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Biomedical subjects

M Yazaki

Publications and source records attributed to M Yazaki.

At least 19 recordsLinked to original sources
PubMed1991

[Bone marrow transplantation for patients with Philadelphia chromosome-positive acute lymphoblastic leukemia].

We reported the results of 6 allogenic bone marrow transplantation (BMT) and 3 autologous BMT for patients with Philadelphia chromosome (Ph1)-positive acute lymphoblastic leukemia (ALL) by Nagoya BMT group. Two of six patients who received allogenic BMT have continued complete remission (CR) on +639 days and +1,597 days. Four of six patients relapsed on +134, +203, +216, and +267 days. Two patients with a disease-free survival for a long time had both acute and chronic GVHD. It is suggested that graft-versus-leukemia (GVL) effect might prevent the relapse. On the contrary, one patient who received with monoclonal antibodies plus complement-treated autologous bone marrow is free of leukemia on +439 days. Our results suggest the follows. 1) We do chromosomal analysis at initial diagnosis in all cases to do BMT in first CR. 2) We intensify the conditioning regimen for BMT. 3) We study on application of GVL effect to prevent the relapse. 4) We establish the best purging method to eradicate residual leukemic cells for autologous BMT. 5) We do allogenic BMT using HLA-matched unrelated donor for patients without related donor.

Adolescent↗
PubMed1990

Exclusion mapping of the hereditary dentatorubropallidoluysian atrophy gene from the Huntington's disease locus.

Hereditary dentatorubropallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder. Clinical and genetic findings in hereditary DRPLA are very similar to those of Huntington's disease (HD). However, it can be differentiated from HD by the pathological findings of dentatorubral and pallidoluysian atrophies and by a lack of prominent atrophy of the striatum at necropsy. The hereditary DRPLA gene has not been localised and the possibility that the two disease loci are allelic has been suggested. We have searched for linkage between the locus for hereditary DRPLA and D4S10 using the G8 probe, which is a genetic marker linked to HD. In four families, there were negative scores at all recombination fractions and the lod score was -2.215 at recombination fraction theta = 0.15. These data indicate that the locus for hereditary DRPLA is not closely linked to D4S10 and that hereditary DRPLA is a distinct disease from HD.

Blotting, Southern↗
PubMed1990

[Relationship between the total IgE levels and specific IgE antibodies].

In this study, nine allergens were selected; i.e. 1289 cases of d1, 1277 cases of e1, 1547 cases of f1, 1063 cases of t17 and others, for investigating the relationship between the total IgE level and RAST score. The results showed that the total IgE level was high in high d1 score, and 29 cases of low IgE level were observed in high f1 score groups. On the other hand, the comparison of the CAP system and the total IgE level showed. The result shows that one case of low total IgE value was found in highest d1 score group.

Allergens↗
PubMed1990

[A comparison of the several methods for determination of specific IgE antibodies].

The accuracy of measurement using kits in the clinical laboratories is important for the patient diagnosis and treatment. In the present paper, the AL-18, AlaSTAT, CAP, FAST and RAST methods were investigated and were compared among kits the results obtained with serum sample, for determination of specific IgE antibodies. Significant differences among kits were observed from the results of those methods. One of the reasons, why the data discrepancy exists, is that each kit uses a different reference and a different inclusion method of allergen. For the evaluation of data discrepancy among those kits, it might be important that the clinical history of symptoms and in vivo tests against the different allergens compared with results of in vivo tests.

Antibody Specificity↗
PubMed1989

[Effect of breathing fluctuations on cerebral blood flow in demented patients and its correction method using end-tidal CO2 concentration].

During mouthpiece respiration of Xe-133 for a measurement of regional cerebral blood flow (rCBF), the breathing pattern of patients fluctuated and it caused a change of end-tidal CO2 concentration that had an excellent correlation with PaCO2 in patient without respiratory disease. The end-tidal CO2 concentration of demented patients varied within lower ranges than senile control group. The range of fluctuation on the end-tidal CO2 concentration was dependent on the type and the degree of dementia, and it fluctuated most widely at the middle stage of Alzheimer disease. Mean cerebral blood flow increased by 13.9% for each 1% increase in end-tidal CO2 concentration (3.6%/mmHg PaCO2) in the case of demented patients without cerebrovascular disease. To improve the reliability of rCBF in demented patients, especially in Alzheimer disease, the correction of rCBF data for end-tidal CO2 concentration should be performed.

Adult↗
PubMed1987

A case of adult neuronal ceroid-lipofuscinosis with the appearance of membranous cytoplasmic bodies localized in the spinal anterior horn.

