Fatal intravascular hemolysis induced by platelet concentrate.
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Biomedical subjects
Publications and source records attributed to H Take.
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Adult Still's disease is a variant of juvenile rheumatoid arthritis occurring most frequently in women 16-35 years of age, but rarely in elderly people. We describe a 75-year-old woman who was considered as having adult Still's disease.
The prevalence of IgG antibody against cytomegalovirus (CMV) was compared between the age-matched (0 month to 2 years of age) groups of 212 breast-fed children and 223 bottle-fed children to examine the role of breast milk for acquisition of CMV. Mothers of both groups of children were also examined for CMV IgG antibodies. Both the breast-fed and bottle-fed children groups showed high seropositivity for CMV at 0 to 2 months of age, which gradually decreased and bottomed at 6 to 8 months of age. Thereafter, in the breast-fed children group, the seropositivity rate increased up to 70% by 1 year of age. In contrast, in the bottle-fed children group, the seropositivity rate remained at the bottom level of lower than 30%, without showing any apparent increases. The serological data of the children whose mothers were confirmed to be seropositive, revealed that mother-to-child transmission of CMV occurred in 11 of 17 (64.7%) of the breast-fed children and in 24 of 87 (27.6%) of the bottle-fed children. All the bottle-fed children born to seronegative mothers remained seronegative for CMV up to 1 year of age. The bottle-fed children showed significantly lower seropositivity than the breast-fed children, although most of both groups of children were born to seropositive mothers. The results strongly suggested that about 40% of the breast-fed children acquire CMV via breast milk and breast-feeding has certain protective effects on congenital CMV disease in the offspring.
We examined the sera of family members of human T cell leukemia virus type I (HTLV-I) seropositive pregnant women who had visited Kagoshima City Hospital since 1986, and studied the routes of transmission of HTLV-I. A new enzyme linked immunosorbent assay (ELISA) for detecting the antibody to an HTLV-I tax gene product, p40tax, has recently been developed. By this ELISA method, the positive rate of anti-p40tax among HTLV-I seropositive subjects, including 96 pregnant women (index subjects), 26 mothers, 13 husbands, and 13 children was investigated. The percentage positive for anti-p40tax among pregnant women, mothers, husbands, and children was 41.6, 50, 53.8 and 53.8%, respectively. This means that the positive rate of anti-p40tax remains almost constant with increasing age. The rate of mother-to-child transmission of HTLV-I was significantly higher in p40tax seropositive (29.6%) than in seronegative mothers (8.1%). The positive rate of anti-p40tax in transmission from husband to wife (29%) and through blood transfusion (17%) was lower than the overall prevalence (46%). Thus, these data suggest that p40tax antibodies are associated with the frequency of HTLV-I transmission and with the differences in the transmission routes.
A 69-year-old woman was admitted to our hospital with a 7-month history of sensory disturbance of the bilateral lower extremities. Since she developed paraplegia of the extremities, urinary incontinence and left hemiplegia several days after admission, neurologic involvement both in the lumbar cord, and in the cervical cord or the brain was suspected. While no abnormalities were noted by computerized tomography of the brain. T2-weighted magnetic resonance imaging (MRI) clearly demonstrated foci in the periventricular and the basal ganglia regions bilaterally. Furthermore, the levels of immunoglobulin G and interleukin 6 were increased in the cerebrospinal fluid (CSF). From physical and other laboratory findings in addition to the MRI and CSF findings, she was diagnosed as having systemic lupus erythematosus with central nervous involvement. The administration of prednisolone resulted in marked improvement in her neurologic symptoms in two months. Thus, it is considered that the MRI and CSF examinations are useful for the diagnosis and treatment of central nervous involvement of systemic lupus erythematosus.
A 21-year-old female with atopic dermatitis showed "dependence" on very hot hot-spring bathing. Her skin disease had been refractory to various treatments including steroid therapy for a long time. Without medical supervision she took four 3-minute 47 degrees C hot-spring baths daily for a month for the purpose of improving her skin symptom. Subsequently, she could not stop bathing in very hot hot-spring water of her own will. However, a month's isolation in a hospital relieved her of the situation. The mechanism of the dependence on very hot hot-spring bathing may be explained by a transient rise in the plasma beta-endorphin level due to hyperthermal stress.
