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Biomedical subjects

I Matsuda

Publications and source records attributed to I Matsuda.

At least 505 records · Page 28Linked to original sources
PubMed1978

Glucose-6-phosphatase activity in liver and blood platelets of two patients with glycogen storage disease type I.

Glucose-6-phosphatase (G-6-Pase) activity in liver and blood platelets of two patients with glycogen storage disease (GSD) type I is described. Both patients had a reduced activity of G-6-Pase in liver. The km value for glucose 6-phosphate (G-6-P) of residual activity in liver of both patients was similar to that of control liver. We could not demonstrate any reduced activity of platelet G-6-Pase in the patients. Platelet G-6-Pase with our assay method seems to represent a nonspecific phosphatase activity. Our observation suggests that it is necessary to examine platelet G-6-Pase of many other patients with GSD type I to confirm that G-6-Pase deficiency can be diagnosed by enzyme assay performed on blood platelets.

Alanine↗
PubMed1978

Methylmalonic acidemia.

A patient presenting with severe metabolic acidosis accompanied by hyperglycinemia, hyperuricemia, hypoglycemia and hypertammonemia is described. Metabolic acidosis was found to be due to accumulated methylmalonic acid and did not respond to vitamin B12 administration. The patient showed favorable growth and development when kept on a low isoleucine, methionine, threonine and valine diet. In vitro studies using a lymphoid cell line derived from the patient showed a deficiency of methylmalonyl-CoA carbonyl-mutase.

Acidosis↗
PubMed1978

Familial occurrence of a syndrome with mental retardation, nasal hypoplasia, peripheral dysostosis, and blue eyes in Japanese siblings.

Two Japanese siblings, a 2-year-old girl and a 7-month-old boy, had a syndrome of mental retardation, severe nasal hyp9plasia, peripheral dysostosis, and blue eyes. The mother showed nasal hyp9plasia of lesser degree and a mild form of peripheral dysostosis. This disorder bears a striking similarity to acrodysostosis, but in view of certain novel features its relationship to the disease is uncertain. The mode of inheritance could be either dominant with variable expressivity or autosomal recessive.

Bone Diseases, Developmental↗
PubMed1978

Extravascular iodine in contrast enhancement with computed tomography.

Using specimens obtained at operation, tissue-blood ratios of iodinated contrast material (iodine), and in some cases also those of red blood cell tracer (51Cr), were measured in 23 patients with various kinds of intracranial mass lesions. Results provided confirmatory evidence on the major role played by extravascular iodine in the positive enhancement effect with computed tomography.

Brain↗
PubMed1978

Mitochondrial fraction of serum glutamic-oxaloacetic transaminase in Duchenne muscular dystrophy.

The mitochondrial fraction of serum glutamic-oxaloacetic transaminase was measured in the serum of 50 patients with Duchenne muscular dystrophy by an immunoadsorbent method. The enzyme activities in patients in the early, midstage, and late stages of the disease and controls were 21.8 +/- 7.4 (N=9), 12.2 +/- 3.7 (N=38), 6.4 +/- 1.2 (N=3) and 4.2 +/1 1.2 units/ml (N=15), respectively. The enzyme level in the early stage was significantly elevated (p less than 0.01, vs. control, p less than 0.05 vs. mid stage). As the disease progressed, the levels gradually declined, but mid-stage values were still higher than the late stage (p less than 0.01) or control values (p less than 0.01). In the late stage, enzyme activity was within the control range.

Adolescent↗
PubMed1978

The role of hemodynamic factors in arterial wall thickening in the rat.

In a study on the pathogenesis of arterial wall thickening, hemodynamic factors in the common carotid artery of the rat were experimentally altered with an autograft. The relationship between flow pattern and wall-thickening was examined in a half-ring bypass model with an induced stenosis, using both flow-visualization in a corresponding in vitro model circuit and observation of the wall by microscopy. Wall-thickening was found in the neighborhood of bifurcations, junctions and curved segments, which corresponded to regions of low-shear in the flow-field. Marked histologic changes in the wall were observed in the post-stenotic segments where the flow field was very disturbed. Histologic changes in the arterial wall correlated well with flow patterns.

Animals↗
PubMed1978

Urinary oligosaccharides of fucosidosis. Evidence of the occurrence of X-antigenic determinant in serum-type sugar chains of glycoproteins.

