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Biomedical subjects

K Tani

Publications and source records attributed to K Tani.

At least 397 records · Page 22Linked to original sources
PubMed1981

Two cases of red cell aldolase deficiency associated with hereditary hemolytic anemia in a Japanese family.

Two cases of red cell aldolase deficiency associated with congenital nonspherocytic hemolytic anemia are reported. The proband is a fourteen-month-old Japanese boy. Consanguineous marriage was not proven but probable in this family, as the parents were born in the same small island. The proband had moderate to mild anemia aggravated by upper respiratory infections, 1 cm hepatomegaly and 2.5 cm splenomegaly, but was unremarkable in other respects and has thus far not shown mental or growth retardation. He did not have dysmorphic features. The red cell aldolase activity was 6% of the normal mean. The enzyme was unstable with respect to heat, and Km for fructose 1,6-diphosphate (F-1,6-DP) was high. The parents and other heterozygotes showed intermediate activity between that of the proband and that of normal subjects. Red cell F-1,6-DP concentration in this case was remarkably increased. Red cell glucose consumption, and lactate formation, as well as hexose monophosphate shunt activity, were decreased as compared with a comparable reticulocyte-rich hereditary spherocytosis patient. Hexose monophosphate dehydrogenase by a high concentration of F-1,6-DP in his red cells. As a result of family study, another homozygous aldolase deficiency case associated with hemolytic anemia was found. He is 13 years old and a nephew of the proband's paternal grandmother. His hemolytic anemia also is moderate to mild and aggravated by upper respiratory infections. He does not seem to have mental or growth retardation, nor does he possess dysmorphic features.

Adolescent↗
PubMed1981

Glucose 6-phosphate dehydrogenase variants: a unique variant (G6PD Kobe) showed an extremely increased affinity for galactose 6-phosphate and a new variant (G6PD Sapporo) resembling G6PD Pea Ridge.

Two new glucose 6-phosphate dehydrogenase (G6PD) variants associated with chronic nonspherocytic hemolytic anemia were discovered, G6PD Kobe was found in a 16-year-old male associated with hemolytic crisis after upper respiratory infection. The enzyme activity of the variant was about 22% of that of the normal enzyme. The main enzymatic characteristics were slower than normal anodal electrophoretic mobility, high Km G6P, increased thermal-instability, an acidic pH optimum, and an extremely increased affinity for the substrate analogue, galactose 6-phosphate (Gal-6P). G6PD Sapporo was found in a 3-year-old male associated with drug-induced hemolysis. The enzyme activity was extremely low, being 3.6% of normal. In addition, this variant showed high Ki NADPH and thermal-instability. G6PD Kobe utilized the artificial substrate Gal-6P effectively as compared with the common natural substrate, glucose 6-phosphate. In G6PD Sapporo, NADPH could not exert the effect of product inhibition. The structural changes of these variants are expected to occur at the portions inducing conformational changes of the substrate binding site of the enzyme.

Adolescent↗
PubMed1981

[Effects of vasopressin analogue DDAVP in patients with postoperative diabetes insipidus (author's transl)].

The effect of DDAVP (1-deamino-8-D-arginine vasopressin), a synthetic analogue of vasopressin, was studied in twelve patients with acute postoperative cranial diabetes insipidus (D.I.). The most severe D.I. occurred in four cases following total removal of tumor (3 pituitary microadenoma, 1 dermoid cyst). The urinary volume over 1000 ml per hour in these four cases could not be controlled by DDAVP but could be controlled by drip infusion of aqueous pitressin (AP) and pitressin tannate in oil (PTO). DDAVP was effective when the urinary volume was decreased in under 500 ml per hour. The mild D.I. occurred in four cases after partial removal of tumor (3 craniopharyngioma, 1 pituitary microadenoma). These four cases could be controlled by drinking water only during one or two postoperative weeks. DDAVP was administered in doses of 10 to 30 microgram two times daily after 2 or 3 postoperative weeks and the urinary production was normalized. The four patients developed D.I. after removal of functioning pituitary microadenoma operated by transsphenoidal route. These four cases were treated with drip infusion of AP and PTO during one or two weeks after the operation and were effectively treated with 5 to 15 microgram of DDAVP intranasally every 8 to 12 hours one or two weeks after the operation. Nine cases in 12 cases with postoperative D.I. became chronic D.I. The maintenance dose of DDAVP gradually lessened in accordance with decreasing urinary volume except the two cases of craniopharyngioma. No side effect was experienced for 19 months of treatment.

Adenoma↗
PubMed1980

Genetic control of contact hypersensitivity. I. I-A subregion as well as non-H-2 loci codes for the gene of 2,4-dinitro-1-fluorobenzene antigen.

As a first step in determining the genetic control of contact hypersensitivity in mice, 2,4-dinitro-1-fluorobenzene (DNFB) was applied to induce ear swellings. Studies with congenic and recombinant inbred strains of mice revealed that the DNFB contact hypersensitivity is controlled by the I-A subregion, as well as non-H-2 regions. The results presented suggest that the major histocompatibility complex-linked immune response genes seem to be playing an important role in the contact hypersensitivity to the DNFB antigen.

Animals↗
PubMed1980

Antigen-pulsed neutrophils bearing Ia antigens can induce T lymphocyte proliferative response to the syngeneic or semisyngeneic antigen-primed T lymphocytes.

Antigen-pulsed neutrophils from mouse peritoneal cavities displayed a remarkable level of lymphocyte proliferative activities to antigen-primed T lymphocytes. Genetic mapping studies demonstrated that compatibility at the I-A, as well as I-E/C, subregions of the major histocompatibility complex (MHC) was essential for effective presentation of the lysozyme antigen. These antigen-presenting activities were remarkably inhibited by anti-Ia sera. Inhibition tests revealed that neutrophil immune-associated (Ia) antigens seem to be essential for antigen presentation during the initial 8 hr. Elimination studies of antigen-pulsed neutrophils with alloantisera plus complement revealed these antigen-presenting neutrophils bearing both I-A and I-E/C gene products on the same cells. These results suggest that Ia-positive neutrophils might play a role in the immune response through antigen presentation.

Animals↗
PubMed1980

Excretion of glutathione by methylglyoxal-resistant Escherichia coli.

A methylglyoxal-resistant mutant of Escherichia coli B excreted glutathione into the growth medium, especially during growth on medium containing methylglyoxal. In the presence of methylglyoxal, the total amount of glutathione excreted was increased about 50-fold over that of the wild-type strain. The resistant mutant had high activities of two enzyme systems: a glutathione-forming enzyme system (consisting of gamma-glutamylcysteine synthetase and glutathione synthetase) and a glyoxalase system (consisting of glyoxalase I and glyoxalase II). Methylglyoxal resistance appeared to be due to the simultaneous increase in the activities of these two enzyme systems.

Drug Resistance, Microbial↗