We describe a 31-year-old patient with ulcerative colitis who was successfully treated with prednisolone (PSL). Immunologic analyses were performed during the treatment course. Suppressed natural killer (NK) cell activity and increased levels of the CD4/CD8 ratio were observed on admission. Transient restoration of NK cell activity was obtained by PSL treatment. However, it returned to the initial low level in spite of the improvement of clinical symptoms. Possible mechanisms are discussed for involvement of the immune system in the pathogenesis of ulcerative colitis (UC).
We report a successful treatment with auranofin in a case of elderly-onset Still's disease. The administration of non-steroidal anti-inflammatory drugs, mizoribine and prednisolone were not only insufficient to suppress the disease activity but were followed by the development of multiple gastric ulcers and severe osteoporosis. On the contrary, treatment with auranofin, 9 mg per day, was effective enough to maintain the disease inactive without any side effects. We also found that plasma levels of interleukin-6 and tumor necrosis factor-alpha were significantly elevated in our patient, suggesting a possible involvement of inflammatory cytokines in this disease.
An enlarged spleen failed to accumulate Tc-99m-labeled phytate, but it showed normal perfusion and uptake of labeled and heat-denatured red blood cells. A blood cell count demonstrated moderately low hemoglobin and hematocrit levels and a markedly low platelet count, and results of direct Coombs' and antiplatelet-antibody (IgG) tests were positive. Steroid therapy resulted in normal splenic function. This case demonstrates reversible functional asplenia caused by reticuloendothelial dysfunction secondary to pure splenic chronic lymphocytic leukemia.
Fifty five children diagnosed as having high-risk acute lymphoblastic leukemia (ALL) between 1985 and 1988 were treated with protocol AL851. The agents used in the protocol were as follows: induction therapy: vincristine (VCR), prednisolone, daunorubicin (DNR) and l-asparaginase, consolidation therapy: an intermediate-dose methotrexate (MTX), central nervous system (CNS) leukemia prophylaxis: intrathecal MTX and 24Gy cranial irradiation, reinduction therapy: VCR, adriamycin, dexamethasone and high dose cytarabine (AraC), maintenance therapy: 6-mercaptopurine, cyclophosphamide, MTX, DNR, VCR and AraC. Patients received chemotherapy for 3 years after achieving complete remission (CR). CR was obtained in 51 patients (92.7%). Twenty-four of them relapsed after achieving CR (bone marrow 16, CNS 3 and testis 5). At median follow-up of 79 (range 64-102) months, the estimated 8-year disease free survival rate was 49.1 +/- 6.7%. Four patients relapsed at bone marrow during the first 6 months of the treatment, indicating that more intensive combination chemotherapy should be included in earlier stage of the protocol. The high incidence of testicular relapse (14.3% in boys) suggests that high-dose MTX or AraC should be needed for improvement of the prognosis of high-risk ALL patients.
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To clarify the molecular basis of the deficiency of glycoprotein IV (GPIV) of the platelet surface, we analyzed GPIV cDNA synthesized from platelet RNA of five unrelated Japanese subjects whose platelets did not express GPIV. We confirmed the presence of normal-sized GPIV mRNA in platelets from subjects with GPIV deficiency. The sequence of platelet GPIV cDNA from GPIV deficient subject showed three differences when compared with the published sequence; 1) a replacement of a 478CCT codon for proline-90 by TCT for serine, 2) a four-base insertion in the 3'-noncoding region, and 3) a substitution of A for 79C in the 5'-noncoding region. The replacement of Pro90 by Ser predominates in subjects with GPIV deficiency; that is, four out of five platelets with GPIV deficiency contained GPIV mRNA encoding GPIVSer-90, while all platelets from 17 GPIV positive subjects had GPIV mRNA encoding GPIVPro-90. The sequence of platelet GPIV cDNA which did not encode GPIVSer-90 from a subject with GPIV deficiency revealed no abnormality in the coding region. The four-base insertion in the 3'-noncoding region and the substitution of A for 79C in the 5'-noncoding region seems to be unrelated to the expression of GPIV. The substitution of Ser for Pro90 might alter the GPIV structure or impair GPIV biosynthesis, resulting in a lack of detectable GPIV. This hypothesis remains to be tested.