An autopsy case of adult neuronal ceroid-lipofuscinosis was examined. The clinical picture was characterized by gait disturbance, bulbar palsy and dementia. Histopathologically, diffuse neuronal loss was found throughout the central nervous system. The remaining neurons, predominantly in the motor nuclei of the spinal cord and brain stem, were swollen with storage material. Observed under the electron microscope the storage material showed various ultrastructures, such as lipofuscin-like bodies, pleomorphic lipid bodies, curvilinear profiles and finger-print profiles, in different regions of the central nervous system. In the ballooned neurons of the spinal anterior horn, many membranous cytoplasmic bodies and curvilinear profiles were intermingled within the same cell and were continuous with each other. Biochemically, N-acetyl neuraminic acid content was significantly increased in the spinal anterior horn. These findings suggest the localized increase of ganglioside in that region.

Gangliosides↗
PubMed1987

Increase of protein synthesis by uridine supplement in lectin-stimulated peripheral blood lymphocytes and EB virus-transformed B cell line of hereditary orotic aciduria type I.

A 2 month-old Japanese girl with hereditary orotic aciduria type I was treated with oral uridine supplement. The activities of orotate phosphoribosyltransferase (OPRT) and orotidine-5'-phosphate decarboxylase (ODC) in erythrocytes were 2.7 and 0.4%, respectively, of those in the controls. Megaloblastic anemia, excessive urinary excretion of orotic acid, lymphopenia and decreased number of OKT3 positive lymphocytes on admission were corrected after the uridine supplement. Peripheral blood lymphocytes (PBL) were cultured for 24 hr in RPMI 1640 medium with 10% heat-inactivated fetal calf serum and further stimulated with PHA-P, ConA or PWM in the presence of 10 to 1000 microM uridine. EB virus-transformed B cell line (LCL) maintained with an optimal concentration of uridine was cultured for 48 hr in uridine free medium and cultured for an additional 48 hr with 1 to 1000 microM uridine. The incorporations of leucine in to PHA-, ConA- and PWM-stimulated PBL and into LCL of the patient increased in the presence of uridine over 10 microM, although they did not increase in controls. These data suggest that low protein synthesis might correlate with an immune deficiency in hereditary orotic aciduria type I.

Antigens, Surface↗
PubMed1986

An autopsy case of adult neuronal ceroid lipofuscinosis.

This is a report of an autopsy case (45-year-old woman) of adult neuronal ceroid lipofuscinosis with a long-lasting course of 25 years. In the course of her illness, gait disturbance, bulbar palsy and dementia were observed. Pathologically severe neuronal loss and hemosiderin-like granules were found in the cortex, basal nuclei, thalamus and others. The residual cells of these tissues were swollen with lipofuscin-like pigments. The extremely swollen nerve cells were found in the anterior horn of the spinal cord. Electron microscopically, numerous curvilinear bodies, myelin figures and fingerprint profiles were seen in those cytoplasms. Ceroid and lipofuscin were proven by a histochemical examination.

Brain↗
PubMed1985

Menkes' kinky hair disease: clinical and experimental study.

A pedigree of Menkes' kinky hair disease (MKHD) is reported. One patient of this family who underwent copper treatment was followed for three years with fundus examinations and ERG measurement. The blood copper level remained normal after six months of age, when intravenous treatment was switched from cupric acetate to cupric sulfate. Optic nerve atrophy and decrease in amplitude of the ERG were observed at three years of age. In an experiment using mouse models of MKHD (macular mouse mutant, Moml), we compared the affected mice that received copper treatment with normal mice. However, there was no difference between them in ERG responses, number of ganglion cells, or thickness of retinal layers. These results support the possibility of prolonged survival and maintained vision in the patients of MKHD by earlier normalization of the copper level.

Animals↗
PubMed1985

Clinicopathologic studies on neuro-Behçet's disease.

Nine cases of neuro-Behcet's disease were investigated clinicopathologically. Pathological pictures of the central nervous system were characterized as follows: the site of predilection was the brain stem, followed by the spinal cord, cerebrum and cerebellum. The pathognomonic changes were recurrent inflammations around small vessels, causing a softening of the tissue. Lesions were composed of a perivascular infiltration of lymphocytes, histiocytes and microglias and, moreover, diapedesis, degenerated nerve cells and oligodendroglias, glial nodule, breakdown of myelin and axon, fatty granule cells and glio-mesenchymal proliferation were present occasionally. Electron microscopic studies on the neurons revealed no evidence of viral particles except for some accumulations of electron dense bodies.

Adult↗
PubMed1976

Gmi-gangliosidosis. A variant with high activity of hepatic neutral beta-galactosidase.

A case of GM1-gangliosidosis with high activity of hepatic neutral beta-galactosidase is reported. GM1-beta-galactosidase was deficient. Ganglioside GM1 was accumulated in the liver of this patient. Clinically this Japanese girl started convulsive seizures at 5 months of age, had hepatomegaly, and macular cherry-red spots, but lacked gargoylelike clinical characteristics. Correlation of clinical and biochemical data is discussed.

Chromatography, Thin Layer↗