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We describe a Japanese family with familial platelet disorder with propensity to develop myeloid malignancies (FPD/MM). Among the three affected individuals, two members developed myeloid malignancies. Sequence studies demonstrate that all affected individuals of the pedigree display a heterozygous single nucleotide deletion in exon 8 of the RUNX1 gene.  相似文献   
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BACKGROUND: In Japan, the inoculation dosage of inactivated influenza vaccine for children under 1 year old is 0.1 mL per dose. The dosage is not half as much as that in Europe and the U.S.A. We considered that low efficacy fate of influenza vaccine in children under 1 year old results from its less dosage. So we designed this study to verify this hypothesis. MATERIALS AND METHODS: This study was prospective in design. Subjects were divided into two groups by age: 8 to 11 months old (n = 26) and 12 to 16 months old (n = 22). Infants received 0.1 mL of inactivated influenza vaccine and over 1 year, 0.2 mL. Forty-eight children were inoculated twice at intervals of over 4 weeks. Serum samples were drawn before the first inoculation and 1 month after the second vaccination. Pre- and post-immunization antibody titers were measured. The titers of hemaglutinatinin inhibiting antibodies to the 3 viral strains were assayed. Antibody titers were determined using HAI. RESULTS: The post-vaccination proportions of children with protective HAI antibody titers were significantly smaller in infants than those in children over 1 year old (A/H1N1; 23% vs. 77%, A/H3N2; 39% vs. 73%, B; 0% vs. 32%). The number of children with >four-fold increased antibodies were significantly smaller in infants than that in 1 year old (A/H1N1; 74% vs. 91%, B; 0% vs. 39%). In the mean antibody titer, there were signficant differences between infants and children over 1 year old (A/H1N1; 19 times vs. 56 times, B; 8 times vs. 14 times). CONCLUSION: We consider that significant differences in antibody titers between infants and children over 1 year old were caused by the difference of dosage in influenza vaccines. To obtain protective levels of antibodies by influenza vaccines in infants, they must be inoculated with enough dosage.  相似文献   
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Recently, the serotonergic nervous system has been receiving attention as part of the blood pressure regulating mechanism of the central nervous system, and it has been postulated that the system may participate in the pathogenesis of hypertension. The purpose of this experiment is to investigate the mechanism of hemodynamic change after i.c.v. administration of 5-HT in SHR and in normotensive Wistar Rats (WR). MATERIALS AND METHODS: Twenty-week-old male SHR (n = 11) and age-matched WR (n = 14) were used. On the day before the experiments, the unilateral carotid artery and jugular vein were cannulated. Also, a cannula was inserted stereotaxically into the anterior horn of the lateral cerebral ventricle. Experiments were performed under the conscious and minimum restrained state. Experiment I: After observation of resting mean arterial pressure (MAP) and heart rate (HR) for 20 minutes, 5-HT (5 micrograms/5 microliter saline) was administrated i.c.v., and MAP and HR were observed for 90 minutes. Then, 200 micrograms of phenoxybenzamine (POB) was given from the jugular cannula. Thirty minutes after the POB administration when MAP was stabilized, 5 micrograms of 5-HT was again given i.c.v., and MAP and HR were recorded for 30 minutes. Experiment II: Plasma norepinephrine (PNE) was measured before and 2 minutes after 5-HT i.c.v. administration. The control sample (1.5 ml) was withdrawn at least 30 minutes before the 5-HT injection, and immediately after the blood sampling, the same amount of blood which was obtained from the age-matched donor rat of the same strain was transfused. RESULTS: Experiment I: Resting MAP was 136.4 +/- 5.1 mmHg in SHR and 99.1 +/- 3.0 mmHg in WR. I.c.v. administration of 5-HT elicited consistent pressor response in SHR and in WR.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   
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After 70 years with no confirmed autochthonous cases of dengue fever in Japan, 19 cases were reported during August–September 2014. Dengue virus serotype 1 was detected in 18 patients. Phylogenetic analysis of the envelope protein genome sequence from 3 patients revealed 100% identity with the strain from the first patient (2014) in Japan.  相似文献   
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Miliary tuberculosis is a potentially lethal type of tuberculosis that results from the hematogenous dissemination of Mycobacterium tuberculosis bacilli. We herein describe the case of a 34-year-old man that presented with a one-month history of cough and fever, while his sputum smear results were negative. Chest computed tomography revealed bilateral centrilobular ground-glass opacification (GGO), suggestive of hypersensitivity pneumonitis; thus, bronchoscopy was performed. Cryobiopsy specimens revealed necrotic granulomas. A re-examination of sputum after bronchoscopy identified Mycobacterium tuberculosis, and miliary tuberculosis was diagnosed. A cryobiopsy might be useful for diagnosing miliary tuberculosis pathologically, particularly when miliary nodules may be masked by GGO.  相似文献   
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Background Glycyrrhizin (GL), an aqueous extract of licorice root, is known to have various immune-modulating and biological response-modifier activities. GL is used in patients with hepatitis to reduce the activity of liver inflammation; however, the mechanism underlying the anti-inflammatory activity of GL is poorly understood. As antigen-presenting dendritic cells (DC) in the tissue play a major role in the regulation of the inflammatory mucosal milieu during tissue inflammation, we studied whether the function of liver DC was altered by GL therapy in a murine model of concanavalin-A (Con A)-induced hepatitis.Methods Liver DC were propagated from control mice or mice with Con-A-induced hepatitis, and the effect of GL on liver DC was evaluated in vivo and in vitro.Results The levels of interleukin (IL)-10 produced by liver DC were significantly lower in mice with Con-A-induced hepatitis compared with control mice. However, treatment with GL caused increased production of IL-10 in mice with Con A-induced hepatitis. The increased production of IL-10 by mice with Con A-induced hepatitis was also confirmed in vitro by culturing liver DC with GL.Conclusions This study indicates that increased production of IL-10 by liver DC due to GL administration may be involved in downregulation of the levels of liver inflammation in mice with Con A-induced hepatitis.  相似文献   
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