Pathological examination was carried out of the skeletal muscle of an 8-year-old boy with abetalipoproteinemia. The patient complained of diarrhea, and showed a deficiency of betalipoprotein, decreased fat-soluble vitamins, acanthocytosis and a mild increase in serum creatine kinase. The prominent histochemical finding was punctate deposits of acid phosphatase activity in most fibers. Ultrastructural lesions revealed a number of giant lysosomes. Although these pathological findings seemed to be related to vitamin E deficiency, other pathological findings such as concentric laminated bodies or filamentous bodies were also observed. The clinical course and the changes in the pathological findings in our patient after long-term vitamin E therapy need to be observed.
HTLV-I transmission routes were found for 66 carrier pregnant women by studying sera, from the carrier pregnant women, their mothers, and their husbands, and by obtaining detailed family histories at interview. Forty-one cases (62.1%) were considered to be instances of vertical transmission, 15 (22.8%) of sexual transmission, 6 (9.1%) of blood transfusion, and 4 (6.1%) undecided. To date, most cases of adult T-cell leukemia (ATL) have been considered to result from vertical transmission. Our results therefore imply that about 30% (22.8% + 9.1%) of the carrier pregnant women are at minimal risk of ATL. Moreover, in case of presumed husband-to-wife transmission, more than half (6/11) were infected between one year and four years after marriage.
Breast feeding is the major route of mother-to-child transmission of human T-cell leukemia virus type I (HTLV-I). Our experiments with rabbits have shown that passive immunization is capable of blocking cell-to-cell infection of HTLV-I by blood transfusion or breast feeding. In this study, sera were collected serially from 3 infants born to seropositive mothers and were tested for the presence of neutralizing antibody to vesicular stomatitis virus (HTLV-I) pseudotype as well as antibodies to viral structural proteins. There was a good correlation between neutralizing and viral antibody titers, both of which were detectable until 3-6 months after birth. Whether maternally transmitted neutralizing antibody is protective against perinatal infection of HTLV-I remains to be studied.
The platelet antigen N(aka) was once considered to be a platelet-specific alloantigen and is carried on platelet membrane glycoprotein (GP) IV. Recent studies suggest that N(aka)-negative subjects lack platelet GPIV. GPIV is an important adhesive receptor and expressed on the surface of monocytes as well as of platelets. In the present study, flow cytometry was used to detect GPIV and N(aka) antigen on the surface of monocytes. N(aka) antigen was expressed on monocytes as well as on platelets in N(aka)-positive subjects (n = 6) (P-GPIV-positive subjects). To our surprise, monocytes of N(aka)-negative subjects (n = 7) (P-GPIV-negative subjects) having no anti-N(aka) antibody in their serum expressed GPIV and N(aka) antigen to almost the same degree as did the monocytes of P-GPIV-positive subjects. Competitive experiments using OKM5 (a monoclonal antibody against GPIV) and anti-N(aka) antibody showed that the epitope of anti-N(aka) antibody on monocytes was very close to that of OKM5. In two P-GPIV-negative subjects having anti-N(aka) antibody in their serum, GPIV and N(aka) antigen were not expressed on the surface of either monocytes or platelets. These results indicate that the GPIV molecules and N(aka) antigen are expressed on the surface of monocytes in the majority of P-GPIV-negative subjects, but that in a very few P-GPIV-negative subjects neither GPIV nor N(aka) antigen is expressed on the surface of their monocytes. We hypothesize that P-GPIV-negative subjects who carry neither GPIV nor N(aka) antigen on their monocytes produce anti-N(aka) antibody as a result of transfusion or pregnancy.
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A rare case of non-Hodgkin's lymphoma (NHL) that showed transient spontaneous regression (SR) is described. After 6 months of remission, recurrence was noted in the lymph nodes, pleura and the spleen. Although transient improvement was observed following combination chemotherapy, the pleural effusion became refractory to chemotherapy and the patient died 9 months after the relapse.