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Biomedical subjects

M Osame

Publications and source records attributed to M Osame.

At least 415 records · Page 23Linked to original sources
PubMed1990

[Pure red cell aplasia with monoclonal gammopathy and von Willebrand disease].

We report a case of pure red cell aplasia (PRCA) with benign monoclonal gammopathy (BMG) of IgA.lambda type and type I von Willebrand disease (vWD). A 61-year-old female patient was treated initially with prednisolone, azathioprine and cyclophosphamide with transient and unsatisfactory reticulocyte response. Oral administration of 200 mg of cyclosporine A (CyA)/day was started from July, 1987. A rapid and marked reticulocytosis was seen from a week later and there was a rapid increase in hemoglobin levels, and remission has been maintained for over 22 months. Patient's serum and IgA taken on admission did not show inhibitory activity to both CFU-E growth from her own bone marrow cells obtained in remission and von Willebrand factor. T cell-mediated suppression to CFU-E growth was detected. On family study, the patient's second son was found to be a type I vWD. These results indicate that there is no direct causal relationships between BMG and PRCA or vWD, and that CyA may have a place in the management of PRCA.

Cyclosporins↗
PubMed1990

[Effects of danazol on plasma levels of protein S C4b-binding protein].

In order to elucidate the response of protein S (PS) and C4b-binding protein (C4bp) to danazol, we investigated the serial changes in plasma levels of PS and C4bp during danazol administration in 4 patients with endometriosis. Both total and free PS levels were significantly increased at the time of 2 to 3 weeks after danazol administration as compared to the pretreatment values, while C4bp levels were not changed at all. These results imply that danazol administration to PS deficiency would produce a significant increase in the basal level of free PS, suggesting a potential adjunct to anticoagulant treatment in PS deficiency.

Adult↗
PubMed1990

[Collagen adhesion-aggregation abnormality (2nd report)--congenital platelet membrane glycoprotein VI deficiency].

Previously, we reported a case of 26-year-old woman with a mild bleeding tendency whose platelets specifically lacked collagen-induced aggregation and adhesion to collagen fibrils. In this report, we investigated the membrane glycoproteins of this patient's platelets and found that her platelets were absent in a 61-kDa glycoprotein, which was identified to be glycoprotein VI (GP VI) of the platelet membrane. Her parents platelets contained about 50% the normal amount of GP VI. These results indicate that our patient has a congenital homozygous GP VI deficiency and that GP VI functions as a collagen receptor.

Adult↗
PubMed1990

The risk of development of HTLV-I-associated myelopathy/tropical spastic paraparesis among persons infected with HTLV-I.

Using data obtained in national surveys of human T-lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) conducted in Japan in 1987 and 1988, we estimated the yearly and lifetime risk that HAM/TSP will develop in an HTLV-I-infected person. "Definite" HAM/TSP was defined as slowly progressive myelopathy with antibodies to HTLV-I in both serum and cerebrospinal fluid. Estimates of HTLV-I infection rates in eight endemic prefectures, by age group and sex, were obtained from serologic studies of blood donors; population figures, by age group, sex, and prefecture, were obtained from the census. Of 589 definite cases of HAM/TSP reported nationally, 397 occurred in residents of the eight endemic prefectures; of these, 170 reported onset of illness during the years 1982-1988 (average incidence, 24.3 cases/year). Using the estimated HTLV-I infection rates and the 1985 census figures, we estimated the number of HTLV-I-infected persons in the eight prefectures in 1985 at 794,800. We therefore estimated the incidence of HAM/TSP among HTLV-I-infected persons at 3.1 x 10(-5) cases/year; assuming a lifetime of 75 years, the lifetime incidence is approximately one quarter of 1%. This estimate is important in counseling persons such as blood donors found to be infected with HTLV-I.

Adolescent↗
PubMed1990

[Fetal survival after the corticosteroid and low-dose aspirin regimen in a case of anti-phospholipid antibody syndrome].

A 27-year-old woman with anti-phospholipid antibody syndrome, whose first pregnancy had ended in intrauterine fetal death, was treated with prednisolone 0.6 mg/kg/day before second pregnancy. Suppression of anti-cardiolipin antibody activity was rapidly achieved and maintained during the pregnancy. After conception, low-dose aspirin 81 mg/day was started and continued until delivery. She gave birth to a normal live infant with natural full-term delivery. It is strongly suggested that the corticosteroid and low-dose aspirin regimen can lead to successful pregnancy in cases with anti-phospholipid antibody syndrome like the present case.

