Uptake of [3H]-fosfomycin into cells of enterohemorrhagic Escherichia coli O157 and E. coli non-O157.
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Escherichia coli↗
Biomedical subjects
H Ida
Publications and source records attributed to H Ida.
Severe skeletal complications in Japanese patients with type 1 Gaucher disease.
To better characterize skeletal complications in Japanese patients with type 1 Gaucher disease (GD), we performed genotyping and clinical and radiological analysis of 35 patients, the vast majority of this population, Skeletal complications tend to be very common, severe and rapidly progressive in Japanese patients with type 1 GD. Twenty (57%) of these patients manifested end points of severe bone disease including avascular necrosis, pathological fracture and/or bone crisis. Mean time from presentation/diagnosis of GD until presentation of this involvement was 3 years 6 months +/- 4 years 1 month. Prevalence of severe bone disease is significantly higher in splenectomized than in non-splenectomized patients--81% (17/21) versus 21% (3/14) (p = 0.0007, Fisher's exact test). Four (29%) of 14 patients receiving enzyme replacement therapy (ERT) or bone marrow transplantation (BMT) manifested severe bone involvement for the first time during or after treatment. All cases occurred in children in whom ERT doses had been lowered after only brief administration of higher starting doses (n = 3) or partial donor marrow engraftment resulted in low glucocerebrosidase (GCR) activity (n = 1). These observations suggest that splenectomy may correlate with accelerated skeletal deterioration with severe skeletal disease, at least in patients with severe phenotypic expression. They also suggest that it is important that sufficient GCR is available in paediatric patients with severe phenotypic expression. Hence ERT dosages should be based on disease severity and on age, with sustained administration of full doses in patients at greater risk of important skeletal complications.
Adolescent↗
Clinical and molecular characteristics of Japanese Gaucher disease.
Clinical signs and symptoms of Gaucher disease are more severe in Japanese than in Jewish and other non-Japanese patients. A higher percentage of bone crises and splenectomy was demonstrated by Japanese patients, and there were five fatalities among patients with type 1 Gaucher disease. Additionally, neonatal Gaucher disease, clinically characterized by hydrops foetalis, was observed. Japanese patients with type 2 and type 3 disease also demonstrate clinical heterogeneity. About 100 alleles of patients with Japanese Gaucher disease were examined for genotype determination with the PCR and SSCP methods. About 18 different mutations, including several novel mutations in Japanese patients, were identified. The most common mutations in Japanese patients were 1448C(L444P), accounting for 41 (41%) of alleles. The second most prevalent mutation was 754A(F2131), accounting for 14 (14%) of alleles. Other alleles identified included the 1324C, IVS2 and other mutations. Unidentified alleles comprised 16% of the total number of alleles studied. To date, neither the 1226G (N370S) nor the 84GG mutation has been identified in the Japanese population, although these mutations account for about 70% and 10% of the mutations in Jewish and other non-Japanese populations, respectively. The phenotype-genotype correlation in Japanese patients is more complex compared with that of the Jewish population. In Japanese patients, the 1448C mutation, in either heteroallelic or homoallelic forms, exhibits both neurological and non-neurological phenotypes. Japanese patients with the 754A mutation also exhibit both neuronopathic and non-neuronopathic disease. On the other hand, patients with the D409H mutation show only type 3 neurological disease, and those with the 1447-1466 del 20 ins TG mutation have the severe, neonatal neurological form of Gaucher disease. The 1503T allele was present only in patients with type 1 non-neurological disease. However, since this correlation was observed only in young patients, we do not as yet know the final phenotypic outcome of this mutation. Probably, Japanese patients with Gaucher disease have few mutations that exhibit non-neurological signs and symptoms.
Age of Onset↗
A DNA probe specific to Streptococcus sobrinus.
