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Biomedical subjects

I Matsuda

Publications and source records attributed to I Matsuda.

At least 415 records · Page 23Linked to original sources
PubMed1983

A fluorescent microscopy study of biopsied muscles from infantile neuromuscular disorders.

The Acridine Orange (AO) stain for muscle biopsies is particularly useful to identify regenerating and ongoing hypertrophic muscle fibers under fluorescent microscopy. This method was applied to muscle biopsies from 65 patients who suffered from various childhood neuromuscular disorders. While normal fibers showed dull green cytoplasm with small green-yellow nuclei, striking fluorescent fibers were observed in eight cases of congenital muscular dystrophy (CMD) and 12 cases of Duchenne muscular dystrophy (DMD); these fibers were characterized as follows: (1) small fibers with big oval or spherical nuclei which fluoresced strongly with a bright orange color; (2) fibers of various sizes and different degrees of orange fluorescence; and (3) opaque fibers with bright yellow cytoplasm. The small diameter fibers in Werdnig-Hoffmann (WH) disease, nemaline myopathy, and congenital fiber type disproportion failed to show apparent AO-RNA fluorescence. Although all the atrophic fibers in Kugelberg-Welander (KW) disease showed a vague orange fluorescent color, this was obviously different from that of regenerating fibers seen in CMD and DMD. In addition to these findings, the hypertrophic fibers in a case of unclassified myopathy also showed moderate orange fluorescence around the entire periphery of the cytoplasm.

Adolescent↗
PubMed1983

Kinetic analysis of argininosuccinate synthetase in a variant form of citrullinaemia.

Argininosuccinate (ASA) synthetase in the liver from a patient with a variant form of citrullinaemia was analysed. Serum citrulline level was approximately 40 times higher than the control level, and the ASA synthetase activity was approximately 10% that of the control. Other urea cycle enzymes were within normal ranges. The specific activity (enzyme activity/enzyme protein, measured by immunochemical method), pH optimum, Michaelis constants, and thermal denaturation in the presence or absence of ASA were similar to those in enzymes obtained from the normal and the patient's liver cells. No in vitro effect of the patient's liver homogenate on ASA synthetase in normal liver was observed. Serum citrulline levels of the patient's parents and young brother were at the maximal level of control at fasting and definitely elevated 4 h after citrulline loading (100 mg/kg).

Adult↗
PubMed1983

Myopathy due to glycogen storage disease: pathological and biochemical studies in relation to glycogenosome formation.

Ten cases of myopathy caused by glycogen storage diseases of type II, III, and V, and phosphorylase b kinase deficiency are reported. So-called "abnormal lysosomes" or glycogenosomes which contain abundant glycogen were found in cases of type II, and in some numbers, in cases of type III, and in one case of phosphorylase b kinase deficiency which revealed a moderate decrease in debranching enzyme (amylo-1,6-glucosidase) activity. In these cases of type III and phosphorylase b kinase deficiency, the glycogenosomes are formed through deposition of abnormal glycogen (limit dextrin structure glycogen).

Adolescent↗
PubMed1983

Ruptured cerebral aneurysms associated with arterial occlusion.

Seven cases of ruptured cerebral aneurysm associated with an occlusion of a large cerebral artery are reported. All seven patients had never suffered from ischemic cerebrovascular disease before the aneurysmal rupture. All nine aneurysms, including the two unruptured ones, arose on the artery serving as an important collateral pathway, and eight of the nine were found in locations where saccular aneurysms are known to occur infrequently. The role of hemodynamic factors in the pathogenesis of these aneurysms is stressed.

Aged↗
PubMed1983

Longitudinal changes in selenium content of breast milk.

The selenium (Se) contents of human milk, serum and hair obtained from 22 lactating mothers were measured by fluorometric analysis. The Se contents in 41 milk samples from different stages of lactation were obtained longitudinally from 10 mothers. They showed a large variance of individual samples at any stage of studies. The highest Se level was found in colostrum (median 80 ng/ml); subsequently, Se content declined significantly during the first month of lactation and then came to a plateau level (median 17-18 ng/ml). No positive correlation of Se content was found between the serum and the milk samples at three months of lactation. No positive correlation of Se content was found between the hair and the milk samples obtained from lactating mothers.

Adult↗
PubMed1983

Glomerular IgA1 and IgA2 deposits in IgA nephropathies.

IgA1 and IgA2 deposits in the renal glomeruli were studied in 29 biopsy specimens from 17 children with IgA nephropathy and 12 children with anaphylactoid purpura nephritis. IgA1 and IgA2 were detected with the indirect immunofluorescence method, using various combinations of dilutions of sheep antihuman IgA subclass antisera and fluorescein isothiocyanate conjugated rabbit antisheep IgG antiserum. The glomeruli of all the 29 specimens studied were strongly positive for IgA1, while only twelve specimens were positive for IgA2. Ten specimens without IgM deposits were all negative for J chain. These results indicate that the glomerular IgA deposits consist mainly of monomeric IgA1, with a minor share for IgA2. The serum IgA in normal individuals consists of about 90% IgA1 and 10% IgA2. Therefore, it was assumed that the serum IgA is deposited irrespective of subclass in the glomeruli of the patients with IgA nephropathies.

