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Biomedical subjects

I Matsuda

Publications and source records attributed to I Matsuda.

At least 451 records · Page 25Linked to original sources

Glycogen storage myopathy with abnormal lactate dehydrogenase.

We studied a 3 year old girl with mental retardation and limb muscle weakness. The muscle glycogen content was 17.4 mg/g tissue, which was approximately three times higher than normal. There were no other known abnormalities noted in this child that could explain the cause of glycogen storage disease. Our in vitro glycolysis study showed marked increase of pyruvate, but no increase in lactate levels. The observed results suggested to us that an abnormal lactate dehydrogenase might account for the abnormal accumulation of glycogen in the muscle.

Child, Preschool↗

Circulating immune complex in glomerulonephropathy associated with hepatitis B virus infection.

Urinary abnormalities combined with positive serum hepatitis B virus surface antigen (HBsAg) were found in 9 children. Most of these patients were found by mass urine screening of school children. Renal biopsy findings revealed 6 cases of membranous glomerulonephropathy (MGN) and 3 cases of minimal change disease. Immune deposits consisting of hepatitis B virus e antigen (HBeAg), IgG and C3 were detected in the glomerular capillary walls by immunofluorescent study in patients with MGN. Circulating immune complex was positive at a rate of 67% (6/9 cases) by the C1q-binding assay and at a rate of 56% (5/9 cases) by the protein-A precipitation test in patients with persistent urinary abnormalities and hepatic dysfunction.

Antigen-Antibody Complex↗

Human erythrocyte prolidase and prolidase deficiency.

Biochemical studies on human prolidase (EC 3.4.13.9) and prolidase deficiency are described. The urine sample from a 32-year-old female with prolidase deficiency was examined. Diagnosis was based on clinical features and defects of prolidase in her erythrocytes. She excreted massive amounts of iminopeptides, where three major peptides were identified; aspartyl-proline, glutamyl-proline and glycyl-proline. The prolidase was purified approximately 10,000-fold from the normal human erythrocytes through an eight step procedure. The purified enzyme consisted of two identical subunits of which the molecular weight was calculated to be 55,000. The relative cleavage rates of the enzyme for glycyl-L-proline, L-alanyl-L-proline, L-leucyl-L-proline, L-prolyl-L-proline, and glycyl-hydroxy-L-proline were 100%, 53%, 27%, 31% and 2%, respectively. The relative substrate specificity of the enzyme offers a reasonable explantation for the presence of a higher level of urinary imidodipeptides in a patient with prolidase deficiency. An attempt at erythrocyte transfusion was performed, aimed at enzyme replacement therapy. After the transfusion (erythrocytes from 800 ml of whole blood), the prolidase activity of the peripheral erythrocyte was elevated to approximately 35% of the normal values and gradually decreased (half-life, 41 days). During this period urinary peptide-bound proline was monitored, but no significant change was observed.

Adult↗

Infantile glycogen storage myopathy in a girl with phosphorylase kinase deficiency.

A 19-month-old girl with moderate hypotonia was studied. Histochemical and electronmicroscopic findings revealed that many skeletal muscle fibers contained an excess amount of glycogen. The phosphorylase reaction was normalized only after activation with 5' AMP. Biochemical studies showed an increased glycogen content and decreased activities of phosphorylase "a" and an active form of phosphorylase kinase, whereas activities of total phosphorylase, total phosphorylase kinase, and cyclic AMP-dependent protein kinase were all in the normal range. Thus, phosphorylase kinase in the patient's muscle seemed to be a variant form, which was activated partially under the physiologic condition. This condition may be inherited as an X-linked recessive trait.

Acid Phosphatase↗

Zinc and copper contents in breast milk of Japanese women.

Longitudinal studies of zinc and copper contents of breast milk were performed in 65 Japanese mothers during their lactation period until 5 months postpartum. They experienced no problems during pregnancy and at delivery. All women had full term healthy infants. Forty-five were multiparous and 20 primiparous. The highest level of zinc content was found in the colostrum, and subsequently the levels declined (p less than 0.005), as lactation progressed. Copper content was stable during the first month of lactation, and then declined gradually (p less than 0.005). Both mineral levels in breast milk ranged with great variance among the subjects at any stages of lactation. Significantly lower zinc level (p less than 0.01) and higher copper level (p less than 0.005) were found in the serum of lactating women three months postpartum, when compared with non-lactating control women. Factors including lactation history, age of lactating women, serum levels and contents of zinc and copper in the hair did not affect the contents of these trace minerals in the breast milk.