Three DNA fragments (SSB-1, -2 and -3) in the dextranase gene (dex) of Streptococcus sobrinus were amplified by polymerase chain reaction and used as DNA probes. The probes were examined for the specificity and the sensitivity of hybridization with DNA of oral streptococcal species. While probes SSB-1 and SSB-2 were specific to both S. sobrinus and Streptococcus downei, SSB-3 was specific only to S. sobrinus. SSB-3 was able to detect 5 ng of chromosomal DNA purified from S. sobrinus NIDR6715 and DNA extracted from 1 x 10(5) cells of the strain. In addition, SSB-3 could differentiate clinical isolates of S. sobrinus from Streptococcus mutans. These results suggest that SSB-3 is an effective DNA-probe to detect and to identify S. sobrinus.
Bacterial Proteins↗
Adult-type metachromatic leukodystrophy with a compound heterozygote mutation showing character change and dementia.
A 26-year-old Japanese woman slowly developed a change of character such as hypospontaneity and blunted affect, followed by obvious mental deterioration. She was diagnosed as having a disorganized type of schizophrenia at the first examination. Brain magnetic resonance imaging demonstrated diffuse high intensity in the cerebral white matter, particularly in the frontal lobes. The single photon emission computed tomography images using 123I-IMP disclosed diffuse cerebral hypofusion, especially in the frontal lobes. Electroencephalogram showed a moderate amount of 5-6Hz theta waves on the background of alpha activity. Nerve conduction velocities in the extremities were delayed. The level of leucocyte arylsulphatase was low. In the arylsulphatase A gene analysis, a compound heterozygote having the 99Gly-->Asp and 409Thr-->Ile mutations was confirmed. The patient was diagnosed as having metachromatic leukodystrophy. She gradually showed obvious dementing symptoms such as memory disturbance and disorientation. The characteristics of the psychiatric symptoms in the leukodystrophy are discussed.
Adult↗
Fracture and dislocation of snowboarder's elbow.
BACKGROUND: This study focuses on the analysis of snowboarding versus skiing injuries, especially fracture, dislocation, or both, of the elbow, based on 7 years of medical records and roentgenograms of patients injured at a ski-snowboard area, Mt. Zao National Park, and demonstrates the precise characteristics of snowboard injury in the elbow region. METHODS: A retrospective study of 1,445 injured snowboarders and 10,152 injured skiers was undertaken to assess both snowboarding and skiing injuries. Sixty-four cases of snowboarding injuries and 152 cases of skiing injuries were available for precise analysis of fracture, dislocation, or both, in the elbow region. RESULTS: Fractures, dislocations, or both, in the elbow were more frequently observed for snowboarders (30 of 64 cases, 46.9%) when compared with that for skiers (26 of 152 cases, 17.1%) (p < 0.001). The rate of dislocation with or without fracture of the elbow was also significantly higher for snowboarders (17 of 64 cases, 26.6%) than for skiers (8 of 152 cases, 5.3%, p < 0.001). Seventeen cases of elbow dislocation in snowboarding were all of the posterior type, which accompanied two coronoid process fractures and two radial neck fractures. Fractures of the coronoid process (five cases), radial head (one case), radial neck (five cases), olecranon (one case), proximal ulnar shaft (one case), and extension-type fracture of distal humerus (four cases) were the fracture types observed in the analysis. CONCLUSION: Posterior dislocation; fractures of coronoid process, radial neck, and radial head; and extension-type fracture of the distal humerus characterize the particular and frequent injury mechanism responsible for snowboarding trauma in the elbow region. Thus, snowboarding injury of the elbow is recognized as a severe injury and is characterized by a frequent risk of posterior dislocation, fracture, or both. The severity of elbow injuries in snowboarding mainly seems to be due to direct mechanical force on the elbow, receiving the full impact of falling down, combined with an outstretched hand and elbow extension, or with an outstretched hand and longitudinal thrust force, to the proximal radius and ulna and distal humerus.
Adolescent↗
Analysis of 16 different matrix metalloproteinases (MMP-1 to MMP-20) in the synovial membrane: different profiles in trauma and rheumatoid arthritis.
