Mutations in XPD cause xeroderma pigmentosum (XP), XP and Cockayne syndrome (CS) crossover syndrome (XP/CS), trichothiodystrophy and cerebro-oculo-facio-skeletal syndrome (COFS). COFS represents the most severe end of the CS spectrum. This study reports two Japanese patients, COFS-05-135 and COFS-Chiba1, who died at ages of <1 year and exhibited typical COFS manifestations caused by XPD mutations p.
View Article and Find Full Text PDFRinsho Shinkeigaku
October 2015
Progressive multifocal leukoencephalopathy (PML) is a rare but fatal demyelinating disease of the central nervous system (CNS) caused by JC virus (JCV). The current diagnostic standard for PML is real-time PCR testing of extracted DNA for assessing the presence of JCV DNA in cerebrospinal fluid (CSF). However, because of its sensitivity, real-time PCR assay for JCV testing has a risk of false-positive results due to DNA contamination.
View Article and Find Full Text PDFRinsho Shinkeigaku
October 2015
Acyclovir (ACV), which inhibits the replication of herpes simplex virus, is the standard drug for the treatment of herpes simplex encephalitis. Thanks to the introduction of ACV, the morbidity and mortality of HSE patients have significantly improved. However, the disease is still the severe infection, because it makes some patients with HSE suffer from severe consequences.
View Article and Find Full Text PDFSevere fever with thrombocytopenia syndrome (SFTS) is an emerging infectious and hemorrhagic disease recently described in China and western Japan. A 71-year-old healthy Japanese woman noticed a tick biting her after harvesting in an orchard and removed it herself. She developed diarrhea, anorexia, and chills eight days later.
View Article and Find Full Text PDFInfection with a dengue virus (DENV) serotype induces cross-reactive, weakly neutralizing antibodies to different dengue serotypes. It has been postulated that cross-reactive antibodies form a virus-antibody immune complex and enhance DENV infection of Fc gamma receptor (FcγR)-bearing cells. We determined whether infectious DENV-antibody immune complex is formed in vivo in marmosets after passive transfer of DENV-specific monoclonal antibody (mAb) and DENV inoculation and whether infectious DENV-antibody immune complex is detectable using FcγR-expressing cells.
View Article and Find Full Text PDFSevere fever with thrombocytopenia syndrome (SFTS) is a disease with a high case fatality rate that is caused by infection with the recently identified tick-borne SFTS virus (SFTSV), for which there are no specific countermeasures. We examined the effects of ribavirin and mizoribine, which are nucleoside analogue drugs with broad antiviral activities, on SFTSV proliferation in vitro. When 3 cell lines were treated with these drugs before and during infection with a Chinese SFTSV strain, the 99% effective concentrations (EC99) of ribavirin were 19-64 μg/ml (78-262 μM); in contrast, the EC99 of mizoribine was >500 μg/ml (1,929 μM).
View Article and Find Full Text PDFHepatitis B surface antigen is widely used in hepatitis B virus surveillance; patients who test negative for the antigen are judged to be uninfected. However, occult hepatitis B virus infection has been confirmed with hepatitis B virus DNA at low levels in the liver and peripheral blood in patients positive for hepatitis B core antibody or hepatitis B surface antibody, even if they test negative for hepatitis B surface antigen. To investigate the prevalence of occult hepatitis B virus in hemodialysis patients, we performed cross-sectional analysis of 161 hemodialysis patients in two related institutions for hepatitis B surface antigen, hepatitis B core antibody, and hepatitis B surface antibody.
View Article and Find Full Text PDFBackground: Rift Valley fever (RVF) is endemic to the tropical regions of eastern and southern Africa. The seroprevalence of RVF was investigated among the human population in Borno State, Nigeria to determine the occurrence of the disease in the study area in comparison with that of Lassa fever and Crimean-Congo Hemorrhagic fever.
Methods: Recombinant nucleoprotein (rNP)-based IgG-ELISAs for the detection of serum antibodies against RVF virus (RVFV), Lassa fever virus (LASV), and Crimean-Congo hemorrhagic fever virus (CCHFV) were used to test human sera in Borno State, Nigeria.
We report two autopsy cases of severe fever with thrombocytopenia syndrome (SFTS) with a high fatality rate in aged Japanese patients. Both cases were caused by a tick-bite. The pathognomonic histological feature was necrotizing lymphadenitis of systemic lymphoid tissue with SFTS viruses and SFTSV-RNA copies.
View Article and Find Full Text PDFHuman monkeypox is a viral zoonosis caused by monkeypox virus, an orthopoxvirus (OPXV). The majority of human monkeypox cases have been reported in moist forested regions in West and Central Africa, particularly in the Democratic Republic of the Congo (DRC). In this study we investigated zoonotic OPXV infection among wild animals in Zambia, which shares a border with DRC, to assess the geographical distribution of OPXV.
View Article and Find Full Text PDFMammalian cells store excess fatty acids in the form of triglycerides within lipid droplets. The intracellular bacterium Orientia tsutsugamush is the causative agent of severe human rickettiosis. We found that O.
View Article and Find Full Text PDFProgressive multifocal leukoencephalopathy (PML) is a subacute demyelinating disease of the brain caused by the JC virus that occurs mainly in immunocompromised patients. The prognosis is very poor. As the lesion looks like non- specific leukoencephalopathy, making a diagnosis at the early stage is very difficult.
