Search PubMed⌕ Search

Biomedical subjects

T Akabane

Publications and source records attributed to T Akabane.

At least 109 records · Page 6Linked to original sources
PubMed1981

Morphologic and functional heterogeneity of chronic neutropenia of childhood with normal neutrophil colony formation in vitro.

In order to obtain further knowledge of chronic neutropenia of childhood, we studied nine neutropenic infants six to ten months of age by in vitro techniques, including bone marrow culture, electron microscopy, and chemotaxis assay. Eight of the nine patients had a benign clinical course and the bone marrow aspirates showed a reduced number of segmented neutrophils. The ninth patient had a moderately severe course and the bone marrow showed maturation arrest at the promyelocyte stage. Bone marrow cultures demonstrated that the in vitro neutrophil colony formation and production of colony-stimulating activity were normal in all of the eight patients studied. Neutrophils from one of the nine patients had ultrastructural abnormalities such as decrease in number of primary and secondary granules and the presence of myelin figures in primary granules. Neutrophil chemotaxis was defective in three of the nine patients. All of the six patients in whom the neutrophil colony formation in agar, the ultrastructure of neutrophils, and neutrophil chemotaxis were normal recovered from the neutropenia between 11 and 30 months of age. These in vitro parameters appear to be useful for evaluating chronic neutropenia of childhood.

Agranulocytosis↗
PubMed1981

Demonstration of T lymphocytotoxic human fetal antibody against maternal T and concanavalin A-inducible adult T cells in cord IgM.

To clarify reasons of the constant presence of IgM in human cord blood, some functions of this immunoglobulin class were analyzed. Cord IgM was collected from cord blood, and the cord IgM F(ab')2 fragment was obtained by trypsin cleavage. Maternal T and adult T cells were stained with fluorescein isothiocyanate (FITC)-conjugated cord IgM and FITC-cord F(ab')2 mu. Cord IgM, unlike cord IgG, bound to about one-third of maternal and one-fourth of adult T cells and killed them, but it killed only a small proportion of the B cells. However, adult F(ab)'2 mu did not kill these cells. Cord T cells were not killed by cord IgM. Thus, it is apparent that cord IgM contains cytotoxic antibodies against maternal and adult T cells. T lymphocytotoxic fetal antibody (TLFA) may be largely responsible for the absence of maternal T cells in cord blood. The percentage of [3H]thymidine incorporation into concanavalin A-induced adult mononuclear cells treated with cord IgM containing TLFA was 56.0 +/- 17.9% (p less than 0.01) and that into pokeweed mitogen-induced cells treated with the cord IgM preparation was 100.3 +/- 17.8% (p greater than 0.05) indicating the possibility that TLFA might kill a certain subset(s) of T cells.

Antilymphocyte Serum↗
PubMed1981

A new approach to detection of heterozygotes for adenosine deaminase deficiency: a hypothetical method.

In a second and third families with ADA deficiency found in Japan, we tried a new approach to evaluate heterozygote detection. This is based on the hypothesis that ADA activity of red blood cell is the quantitative sum of the activities of ADA proteins expressed by two allelic genes at the ADA autosomal locus, and that these activities are not changed by the gene transmission from parents to children. We have detected red blood cell-ADA activities expressed by the one normal allelic gene in heterozygotes (including parents and paternal or maternal grandfather or grandmother) and from these values have determined combinations for the pair of ADA activities expressed by the two allelic genes of other family members. These combinations were consistently made in all relatives examined in the two families, and we conclude that several members of each family who were judged to have nil activity in the combinations were heterozygotes for ADA deficiency.

Adenosine Deaminase↗
PubMed1981

Development of the accelerated phase during ascorbic acid therapy in Chediak-Higashi syndrome and efficacy of colchicine on its management.

Clinical studies were done on a patient with Chediak-Higashi syndrome (CHS) with special emphasis on the accelerated phase. In order to obtain further information on the accelerated phase, haematopoiesis was studied by bone marrow culture techniques. The patient was placed on ascorbic acid therapy but she entered the accelerated phase, although the therapy improved in vitro neutrophil function to some extent. Administration of microtubulytic drugs such as vincristine, vinblastine and colchicine was effective in the management of the accelerated phase. Numbers of macrophage-granulocytic (CFU-C) and erythroid (CFU-E) progenitor cells were markedly decreased or absent during the accelerated phase, being another indicator of the accelerated phase.