OBJECTIVE: To define the pattern of mRNA expression of all human matrix metalloproteinases (MMPs) described to date in rheumatoid arthritis (RA) and traumatic synovial membrane, in order to differentiate between a physiological tissue remodelling pattern and that associated with inflammatory tissue destruction. METHODS: Analysis of SwissProt protein and EMBL/GenBank nucleotide sequence banks, protein sequence alignment, reverse transcriptase-polymerase chain reaction and nucleotide sequencing were used. RESULTS: MMP-2 (gelatinase A), MMP-3 (stromelysin-1), MMP-11 (stromelysin-3) and MMP-19 were constitutively expressed. MMP-1 (fibroblast type collagenase), MMP-9 (gelatinase B) and MMP-14 (MT1-MMP) were expressed in all RA, but only in 55-80% of trauma samples. MMP-13 (collagenase-3) and MMP-15 (MT2-MMP) were expressed exclusively in RA (80-90% of the samples). MMP-20 (enamelysin) was absent and MMP-8 (collagenase-2) was rarely found in RA or trauma. All other MMPs (-7, -10, -12, -16, -17) had an intermediate pattern of expression. CONCLUSIONS: Some MMPs without interstitial collagenase activity seem to have a constitutive pattern of expression and probably participate in physiological synovial tissue remodelling. Some MMPs are exclusively associated to RA synovitis, for example, MMP-13, which preferentially degrades type II collagen and aggrecan, and MMP-15, which activates proMMP-2 and proMMP-13 and is involved in tumour necrosis factor alpha processing. This clear cut rheumatoid/inflammatory MMP profile, more complex than has been previously appreciated, may facilitate inflammatory tissue destruction in RA.
Adult↗
Insulin-like growth factor I stimulates proliferation and Fas-mediated apoptosis of human osteoblasts.
In vitro studies have shown that insulin-like growth factor I (IGF-I) is a potent growth factor for osteoblasts, although both bone formation and resorption are upregulated by IGF-I in vivo. To understand the difference in the action of IGF-I observed in vitro and in vivo experiments, we examined the effect of IGF-I on the proliferation and Fas-mediated apoptosis of human osteoblasts in vitro. Human osteoblastic cell line MG63 and human primary osteoblast-like cells obtained from biopsy specimens were used as human osteoblasts. Cells were cultured with or without various concentrations of IGF-I followed by determination of the proliferative response and Fas-mediated apoptosis. IGF-I dose dependently stimulated the proliferation of cultured human osteoblasts. Both Fas expression and the degree of anti-Fas IgM-induced apoptosis of human osteoblasts was also augmented by IGF-I. Furthermore, the cytotoxicity of Fas ligand (FasL) cDNA transformants against human osteoblasts was increased when IGF-I-stimulated osteoblasts were used as target cells, indicating that stimulation of IGF-I increased functional Fas expression on human osteoblasts as well as their proliferation. The addition of DEVD-CHO, a specific inhibitor of CPP32, to the culture resulted in a significant inhibition of Fas-mediated apoptosis of both unstimulated and IGF-I-stimulated osteoblasts, although it did not affect the proliferative response or Fas expression. Our data suggest that activation of CPP32 is necessary for Fas-mediated apoptosis of human osteoblasts, and treatment of IGF-I increased this signaling pathway. In contrast, regulation of proliferation and Fas expression of the cells were probably not affected by CPP32 activation. Our results suggest that IGF-I acts on cultured human osteoblasts by increasing their proliferation and induction of Fas-mediated apoptosis by neighbouring FasL+ cells such as osteoclasts, thus probably functioning as a local coupling factor in the bone in vivo, stimulating both bone formation and resorption.
Apoptosis↗
Activation-induced NK cell death triggered by CD2 stimulation.