Colostrum↗

Serum zinc and vitamin E concentrations in handicapped children treated with anticonvulsants.

Serum zinc and vitamin E concentrations were measured in handicapped children aged 4-17 years, treated (n = 32) and untreated (n = 13) with phenytoin and phenobarbital. The mean serum zinc levels were 81 +/- 16 and 82 +/- 7 micrograms/dl in the treated and untreated children, respectively. The difference was not significant between these two groups. Hypozincnemia (below 70 micrograms/dl), however, was found in 7 of the 32 treated patients and in none of the 13 untreated children. The serum vitamin E level was 0.58 +/- 0.18 mg/dl in the treated and 0.67 +/- 0.17 mg/dl in the untreated children, indicating significantly lower levels in the treated patients (p less than 0.005). A significant positive correlation was found between the serum zinc and vitamin E levels (r = 0.45, p less than 0.005). Thus, long-term use of anticonvulsants results in a vitamin E and zinc deficiency, which may produce several unfavorable problems in children.

Adolescent↗

Histopathological definition of the adult and calf types of bovine leukosis.

Histopathological distinctions were examined on 36 cattle 2 months to 12 years of age affected with leukosis. The distribution of tumors pointed out previously as the characteristic of the adult type of leukosis was observed in 27 adult cases, which, except two, were positive for the immunodiffusion test of bovine leukemia virus (BLV). The noticeable development of massive and lumpy tumors in serosa, loose connective tissue, and lymph nodes seemed to suggest a neoplastic accumulation in areas related to the lymphatic way. Splenic involvement and intrasinusoidal leukotic immigration in the liver were present in cases with the leukemic manifestation of peripheral blood. The neoplastic involvement of bone marrow, however, was difficult to find. On the other hand, in 5 calves and 4 adolescent or adult cattle, the neoplastic proliferation was observed throughout such hematopoietic organs as lymph nodes, bone marrow, liver, spleen, thymus and tonsil. The hepatic lesion was characterized by the neoplastic proliferation in the portal triads No BLV antibody could be detected from these cattle, except one adult. As the conclusion of the present study, the tumor of adult type regarded as the pathologic manifestation of enzootic bovine leukosis is defined as malignant lymphoma (non-Hodgkin's lymphoma or lymphosarcoma) of undifferentiated-, histiocytic-, or poorly- differentiated lymphocytic- cell type. And lymphocytic leukemia seems to be desirable term to distinguish the lymphoid malignancy regarded as the characteristic of the calf type from that of the adult type.

Age Factors↗

Trisomy 17p due to A t(5;17) (p15;p11) pat translocation.

A 6-month-old Japanese boy with trisomy 17p, resulting from a paternal balanced translocation t(5;17)(p15;p11), is described. Comparison of his clinical features with those of two previously reported patients with trisomy 17p revealed a characteristic combination of clinical features. They included intra- and extra-uterine growth retardation, developmental retardation, microcephaly, internal hydrocephalus, a facies with lateral displacement of the inner canthi, antimongoloid slanting of the palpebral fissures, a broad nasal bridge, and micrognathism, low-set, large and simple ears, a short and webbed neck, and flexion deformities of the fingers. The external genitalia in the two male patients were characterized by a small penis, undescended testes and a hypoplastic scrotum. Congenital cardiac defect was present in two of the three patients.

Abnormalities, Multiple↗

Lipid and apolipoprotein levels in patients with nephrotic syndrome.

Plasma lipids and 6 plasma apolipoproteins (apoA-I, apoA-II, apoB, apoC-I, apoC-II and apoC-III) were studied in 23 patients with nephrotic syndrome. The elevated total cholesterol, triglyceride and apoB levels in nephrotic patients decreased gradually, after the disappearance of proteinuria. During the acute stage high density lipoprotein cholesterol, as well as the sum of apoA-I and apoA-II were similar in the patients and the controls. ApoA-I and apoA-II were transiently elevated during the recovery stage. All three apoC proteins (apoC-I, apoC-II and apoC-III) were elevated during the acute stage. A significant decrease in apoC-I, apoC-II and apoC-III was observed during the first 3 weeks after normalization of the urine protein. The ratio of apoC-II/apoC-III was reduced during the first 4 weeks after normalization of urine protein and then returned to the control level. The results suggest that as far as total levels are concerned, changes of apoC-II, apoC-III and the ratio of apoC-II/apoC-III appear to have no effect on the development of hyperlipidemia in th nephrotic syndrome.

Adolescent↗