Urine of a fucosidosis patient contained a large amount of fucosyl oligosaccharides and fucose-rich glycopeptides. Six major oligosaccharides were purified by a combination of Bio-Gel P-2 and P-4 column chromatographies and paper chromatography. Structural studies by sequential exoglycosidase digestion and by methylation analysis revealed that their structures were as follows: Fucalpha1 leads to 6GlcNAc, Fucalpha1 leads to 2Galbeta1 leads to 4(Fucalpha1 leads to 3)GlcNAcbeta1 leads to 2Manalpha1 leads to 3Manbeta1 leads to 4GlcNAc, Galbeta1 leads to 4(Fucalpha1 leads to 3)GlcNAcbeta1 leads to 4Manalpha1 leads to 4GlcNAc, Galbeta1 leads to 4(Fucalpha1 leads to3)GlcNAcbeta1 leads to 2Manalpha1 leads to 6Manbeta1 leads to 4GlcNAc, and Galbeta1 leads to 4(Fucalpha1 leads to 3)GlcNAcbeta1 leads to 4Manalpha1 leads to 6Manalpha1 leads to 6Manbeta1 leads to 4GlcNAc. In additon, the structure of a minor decasaccharide was found to be Galbeta1 leads to (Fucalpha1 leads to)GlcNAcbeta1 leads to Manalpha1 leads to [Galbeta1 leads to (Fucalpha1 leads to)GlcNAcbeta1 leads to Manalpha1 leads to]Manbeta1 leads to 4GlcNAc.

Carbohydrate Metabolism, Inborn Errors↗
PubMed1978

Dosage schedule of gentamicin for chronic renal insufficiency in children.

Gentamicin was given to paediatric patients with chronic renal disease complicated by infections by Gram-negative organisms, in which renal function varied from normal to severely insufficient. Peak serum levels after an intramuscular dose of 1 mg/kg body weight ranged from 3.1 to 9.4 microgram/ml, which appeared adequate for therapy. The peak value was not related to the renal function of the individual patients. The serum half-life of gentamicin correlated inversely with the value for endogenous creatinine clearance. A diagram for the estimation of the serum half-life of gentamicin using the creatinine clearance value is presented. As a practical guide, it is recommended that the dose of gentamicin in children with renal function impairment be 1 mg/kg given intramuscularly and that the interval between doses be almost three times as long as the serum half-life, which can be estimated by means of the diagram for individual patients. The accuracy and safety of this method were confirmed by treating children with this adjusted dosage schedule.

Adolescent↗
PubMed1978

Inactive form of erythrocyte carbonic anhydrase B in patients with primary renal tubular acidosis.

Evidence was found for an inactive form of carbonic anhydrase type B in the erythrocytes of two children with primary renal tubular acidosis. The addition of zinc chloride to hemolysates from these patients resulted in a marked increase in the activity of this enzyme. No such effect was noted with hemolysates of control subjects. No significant differences were observed in the zinc levels of hemolysates of these patients and of normal individuals. However, the level of zinc in the carbonic anhydrase B isolated from one of these patients was low, suggesting a modified form of the enzyme. The restoration of activity upon the addition of zinc was reversed by ethylenediamine tetraacetate, but no such effects were noted for the carbonic anhydrase B of normal individuals. Thus the abnormal carbonic anhydrase B has decreased zinc binding. The ultraviolet difference spectrum of the carbonic anhydrase B of normal individuals and that of a patient showed a peak at 305 nm which decreased upon the addition of zinc. The abnormal form of carbonic anhydrase B was not distinguishable from that of normal individuals by either immunological or electrophoretic criteria.

Acidosis, Renal Tubular↗
PubMed1977

Lysosomal enzyme activities in cultured lymphoid cell lines.

Several lysosomal enzyme activities in cultured lymphoid cell lines were studied during 3 phases of cell culture; logarithmic growth phase, stationary phase and decline phase. Enzyme induction during cell growth was found in N-acetyl-hexosaminidase, beta-galactosidase and alpha-L-fucosidase, but no induction in alpha-D-mannosidase, alpha-glucosidase and beta-glucuronidase. The latter two enzymes were unchanged during all cell culture phases. A drop in alpha-L-fucosidase and alpha-D-mannosidase activity was found during the stationary and decline phases of cell culture.

Cell Division↗
PubMed1977

The effects of oral alanine administration on blood glucose, pyruvate, lactate, serum glucagon and insulin in human newborns.

Alanine (500 mg/kg body weight) was given orally to 27 healthy full term newborn infants, and the changes in blood glucose, pyruvate, lactate, alanine, glucagon and insulin were determined. Significant increments in blood glucose were found in 15 infants with blood glucose levels below 60 mg/100 ml, 4 of whom showed significant elevation of serum glucagon levels on day 1. This observation suggests that hepatic gluconeogenesis is possible immediately after birth.

Age Factors↗