Ascorbic Acid↗
PubMed1980

Transient 'lazy-leukocyte' syndrome during infancy.

A male infant with transient severe neutropenia and defective neutrophilic mobility is described. He had recurrent upper respiratory infections that responded well to antibiotics. The neutrophilic counts continued to be less than 300/cu mm, and neutrophilic random mobility and chemotaxis were persistently defective during early infancy but were normal at 16 months of age. No ultrastructural abnormality was found in the neutrophils. Phagocytic and bactericidal abilities of the cells were not impaired. Bone marrow was normal. The clinical and laboratory findings suggest that this disorder is transient "lazy-leukocyte" syndrome.

Agranulocytosis↗
PubMed1980

Intrinsic neutrophil defects in a child with impaired neutrophil chemotaxis and immunodeficiency.

A boy with recurrent pyogenic infection was found to have occasional neutropenia, defective neutrophil chemotaxis, hypogammaglobulinemia with increased IgM, and impaired cellular immunity. The T and B lymphocytes were defective in IgG production in vitro. Ultrastructure of the neutrophils was normal. The marrow cells formed normal numbers of granulocytic colonies in culture, but the colonies were apparently small in size. The levels of colony-stimulating activity were normal. The lymphocytes did not impair granulopoiesis of control marrow cells. These data indicate that the neutropenia and defective neutrophil chemotaxis are due to the intrinsic neutrophil defects and are not secondary to T and/or B lymphocyte dysfunctions in the patient.

Agammaglobulinemia↗
PubMed1980

Delayed in vitro immunoglobulin production by cord lymphocytes.

Pokeweed mitogen-induced immunoglobulin (Ig) production by cord lymphocytes was studied in vitro by Ig-secreting plaque-forming cell (Ig-PFC) assay. Although adult mononuclear cells generated all of IgM-, IgG-, and IgA-PFC, cord mononuclear cells generated only IgM-PFC when cultured for seven days. The number of cord IgM-PFC was 102 +/- 26/10(4) mononuclear cells, being about one fourth of that of adult IgM-PFC. When cultured for 14 days, cord mononuclear cells formed increased numbers of IgM-PFC in contrast to adult cells and yielded IgG-PFC as well, indicating delayed Ig production. Cord T cells were much less effective at helping adult B cells to differentiate into Ig-PFC as compared with adult T cells. Substitution of adult T cells for cord T cell markedly improved the response of cord B cells. The present study demonstrates Ig secretion by cord lymphocytes in response to pokeweed mitogen stimulation. The results further indicate that the delayed Ig production by cord lymphocytes is largely due to functional immaturity of the T cells.

B-Lymphocytes↗
PubMed1980

Effect of peripheral blood mononuclear cells from aplastic anemia patients on the granulocyte-macrophage and erythroid colony formation in samples from normal human bone marrow in vitro--a cooperative work.

Thirty-six patients with aplastic anemia and three with aplastic anemia-PNH syndrome in eight institutes were studied for the presence of peripheral blood mononuclear cells having a suppressive effect on granulocyte-macrophage (G-M) and erythroid colony formation using a uniform protocol. In 11 cases of 29 (38%) and 9 cases of 29 (31%), the presence of mononuclear cells with a suppressive effect on G-M and erythroid colony formation, respectively, was demonstrated. However, the presence of mononuclear cells suppressive both to G-M and erythroid colony formation was demonstrated only in 3 of 19 cases (16%).

Adolescent↗
PubMed1979

Abnormal neutrophil maturation in a neutrophil defect with morphologic abnormality and impaired function.

Neutrophils from a patient with recurrent pyogenic infections since infancy were found to have morphologic abnormalities and impaired functions. The neutrophils had an abnormal nuclear shape, no or few secondary granules, and no alkaline phosphatase activity. Primary granules were normal in number and structure, and were positive for peroxidase. Immature granulocytes were structurally normal. The neutrophils were impaired in chemotaxis and bactericidal capacity. The patient's marrow cells formed increased numbers of granulocytic colonies of small size in culture. Her peripheral leukocytes produced elevated levels of CSA and adherent marrow cells did not inhibit colony formation. These data indicate an intrinsic neutrophil defect which allows normal proliferation of precursor cells, but results in abnormal morphogenesis and impaired function as the cells mature.

Bone Marrow↗