Hodgkin's disease and Sjögren's syndrome.
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Biomedical subjects
Publications and source records attributed to M Nagai.
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The gene encoding the beta-amylase of Bacillus cereus BQ10-S1 (SpoII) was cloned into Escherichia coli JM 109. A sequenced DNA fragment of 2,001 bp contains the beta-amylase gene. The N-terminal sequences (AVNGKG MNPDYKAYLMAPLKKI), the C-terminal sequences (SHTSSW), and the amino acid sequences of the five regions in the beta-amylase molecules were determined. The mature beta-amylase contains 514 amino acid residues with a molecular mass of 57,885 Da. The amino acid sequence homology with those of known beta-amylases was 52.7% for Bacillus polymyxa, 52.0% for Bacillus circulans, 43.4% for Clostridium thermosulfurogenes, 31.8% for Arabidopsis thaliana, 31.5% for barley, 29.9% for sweet potato, and 28.9% for soybean. Ten well-conserved regions were found between the N terminus and the area around residue 430, but the C-terminal region of 90 residues has no similarity with those of the plant beta-amylases. The homology search revealed that this C-terminal region has homology with C-terminal regions of the beta-amylase from C. thermosulfurogenes, some bacterial alpha-amylases, cyclodextrin glucanotransferase, and glucoamylase. Some of these sequences are known as the raw-starch-binding domain. These results suggest that B. cereus beta-amylase has an extra domain which has raw-starch-binding ability and that the domain has considerable sequence homology with those of other amylases or related enzymes from a wide variety of microorganisms.
A necropsy case of human T lymphotropic virus I (HTLV-I) associated myelopathy (HAM) in a 64 year old man with serological and genetical confirmation of HTLV-I infection is reported. The spinal cord, lung, and skin were mainly affected. Severe degeneration had occurred in the spinal cord, not only in the lateral columns but also in the anterior and posterior columns. The degenerate lesions showed proliferation of capillaries, loss of myelin and axon, and perivascular and parenchymal infiltration with T lymphocytes and foamy macrophages in the white matter. T lymphocytes had infiltrated the lung and there was vascular proliferation in the peribronchus. OPD4 positive cells predominated in the lung. The patient also had erythrodermia where dense and bandlike HTLV-I infected lymphoid cell infiltration was observed, with mild atypia and epidermotropism. HTLV-I may cause multiorganic inflammatory disorders, although the definitive role of HTLV-I in the pathogenesis is still unknown.
The frequency of streptococcal infections has been reported to be increasing. To determine the significance of such infections complicating treatment of leukemia, we studied the incidence and clinical features of Streptococcus mitis septicemia among 51 leukemia patients in our department. During 166 consecutive treatment courses for leukemia, eight episodes of Streptococcus mitis septicemia were observed in 35 septicemic patients. In seven out of eight episodes (88%), severe mucositis developed after aggressive chemotherapy, suggesting that oral mucosa might be the site of entry for Streptococcus mitis. The isolates were sensitive to imipenam/cilastatin and cefuzonam, and were relatively resistant to amikacin. Although none of the patients died of Streptococcus mitis septicemia, life-threatening adult respiratory distress syndrome (ARDS) developed in two independent treatment courses. We should thus be aware of the risk of ARDS in patients with Streptococcus mitis septicemia.
Here we report a 58-year-old man with chronic myelogenous leukemia in the chronic phase, who developed acute respiratory failure following administration of high dose natural interferon delta (6,300 x 10(4) units/week). Radiological and histological findings were consistent with acute interstitial pneumonia (AIP). Although the pathogenesis remains unclear, it is important to watch for the possible development of AIP when employing interferon delta therapy, especially at high doses.
The authors report a case of 6-month-old boy with cerebellar medulloblastoma associated with malignant rhabdoid tumor of the kidney, presenting with an abdominal mass, large head and projectile vomiting. Following removal of the renal tumor, the mass arising from the superior vermis about 6 cm in diameter was removed by a combined right occipital transtentorial and suboccipital approach. The patient had been well for 3 months after surgery followed by chemotherapy but died eventually of tumor recurrence in the abdomen. Reported cases of malignant rhabdoid tumor of the kidney associated with brain tumor are reviewed and its characteristics are discussed.
The causes of death in long-term survivors of stomach cancer after a gastrectomy have been investigated in a follow-up study of 320 such patients, with special focus on the postoperative development of a second primary cancer. The five- and 10-year overall survival rates of early stomach cancer patients were 87.5 and 75.8%, respectively, and of advanced stomach cancer patients, 44.2 and 37.5%, respectively. A multivariate analysis identified the patient's age and stage of cancer at the time of diagnosis as separate prognostic factors for overall survival. During the observation period, 15 (4.7%) patients developed a second primary cancer, so that no significantly elevated risk of developing a second primary cancer was demonstrated. During the same observation period, however, 48 (15%) of the patients died of a non-cancerous disease, 12 succumbing to an acute myocardial infarction and/or heart failure. Given these results, it is felt that continued follow-up of long-term stomach cancer survivors should be pursued to prevent or impede the development of a non-cancerous disease and/or a second primary cancer, so that the life of such former patients can be prolonged.
A 57-year-old female, who had a 14-year history of rheumatoid arthritis, developed aplastic anemia. Treatment with anti-lymphocyte globulin (ALG) and methylprednisolone pulse therapy achieved complete remission. However, twenty months after ALG treatment she developed acute monocytic leukemia. In spite of the chemotherapy, consisting of cytosine arabinoside and etoposide, she died of pneumonia. Recently, reports from Western countries have claimed that patients with aplastic anemia frequently develop clonal disorders following ALG treatment. To our knowledge, this is the first report in Japan of a case developing acute leukemia following ALG treatment.