Adolescent↗
PubMed1983

Hair copper and zinc concentrations in handicapped children treated with anticonvulsants.

Hair copper and zinc contents were measured in 95 handicapped children aged from 4 to 17 years and 48 age- and sex-matched control children. The patients consisted of 5 groups: children untreated with anticonvulsants (n = 7), those treated with phenytoin and phenobarbital (n = 32), those treated with phenytoin, phenobarbital and diazepam (n = 18), those treated with diazepam alone (n = 16) and those treated with phenobarbital alone (n = 12). The patients were all institutionalized in the same medical care unit and received the same diet, containing decreased amounts of copper (75% of control) and sufficient amounts of zinc. The patients belonging to all of the 5 groups had less amounts of hair copper (p less than 0.05) and erythrocyte hemoglobin (p less than 0.01) in comparison to controls. The patients receiving diazepam alone or in addition to other anticonvulsants had significantly less hair zinc content (p less than 0.05) in comparison to controls or other patient groups. Thus, diazepam seemed to have an adverse effect, producing zinc deficiency.

Adolescent↗
PubMed1983

Chemotactic receptor of cord blood granulocytes to the synthesized chemotactic peptide N-formyl-methionyl-leucyl-phenylalanine.

Chemotactic mobility of cord blood granulocytes (CBG) was studied under varying concentrations of synthesized chemotactic peptide, N-formyl-methionyl-leucyl-phenylalanine (f . Met-Leu-Phe). The maximal chemotactic mobility was found at a concentration of 2 X 10(-7)M in CBG and 1 X 10(-7)M in adult blood granulocytes (ABG) (n = 8). The maximal distance of granulocyte mobility of CBG was significantly shorter than that of ABG (P less than 0.01). The number of chemotactic receptors and the affinity constant were assayed by the competitive inhibition method, using f . Met-Leu-[3H]Phe, and the data were subjected to Scatchard analysis. The number of chemotactic receptors of ABG was 2.5-fold of CBG, as shown by 21,800 +/- 7800 per cell in ABG (n = 3) and 9000 in CBG (n = 1). This figure was confirmed by the one point assay method by increasing the sample numbers of cord blood. It was found that bound chemotactic peptide (X 10(-14) moles/10(7) cells) was 7.9 +/- 0.7 in ABG (n = 4) and 3.4 +/- 0.7 in CBG (n = 5). Affinity constants were similar in CBG and ABG.

Chemotaxis, Leukocyte↗
PubMed1983

Four-hydroxyphenylpyruvic acid oxidase deficiency with normal fumarylacetoacetase: a new variant form of hereditary hypertyrosinemia.

Enzymatic studies on the liver of an infant are described-a case of hypertyrosinemia without hepatic dysfunction. His parents were siblings and the mother had hypertyrosinemia. Excessive amounts of 4-hydroxyphenylpyruvic acid (pHPP), 4-hydroxyphenylacetic acid (pHPL), and 4-hydroxyphenylacetic acid (pHPA) were found to be excreted in the patient's urine as well as in the urine of the mother and the inhibitor of porphobilinogen synthetase was not found. Soluble tyrosine aminotransferase (s-TAT), separated from that of the mitochondrial form (m-TAT) by DE 52 column chromatography, was normal in the patient's liver, both quantitatively and qualitatively. The activities of fumarylacetoacetase in the patient's liver and in the peripheral leucocytes from the parents were normal. The activity of pHPP oxidase in the patient's liver was approximately 5% of the control and the enzyme had a high Km value for pHPP (controls: 0.06 +/- 0.01 mM, patient: 0.23 +/- 0.03 mM). From these results, the patient was thought to be different from previously described types of tyrosinemia and perhaps representative of a new variant form. This is the first report concerning 4-hydroxyphenylpyruvic acid oxidase deficiency alone. Mild metal retardation and mild hypertyrosinemia may be offered as typical clinical features of the disease.

4-Hydroxyphenylpyruvate Dioxygenase↗
PubMed1983

Childhood-type myositis and linear scleroderma.

A 5-year-old girl had linear scleroderma on the flexor surface of the right arm; muscle wasting included the shoulder girdle. IgM fluorescence on blood vessels and along dermal-epidermal junction was observed by direct immunofluorescence in biopsied skin. Biceps muscle underlying the plaque of the scleroderma showed atrophy of entire fascicles, perifascicular atrophy, and cellular infiltration around blood vessels that are quite similar to those found in childhood-type dermatomyositis. In addition, various abnormalities, including edema and thickening of basal lamina, were found on blood vessels in muscle tissue. The results suggested that the autoimmune collagen vascular disorder is responsible for this condition.

Autoimmune Diseases↗
PubMed1983

A case of hemolytic uremic syndrome with high concentration of circulating immune complex in the initial stage.

A case of a 13-year-old boy with hemolytic uremic syndrome is reported, in whom an increased concentration of circulating immune complex and depressed concentration of C3 were found in the initial stage of the disease. Renal biopsy performed at the 52nd day of his illness disclosed subepithelial dense deposits in glomerular tuft, in addition to thrombosis and thickening of intralobular arteries, by electron microscopic study. The observations suggest that an immune mechanism may be operative in some cases of hemolytic uremic syndrome.

Adolescent↗