Adult↗
PubMed1990

[Increase of serum acidic isoferritins in patients with hemophagocytic histiocytosis].

We investigated serum isoferritin patterns in 2 patients with reactive hemophagocytic histiocytosis (HH) and 1 patient with neoplastic HH (malignant histiocytosis) whose serum ferritin levels were extraordinarily high. Serum ferritins in 2 patients with reactive HH were composed of mildly (pI 5.55 to 5.10) to intermediately (pI 4.81 to 4.82) acidic isoferritins, while those in patient with neoplastic HH were composed of more acidic (pI 4.70 to 4.45) isoferritins. These results indicate that the main constituents of the increased serum ferritins in HH would be acidic isoferritins, and that more acidic isoferritins would be increased in neoplastic HH than reactive HH.

Adult↗
PubMed1990

[Two cases of HTLV-I associated myelopathy (HAM) complicated with Sjögren's syndrome, T-lymphocyte alveolitis and arthropathy].

Two cases of HTLV-I-associated myelopathy (HAM) complicated with Sjögren's syndrome (SjS), T-lymphocyte alveolitis and arthropathy were reported. Case 1 was a 55-year-old woman. Since 40 years of age she had been suffering from repetitive pulmonary infection. She also noted polyarthralgia since 42 years of her age and was diagnosed as SjS at her age 43. She developed gait disturbance since April 1988. Case 2 was a 65-year-old woman. She began to have gait disturbance at 62 years of age. A right knee joint pain started in December 1988. The two cases have the following features in common: 1. The diagnosis of HAM is definite because of pyramidal signs and positive anti-HTLV-I antibody in both serum and cerebrospinal fluid. 2. The histological findings of the minor salivary glands are compatible with SjS. 3. Differential cell count in bronchoalveolar lavage fluid (BALF) showed an increase in lymphocytes which suggested the presence of clinical or subclinical T-lymphocyte alveolitis. 4. A mono-or polyarthropathy is observed. These findings are suggestive that HAM is not the disease restricted within the central nervous system but the disease with systemic involvement which may be caused by the activated T-lymphocytes. Thus the two cases are interesting for the understanding of the pathogenesis of HAM.

Aged↗
PubMed1990

[A family with von Willebrand disease and hypofibrinogenemia].

A family with two complex disorders of hemostasis, von Willebrand disease (vWD) and hypofibrinogenemia was reported. The probands were 21- and 16-year-old full brothers suffering from serious bleeding tendencies from childhood. The elder brother had a subarachnoid hemorrhage at the age of 15. The younger brother had repeated episodes of gastrointestinal bleedings since he was 10 years of age. Coagulation studies revealed that both of them had almost the same hemostatic abnormalities, i.e. severe vWD, 9 to 12% of plasma vWF levels, and mild hypofibrinogenemia, 125 to 130 mg/dl of plasma fibrinogen levels. Multimeric compositions of their vWF were normal, and functional assay for fibrinogen concentration yielded essentially the same values as did immunologic assay. These results indicated that they had two complex disorders, extreme type I vWD and heterozygous state of afibrinogenemia resulting in serious bleeding tendency. Family study showed that these two hemostatic disorders were paternal inheritance, and it was strongly postulated that vWD and hypofibrinogenemia might be highly-combined hemostatic disorders.

Adolescent↗
PubMed1990

[Peculiar increase of plasma plasminogen activator inhibitor 1 levels in patients with hemophagocytic histiocytosis].

There was a markedly significant increase of plasma levels of tissue-type plasminogen activator, plasminogen activator inhibitor 1 (PAI-1) and PAI activity in 3 patients with hemophagocytic histiocytosis (HPH) as compared with DIC patients with multi-organ failure (MOF). Of particular interest was the peculiar increase of PAI-1 levels, that is, the average PAI-1 level was 2,673 ng/ml, while that in DIC patients with MOF was 66 ng/ml. We conclude that the striking increase of plasma PAI-1 levels may be a pathognomonic feature of HPH and may contribute to the pathogenesis of DIC and/or MOF associated with HPH.

