index (CI) was measured at rest, at peak exercise and successively during recovery by the thermodilution method. The heart rate (HR), mean blood pressure (mBP) and mean pulmonary arterial pressure (mPAP) were measured every minute.

[Coronary arterial rupture during percutaneous transluminal coronary angioplasty: a case report].

A case who developed rupture in a diagonal branch of the left anterior descending coronary artery (LAD) during percutaneous transluminal coronary angioplasty (PTCA) is reported here. The present case was 80-year-old man with severe focal stenosis of the LAD at its junction with a diagonal branch. PTCA for the LAD lesion was successfully performed, but occlusion of the diagonal branch developed later.

[Left atrial systolic time intervals: their relations to left atrial preload, afterload and acute left ventricular pressure loading].

Using pulsed Doppler echocardiography, the left atrial pre-ejection period (LAPEP) and left atrial ejection time (LAET) were studied in relation to left atrial loading and acute left ventricular pressure loading conditions in 17 patients with various heart diseases. LAPEP was defined as the time interval from the onset of a right atrial pacing pulse to the upstroke in the atrial contraction phase on the mitral flow velocity pattern; LAET was defined as the duration of left ventricular filling due to atrial contraction. 1.

Multi-institutional survey of the Aicardi syndrome in Japan.

The first multi-institutional survey of the Aicardi syndrome in Japan was performed during 1985 and 1986. Among the 20 collected cases, 9 fulfilled the typical clinical triad; infantile spasms, agenesis of the corpus callosum and chorioretinal lacuna. Three cases had agenesis of corpus callosum and chorioretinal lacuna.

Liberation of oligosaccharides from glycosphingolipids on PC12 cell surface with endoglycoceramidase.

Endoglycoceramidase (EGCase) cleaves the linkage between the oligosaccharide and ceramide of glycosphingolipids (Ito, M., and Yamagata, T., J.

Characterization of the glycosphingolipids of pig cortical bone and cartilage.

Neutral glycosphingolipids and gangliosides were extracted from pig cortical bone and cartilage. To ensure the completeness of extraction, the cortical bone was demineralized and reextracted. Globotriaosylceramide and globoside were noted to be present at high content in the cortical bone.

Purification and characterization of glycosphingolipid-specific endoglycosidases (endoglycoceramidases) from a mutant strain of Rhodococcus sp. Evidence for three molecular species of endoglycoceramidase with different specificities.

Two molecular species of endoglycoceramidase (designated as endoglycoceramidases I and II) were purified 32,700 and 43,000 times with overall recoveries of 4.8 and 2.9%, respectively, from a culture fluid of the mutant strain M-750 of Rhodococcus sp.

The glucose polymer of PC12 cells is susceptible to trypsinization.

The glucose polymer of PC12 cells (Rasilo, M.-L. and Yamagata, T.

[Left ventricular diastolic reserve during acute pressure loading in hypertrophic cardiomyopathy and dilated cardiomyopathy].

To evaluate left ventricular diastolic reserve during acute pressure loading, changes in mitral flow velocity patterns before and after the elevation of blood pressure were analyzed by pulsed Doppler echocardiography in 11 cases of hypertrophic cardiomyopathy (HCM), nine cases of dilated cardiomyopathy (DCM), and 11 control subjects. Systolic blood pressure was elevated 25% above basal values by methoxamine infusion (0.01 mg/kg/min).

Characterization of a glucose polymer from PC12 cells and neuronal cells of rat embryo.

A large-sized glucose polymer was isolated by pronase digestion from line PC12 pheochromocytoma cells metabolically labeled with [1-3H]galactose. The polymer was included on a column of concanavalin A-Sepharose and could be eluted with 10 mM methyl-alpha-mannoside. Its slight retention in a column of Bio-Gel A-5m suggested that its molecular weight was in the several millions.

[The suction signal detected by color Doppler echocardiography in patients with mitral regurgitation: its clinical significance].

The present study clarified the clinical significance of the suction signal--regurgitant signal near the mitral valve in the left ventricle--as obtained by two-dimensional color Doppler echocardiography in patients with mitral regurgitation. The study population consisted of 39 patients with various heart diseases having mitral regurgitation. The presence of a mitral regurgitant signal was determined not only in the left atrium but in the left ventricle using the long-axis view, four-chamber view, and short-axis view.

Characterization of a major neutral glycolipid in PC12 cells as III3Gal alpha-globotriaosylceramide by the method for determining glycosphingolipid saccharide sequence with endoglycoceramidase.

Neutral glycolipids in PC12 cells were examined. A major neutral glycosphingolipid, isolated from a chloroform/methanol extract of the cells, was found to contain only galactose and glucose at a ratio of 3:1 and identified as ceramide tetrahexoside by fast atom bombardment (FAB) mass spectrometry. Its saccharide sequence was determined by a new method developed here using endoglycoceramidase (Ito, M.

Occurrence of glucose polymer in undifferentiated PC12 cells.

A large glucose polymer was found, following pronase digestion, in PC12 pheochromocytoma cells metabolically labeled with [1-3H]galactose. The polymer was excluded from a Bio-Gel A-0.5 m column and adsorbed by immobilized concanavalin A-Sepharose from which it was eluted with 10 mM alpha-methylmannoside.

Premature opening of the pulmonary valve in endomyocardial fibrosis.

Two-dimensional and Doppler echocardiographic findings in a 67-year-old man with endomyocardial fibrosis (EMF) are described. The two-dimensional echocardiogram showed typical features of EMF, right ventricular endomyocardial calcification, a thickened right ventricular wall, obliteration of the apex of the right ventricle and marked dilatation of the right atrium. In addition, premature opening of the pulmonary valve was observed during late diastole.