View Article and Find Full Text PDFThe pathogenesis of severe human monkeypox, which causes systemic and fulminant infections, is not clear. This study presents a case repot of fulminant monkeypox with bacterial sepsis after experimental infection with monkeypox virus in a cynomolgus monkey (Macaca fascicularis). In our previous study (Saijo et al.
View Article and Find Full Text PDFIn this study, we investigated the prevalence of genital Chlamydia trachomatis isolated in Japan using a high-resolution genotyping method, the multilocus VNTR analysis (MLVA)-ompA typing method. Seventeen serotypes of C. trachomatis standard strain (A-L3) and 44 clinical isolates were obtained from clinical settings.
View Article and Find Full Text PDFQuantitative PCR testing for JC virus (JCV) DNA in the cerebrospinal fluid (CSF) is one of the diagnostic standards for progressive multifocal leukoencephalopathy (PML). The present study was conducted to examine its reliability using CSF specimens that had been preserved with guanidine lysis buffers in commercial nucleic acid extraction kits under different conditions. When CSFs were mixed with guanidine buffers, JCV DNA levels were not statistically reduced even after storage for 1 month at room temperature or for 3 months at -80℃, compared with the control samples.
View Article and Find Full Text PDFA method for the determination of 12 statins [atorvastatin (ATOR), cerivastatin (CERI), fluvastatin (FLU), lovastatin (LO), lovastatin acid (LOA), mevastatin (ME), mevastatin acid (MEA), pitavastatin (PITA), pravastatin (PRA), rosuvastatin (ROSU), simvastatin (SIM), and simvastatin acid (SIMA)] in dietary supplements by ultra-performance liquid chromatography (UPLC) has been developed. Statins were ultrasonically extracted with 50% (v/v) methanol. Clean-up was performed using an Oasis MAX mini-cartridge column with methanol and methanol containing 0.
View Article and Find Full Text PDFSevere fever with thrombocytopenia syndrome (SFTS) is an emerging infectious disease with a high case fatality risk and is caused by the SFTS virus (SFTSV). A retrospective study conducted after the first identification of an SFTS patient in Japan revealed that SFTS is endemic to the region, and the virus exists indigenously in Japan. Since the nucleotide sequence of Japanese SFTSV strains contains considerable differences compared with that of Chinese strains, there is an urgent need to establish a sensitive and specific method capable of detecting the Chinese and Japanese strains of SFTSV.
View Article and Find Full Text PDFWe detected two viruses, Japanese encephalitis virus (JEV)/Kochi/01/2005 and Getah virus (GETV)/Kochi/01/2005 in the same culture supernatant obtained by inoculation of Vero cells with a swine serum sample and subsequent passaging of the supernatant in Vero cells. Phylogenetic analysis using the nucleotide sequences of the complete genome and the E2 region of GETV indicated that GETV/Kochi/01/2005 is most similar to a Mongolian strain. In contrast, a partial sequence of the nsP1 protein coding region of GETV/Kochi/01/2005 showed that it was similar to Japanese strains isolated in the 1980s.
View Article and Find Full Text PDFProgressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease caused by the JC polyomavirus (JCV). Most patients with PML after renal transplantation have had poor outcomes. We describe a patient with PML after renal transplantation who had a good response to the withdrawal of immunosuppressant therapy.
View Article and Find Full Text PDFUnlabelled: Several arenaviruses are known to cause viral hemorrhagic fever (VHF) in sub-Saharan Africa and South America, where VHF is a major public health and medical concern. The biosafety level 4 categorization of these arenaviruses restricts their use and has impeded biological studies, including therapeutic drug and/or vaccine development. Due to difficulties associated with handling live viruses, pseudotype viruses, which transiently bear arenavirus envelope proteins based on vesicular stomatitis virus (VSV) or retrovirus, have been developed as surrogate virus systems.
View Article and Find Full Text PDFA Japanese man suffered from acute respiratory tract infection after returning to Japan from Bali, Indonesia in 2007. Miyazaki-Bali/2007, a strain of the species of Nelson Bay orthoreovirus, was isolated from the patient's throat swab using Vero cells, in which syncytium formation was observed. This is the sixth report describing a patient with respiratory tract infection caused by an orthoreovirus classified to the species of Nelson Bay orthoreovirus.
View Article and Find Full Text PDFJC polyomavirus (JCV) is the causative agent of progressive multifocal leukoencephalopathy (PML), a fatal demyelinating disease. JCV isolates from PML patients have hypervariable mutations in the noncoding control region (NCCR) of the viral genome. Although nucleotide sequencing analysis of NCCR mutation is useful for the confirmation of PML diagnosis and basic studies examining JCV variants, it is often labor-intensive, time-consuming, and expensive.
View Article and Find Full Text PDFBackground: Progressive multifocal leukoencephalopathy (PML), a fatal demyelinating disease caused by JC virus (JCV), occurs mainly in immunocompromised patients. While JCV DNA is detected in the cerebrospinal fluid (CSF) from a certain proportion of patients suspected of having PML, JCV-negative patients may also develop brain lesions due to other infectious agents. This study assessed the prevalence of six herpesviruses in the CSF from patients diagnosed with or suspected of PML.
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