Adolescent↗
PubMed1990

[Accessory use of protease-inhibiting agents for disease remission in a patient with autoimmune hemolytic anemia associated with B-CLL].

Protease inhibiting agents, which have inhibitory effects on complement system, were used to treat a patient with autoimmune hemolytic anemia (AIHA) associated with B cell chronic lymphocytic leukemia (B-CLL). Although this patient had failed to respond thoroughly to prednisolone, the additional use of protease inhibitors induced a disease remission of hemolytic anemia. The result suggests that complement components are of importance in the pathogenesis of hemolysis in AIHA, and supports the accessory use of protease inhibiting agents in autoimmune hematologic diseases associated with the complement activation and hypersplenism.

Aged↗
PubMed1990

[Coincidental occurrence of idiopathic thrombocytopenic purpura in a patient with familial von Willebrand disease].

A very rare case with coincidental idiopathic thrombocytopenic purpura (ITP) and familial von Willebrand disease (vWD) was reported. A 23-year-old female was admitted because of unusual bleeding tendency lasting still after the remission of thrombocytopenic state associated with ITP. Coagulation studies indicated the presence of a mild vWD, which was thought to be responsible for the bleeding tendency. The qualitative analysis of von Willebrand factor by crossed immunoelectrophoresis and SDS-1.2% agarose gel electrophoresis showed normal multimeric composition compatible with type I vWD. vWF-inhibitor was negative, and family study revealed that her mother was also affected with type I vWD. These results suggested that there was no immediate causal relation between the two disease states. The prevalence of concomitant disease states with ITP and vWD was discussed.

Adult↗
PubMed1990

[Occurrence of hemophilia A and von Willebrand disease in the same family].

Occurrence of the two congenital hemorrhagic disorders, von Willebrand disease (vWD) and hemophilia A, was found in the same family. The propositus was a 21-year-old male patient with findings of moderate hemophilia A. Father, the eldest sister and second eldest sister were found to have mild type I vWD. Mother was confirmed to be a hemophilia A carrier, and the eldest son of second eldest sister to be a moderate hemophilia A. These results suggest that the occurrence of the two hemorrhagic disorders may have been resulted from the incidental mating of father's vWD gene and mother's hemophilia A gene, and suggest that second eldest sister may be the double heterozygosity of type I vWD and hemophilia A.

Adult↗
PubMed1990

[Occurrence of subdural hematoma closely associated with danazol administration in a patient with refractory ITP].

We reported a 34-year-old female patient with refractory idiopathic thrombocytopenic purpura (ITP) in whom a subacute subdural hematoma occurred without any preceding trauma during danazol administration which resulted in a marked decrease of plasma fibrinogen level. It is strongly suggested that danazol should be very carefully administered in ITP patients with serious bleeding tendency.

Adult↗
PubMed1990

[Efficacy of leukocytapheresis and low-dose prednisolone treatment in a patient with HTLV-I-associated myelopathy (HAM)].

The patient, a 66-year-old woman, was admitted because of a two year history of slowly progressive gait disturbance. A diagnosis of HTLV-I-associated myelopathy (HAM) was made on the basis of the clinical and serological criteria. Although the ordinary dose of oral prednisolone (PSL) is more than 30 mg in the treatment of HAM, we treated this patient by low-dose (5-10 mg) oral PSL administration. A series of leukocytapheresis performed before the PSL treatment halted the progression of symptoms transiently. Alterations in peripheral blood lymphocyte (PBL) subpopulations and augmented autologous proliferative response of PBLs improved concurrently with the resolution of neurological symptoms after the low-dose PSL treatment. The result suggests that some cases with HAM may respond with PSL treatment in low doses.

Administration, Oral↗
PubMed1989

Lymphocyte alpha-glucosidase in late-onset glycogenosis type II.

We describe the biochemical characterization of lymphocyte alpha-glucosidase in a 23-year-old man with intermediate clinical features between the childhood and adult forms of glycogenosis type II (Pompe's disease). Acid alpha-glucosidase activity was markedly reduced, but immunologic cross-reactive material against human liver acid alpha-glucosidase protein could be detected, and its amount was normal. In this patient, the disorder was induced by the catalytically inactive enzyme with a normal amount of enzyme protein.

Adult↗