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The following presents 6 cases of therapy related leukemia (TRL) along with discussion of their clinical features in comparison with those previously reported in Japan. Common primary malignancies were mammalian cancer, lung cancer and malignant lymphoma in both groups. It was observed that, 1) average age was higher (68 years), 2) average latent period from primary malignancy to leukemia was longer (10 years), particularly in patients treated solely with radiation, 3) in 4 out of 6 patients (67%) karyotype analysis of leukemia cells showed normal results, but in one case previously administered etoposide, translocation involving 15q+, 17q- was noted, and 4) the CR ratio in our cases was 83%; half the cases are still alive at 10 months of follow-up, while in previously reported cases the CR ratio was 41%, and the median survival time was 12 months according to Kaplan-Meier analysis. Although poor response to conventional chemotherapy has been reported in TRL patients, the present data indicated TRL in some cases to achieve complete response and long-term survival. Aggressive chemotherapy should be considered for such patients.
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Chromosome translocation t(15;17) specifically found in acute promyelocytic leukemia (APL) results in cleavage in the introns of PML gene on chromosome 15 and in the intron of the retinoic acid receptor alpha (RAR alpha) gene on chromosome 17, creation and expression of PML-RAR alpha and RAR alpha-PML fusion genes. Reverse transcription-polymerase chain reaction (RT-PCR) was applied to detect the PML-RAR alpha fusion transcripts rapidly in APL patients. The fusion transcripts could be detected in all of the 10 APL patients studied. Of the two breakpoints in the PML gene so far reported, seven APL patients had the fusion transcript compatible with the downstream (3') breakpoint, and the other three APL patients were considered to have the upstream (5') breakpoint. RT-PCR could detect the fusion transcripts from as little as 50 pg bone marrow RNA, and from as little as 0.5 pg bone marrow RNA with the nested PCR. This method was applied to detect minimal residual leukemia cells in an APL patient who had undergone allogeneic bone marrow transplantation, in whom the RT-PCR could not detect the PML-RAR alpha fusion transcripts at several post-transplant time points. This system could be useful to detect minimal residual leukemia cells and accordingly modify the treatment strategy, as well as to make a quick diagnosis with a small amount of clinical sample.
A case of acute non-lymphocytic leukemia who relapsed 7 years after the first remission is reported. The leukemic cells at onset had mature monocytic features with positive reactions for anti-CD14 antibody and alpha-NBE staining. By contrast, leukemic cells at relapse showed distinct morphological features from those at onset and lost their monocytic characters. No karyotypic abnormalities were found, but we could not exclude the possibility of secondary leukemia induced by the initial anti-leukemic chemotherapy.
From 1985 to 1991, 20 patients with adult acute lymphoblastic leukemia (ALL) were treated with the DCVP-l-asp protocol, which consists of daunorubicin, cytosine arabinoside, vincristine, prednisolone, and I-asparaginase. of these 17 patients (85%) achieved complete remission. A Kaplan-Meier analysis predicted that 30% of the patients in remission would remain disease-free at 5 years. Four patients relapsed between 1 and 2.5 years from the start of treatment. The median survival time for the four patients with an abnormal karyotype was 15 months. No meningeal relapses occurred in patients treated with regular CNS prophylaxis. Myelosuppression was severe, but was well tolerated with supportive therapy, and there were no treatment-related deaths. We conclude that DCVP-l-asp is a feasible and effective protocol for adult ALLs including elderly patients. However, new treatment modalities, such as bone marrow transplantation, should be explored in patients with unfavorable prognostic factors.
To evaluate the efficacy of hemoglobin-Alc, fructosamine, and 1,5-anhydroglucitol tests in screening for diabetes mellitus in communities, the sensitivity and the specificity of these tests were observed on the island of Ojika, Goto islands, Nagasaki prefecture Japan. A 75 gram glucose tolerance test was performed simultaneously on every participant, and WHO diabetes mellitus criteria were used to classify individuals. A total of 554 males and 820 females, older than 34 years, participated in the examination, representing a response rate of 42% and 52% respectively, and 86 were classified as diabetic. The sensitivity and the specificity of several screening levels were determined for each test. Each of the tests attained greater than 70% sensitivity and specificity simultaneously, but could not achieve greater than an 80% level simultaneously. If screening levels are selected for a sensitivity of as low as 50%, it is possible to produce a high specificity of 97-98%. These results provide valuable information for the planning of efficient diabetes mellitus detection programs in communities.
Previously we reported that there is a kallikrein deficiency in the cerebral tissue of patients with Alzheimer-type dementia. The present study was performed to investigate protease changes in the serum of these patients. The results showed that the kallikrein activity was normal, but that the activities of plasmin and urokinase were significantly low. The present findings indicate a derangement in the clotting and fibrinolytic systems in Alzheimer patients.
The effect of Bordetella heat-labile toxin (HLT) on cultured vascular or tracheal smooth cells labeled with [14C]arachidonic acid for 3 h was examined. At 37 degrees C, in the presence of Ca2+, HLT induced the release of radioactive substances from the cells in a dose-dependent manner but HLT had no effect on release of radioactivity from cells at 0 degrees C or in the absence of Ca2+. After cells were exposed to HLT, a 2-h lag period occurred before release of radioactivity was detected. The substances released from cells by HLT were identified as arachidonic acid and phosphatidylcholine.