PubMed Health⌕ Search

Biomedical subjects

N Ninomiya

Publications and source records attributed to N Ninomiya.

29 records · Page 2Linked to original sources

Seven pyruvate kinase variants characterized by the ICSH recommended methods.

Seven new red-cell pyruvate kinase (PK) variants were characterized by the methods recently recommended by the International Committee for Stanardization in Haematology. The cases were all true homozygote as evidenced by consanguineous marriages of the parents; all are Japanese. These variants were designated as PK Tokyo, PK Nagasaki, PK Sapporo, PK Maebashi, PK Itabashi, PK Fukushima and PK Aizu, respectively. Low substrate affinity (high K0.5S for phosphoenolpyruvate) and thermal instability appear to play major roles in causing defective enzyme function, resulting in chronic haemolytic anaemia. Product inhibition of PK by ATP may also play an additional role in causing haemolysis in more than half the cases.

Adolescent↗

Gaucher-like cells in juvenile GM1-gangliosidosis and in beta-thalassemia -- A histochemical and ultrastructural observation.

Peculiar storage cells appearing in bone marrow aspirates from a patient with juvenile GM1-gangliosidosis and from one with beta-thalassemia were examined light microscopically, histochemically and electron microscopically. Light microscopically, most of the storage cells closely resembled Gaucher cells pathognomonic for Gaucher's disease. The cytoplasm of the Gaucher-like cells contained numerous variable-shaped membrane-bound inclusions mostly arranged in a mosaic pattern and filled with fibrillar materials. Intermingled tubular structures were usually narrow as compared to those of the Gaucher cells. These ultrastructural differences of the stored materials between the Gaucher-like cells and Gaucher cells were more clearly substantiated by the high resolution electron microscopy with negative staining technique. Enzyme cytochemically, acid phosphatase activity was proved in or around the storage inclusions, suggesting their lysosomal origin. Histochemically, it might be suggested that the stored materials of the Gaucher-like cells in juvenile GMI-gangliosidosis were non-sulfated acid mucopolysaccharides and glycopeptides, whereas glycoproteins were the major component of the storage cells in beta-thalassemia. Possible mechanisms of storage in the Gaucher-like cells were discussed in both disorders.

Child↗

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↗

Thrombocytopenic purpura occurring during the early phase of a mumps infection.

Thrombocytopenic purpura with reduced megakaryocyte count in bone marrow was found in a very early phase of a mumps infection in a one year 9 months old boy. No antithrombocyte antibodies were detected. Three weeks later, the mumps antibody titer in the serum was significantly elevated, and thrombocyte and megakaryocyte counts returned to normal.

Antibodies, Viral↗

Demonstration of acid alpha-glucosidase in different types of Pompe disease by use of an immunochemical method.

The nature of mutant acid alpha-glucosidase (AAG) in muscle was studied in 6 patients with Pompe disease, consisting of 2 each of the infantile, childhood and adult types. Anti-human liver AAG rabbit antibody prepared in the present study was confirmed to be monospecific by immunodiffusion, immunotitration and immunohistochemical methods. It was found by the immunodiffusion and enzyme immunoassay methods using this antibody that the mutation produced a normal amount of enzyme protein but the latter was an inactive form, suggesting structural gene mutation in 5 of the 6 cases. In the remaining childhood type case there was no detectable amount of enzyme protein, suggesting that the mutation causes a reduction in the amount of the enzyme protein or synthesis of unstable enzyme protein. Similarly, the enzyme activity of AAG was markedly reduced in all patients, but that of neutral alpha-glucosidase was the least reduced in the adult type, medium in the childhood type, and the most reduced in the infantile type.

Adult↗

Aseptic efficacy of prefilled syringes in a polluted environment.