Both T cells and natural killer (NK) cells express CD2, the target of an alternative activation pathway that induces the proliferation of both cell types. The mitogenic response to CD2 ligation requires the co-expression of CD3:TCR in T cells and FcgammaRIII in NK cells, suggesting that these receptors are involved in transducing the response initiated by CD2. The ability of FcgammaRIII to trigger the activation-induced death of IL-2-primed NK cells led us to investigate the potential for CD2 to trigger activation-induced NK cell death. Our results reveal that the same anti-CD2 monoclonal antibodies (mAb) that activate freshly isolated NK cells induce apoptosis in IL-2-primed NK cells. CD2-induced apoptosis results in chromatin condensation, DNA fragmentation and cleavage of caspase-3. Activation-induced NK cell death triggered by CD2 ligation is extremely rapid (DNA fragmentation is first observed at 90 min) and it is not inhibited by neutralizing antibodies reactive with TNF-alpha or Fas ligand. Whereas mAb reactive with distinct CD2 epitopes (i.e. T11.1, T11.2, and T11.3) are required for activation-induced T cell death, mAb reactive with a single CD2 epitope are sufficient for activation-induced NK cell death. The ability of CD2, CD16, and CD94 to induce apoptosis in IL-2-primed lymphocytes suggests that cytokine priming changes the response to a signaling cascade that is common to each of these activation receptors.
Apoptosis↗
Type 1 Gaucher disease: phenotypic expression and natural history in Japanese patients.
Gaucher disease is caused by a deficiency of glucocerebrosidase, resulting in hepatosplenomegaly, pancytopenia, growth retardation and skeletal involvement. We analyzed data on genotype and key clinical parameters in 35 Japanese patients with Gaucher disease type 1. Our data demonstrated that over 60% of patients had onset of Gaucher disease signs/symptoms at less than 5 years. Sixty percent and 46% of evaluable patients were splenectomized and developed severe bone involvement, respectively. Within mean follow-up periods of 8 years and 4 months, mean relative height and weight, severity score index and platelet count all worsened to a highly significant degree. These data suggest that type 1 Gaucher disease tends to be severe and progressive in Japanese patients, most of whom would be suitable for treatment and might indeed require earlier and more aggressive therapy.
Adult↗
Prevalence of arylsulphatase A mutations in 11 Japanese patients with metachromatic leukodystrophy: identification of two novel mutations.
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Cerebroside-Sulfatase↗
An autopsy case of fetal Gaucher disease.
BACKGROUND: A case of fetal form of Gaucher disease in a Japanese fetus is presented. RESULTS: A macerated baby showing hydrops fetalis was dissected at 29 weeks of gestation. The fetus was heavier in the body, liver and spleen than a normal fetus at the same gestation period. It also suffered from pericardial effusion and ascites. The diagnosis of Gaucher disease was made by histological and biochemical findings. In microscopical examinations 'Gaucher cells', which were periodic acid-Shiff (PAS)-positive, alcian blue-positive and CD68-positive, existed in the lungs, liver, spleen, thymus, adrenal glands, bone marrow and brain. In thin layer chromatography, a large quantity of glucocerebroside was seen to have accumulated in the patient's organs.
Adult↗
Antibiotic susceptibility of enterohemorrhagic Escherichia coli O157:H7 isolated from an outbreak in Japan in 1996.
The antibiotic susceptibilities of 43 strains of Escherichia coli O157:H7 identified in the summer of 1996 in Japan were investigated. Growth of 90% of O157 strains was inhibited at a concentration of < or = 0.5 micro/ml by several agents including fosfomycin with glucose-6-phosphate.
Anti-Bacterial Agents↗
Enzyme therapy in Gaucher disease type 2: an autopsy case.
A Japanese patient with Gaucher disease type 2 was treated with enzyme therapy, alglucerase, from 7 to 22 months of age. Whereas hematologic parameters were normalized and hepatosplenomegaly was alleviated, no improvement in neurologic symptoms occurred, and the patient died of respiratory failure at age 22 months. Postmortem examination revealed massive intra-alveolar infiltration of Gaucher cells in lungs and in the central nervous system, i.e., the presence of Gaucher cells in the perivascular Virchow-Robins spaces in the cortex and deep white matter and extensive lamilar necrosis with reactive proliferation of blood vessels and macrophage infiltration of the cerebral cortex. It is suggested that enzyme therapy, with thus far recommended dose, does not prevent long-term respiratory and central nervous system involvement in severe varients of Gaucher disease.