INTRODUCTION: To evaluate the aseptic efficacy of prefilled syringes compared with ampules when used in a polluted environment similar to that at a disaster site. METHODS: The researchers tested epinephrine, 0.1%, atropine sulfate, 0.05%, and lidocaine hydrochloride solutions, 2% (Group A) as well as lidocaine hydrochloride, 10%, sodium bicarbonate, 8.4%, and glucose solutions, 50% (Group B), that frequently are used for intravenous injection and intravenous infusion respectively in Disaster Medicine. Each of these solutions in 10 prefilled syringes (PFSs) and 10 ampules was placed in a box of contaminated soil along with needles and empty syringes for ampules. In the box, each was taken out of its package, all syringes were connected with a needle, and empty syringes were filled with a solution. After this procedure, all syringes were taken out of the box to check their contents for bacterial contamination. RESULTS: No bacterium was observed in any of the 10 PFS samples of Group A and B solutions. In contrast, out of 10 ampule samples, six of the 10 samples containing epinephrine, nine of the 10 containing atropine sulfate, all 10 samples containing lidocaine hydrochloride, 2%, and all of the ampule samples containing Group B solutions tested positive for bacteria. A statistically significant difference was observed between the PFS and ampule samples in all six solutions. CONCLUSION: Results indicate that, in environments with airborne contaminants, the use of prefilled syringes may be useful for preventing bacterial contamination of the medicine inside.

Air Microbiology↗

Education issues in disaster medicine: summary and action plan.

INTRODUCTION: Change must begin with education. Theme 8 explored issues that need attention in Disaster Medicine education. METHODS: Details of the methods used are provided in the introductory paper. The chairs moderated all presentations and produced a summary that was presented to an assembly of all of the delegates. The chairs then presided over a workshop that resulted in the generation of a set of action plans that then were reported to the collective group of all delegates. RESULTS: Main points developed during the presentations and discussion included: (1) formal education, (2) standardized definitions, (3) integration, (4) evaluation of programs and interventions, (5) international cooperation, (6) identifying the psychosocial consequences of disaster, (7) meaningful research, and (8) hazard, impact, risk and vulnerability analysis. DISCUSSION: Three main components of the action plans were identified as evaluation, research, and education. The action plans recommended that: (1) education on disasters should be formalized, (2) evaluation of education and interventions must be improved, and (3) meaningful research should be promulgated and published for use at multiple levels and that applied research techniques be the subject of future conferences. CONCLUSIONS: The one unanimous conclusion was that we need more and better education on the disaster phenomenon, both in its impacts and in our response to them. Such education must be increasingly evidence-based.

Clinical Competence↗

The dimer and trimer of 3-hydroxybutyrate oligomer as a precursor of ketone bodies for nutritional care.

BACKGROUND: Ketone bodies have been considered as a means of providing energy because of their good penetration and rapid diffusion in peripheral tissues. However, because the currently available form of 3-hydroxybu-tyrate is the sodium salt, the sodium load is problematic. To avoid it, a mixture of dimer and trimer has been prepared as a precursor of D-3-hydroxybutyrate. The purpose of this study was to investigate whether and how the solution would be converted to monomers. METHODS: The plasma concentration of 3-hydroxybutyrate monomer was measured in 10 rats during infusion of dimer and trimer. Stepwise dilutions of the solution were incubated with serum and liver homogenates from five rats, serum samples from five volunteers, and a liver sample from one patient with liver injury. The solution also was incubated with carboxylesterase and triacylglycerol lipase. The concentration of monomer in the medium was measured after incubation. RESULTS: The plasma concentration of 3-hydroxybutyrate monomer reached 572 +/- 11 micromol/L 15 minutes after beginning infusion of the mixture at a rate of 25 micromol x kg(-1) x min(-1) and 270 +/- 40 micromol/L at a rate of 12.5 micromol x kg(-1) min(-1). The solution was converted completely to monomers when incubated with rat serum or liver homogenate for 10 minutes. The mixture also was hydrolyzed by human liver homogenate but not by serum. CONCLUSIONS: The dimer and trimer of 3-hydroxybutyrate can be converted rapidly to monomer in rat and human tissues. 3-Hydroxybutyrate oligomers could be an energy substrate for injured patients.

3-Hydroxybutyric Acid↗