Brain↗
[Gaucher disease].
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Bone Marrow Transplantation↗
The membrane-type-matrix metalloproteinase/matrix metalloproteinase-2/tissue inhibitor of metalloproteinase-2 system in periprosthetic connective-tissue remodeling in loose total-hip prostheses.
The aim of the present study was to investigate the proteolytic potential and localization of matrix metalloproteinase (MMP)-2 in relation to its regulatory protein, membrane-type-MMP (MT1-MMP), and tissue inhibitor of metalloproteinase-2 (TIMP-2), as well as to clarify an important step in the cascade of periprosthetic connective-tissue remodeling in loose total-hip prostheses. Immunohistochemical analysis revealed increased expression of MT1-MMP, MMP-2, and TIMP-2 in fibroblasts, synovial lining-like cells, and endothelial cells, as well as, to some extent, in monocyte/macrophage-like cells in both tissues from the bone-implant interface and reactive cellular tissues from regenerating capsules in loose hip joints, when compared with control fibrous tissues between bone and implants retrieved from unloosened hip joints. In loose hip joints, reverse transcription-PCR analysis showed the presence of MT1-MMP, MMP-2, and TIMP-2 mRNA in both the bone-implant interface and regenerating capsular tissues. Increased protein levels of MMP-2 and TIMP-2 were also demonstrated by an ELISA, and those of MT1-MMP were shown by immunoblot analysis. Gelatin-zymographic analysis confirmed the presence of both pro- and active forms of MMP-2, which suggested the in situ activation of MMP-2 by MT1-MMP in the loose hip joints. Collectively, these data suggest that the MT1-MMP/MMP-2/TIMP-2 system participates in the extracellular matrix degradation and periprosthetic connective-tissue remodeling in loose hip joints, and may thus contribute to the periprosthetic weakening, loosening, and osteolysis that can occur around implants.
Aged↗
Matrix metalloproteinases and tissue inhibitors of metalloproteinases in loose artificial hip joints.
The role of extracellular matrix metalloproteinase enzymes and the tissue inhibitors of metalloproteinase in the periprostetic connective tissue matrix of loose artificial hip joints is reviewed. In the periprosthetic granulomatous interface connective tissues between bone and implants and inner cellular regenerating pseudocapsular tissues, matrix metalloproteinase 1, matrix metalloproteinase 2, matrix metalloproteinase 3, matrix metalloproteinase-9, and membrane type 1 matrix metalloproteinase enzymes can be shown in the light of immunohistochemistry, enzyme activity analysis, and messenger ribonucleic acid levels. Tissue inhibitors of metalloproteinase 1 and tissue inhibitors of metalloproteinase 2 also are found in the corresponding tissues. Analysis of matrix metalloproteinase and tissue inhibitors of metalloproteinase interaction shows imbalance between the enzymes and the endogenous inhibitors in favor of matrix metalloproteinase. This induces pathologic connective tissue remodeling in the interface and pseudocapsule. The data suggest that matrix metalloproteinase and tissue inhibitors of metalloproteinase system participate in the extracellular matrix degradation and tissue remodeling in artificial hip joints, and may contribute to the periprosthetic weakening, implant loosening, and osteolysis around implants. More evidence for their active involvement is sought by intervention studies with type specific matrix metalloproteinase inhibitors.
Animals↗
Appearance of fosfomycin resistant Rahnella aquatilis clinically isolated in Japan.
Among recent clinical isolates in Japan, strain CU264 was discovered which formed unusual colonies. This strain was identified as Rahnella aquatilis which is usually found in water. The antibiotic susceptibilities against tetracycline, carbenicillin, chloramphenicol, streptomycin, kanamycin, gentamicin, sulphonamide, neomycin, fosfomycin, rifampicin, norfloxacin and nalidixic acid, were investigated. The result demonstrated that the strain was highly resistant to fosfomycin only. It was further shown that this resistance was transmissible with low frequency to Serratia marcescens whereas it was not transmissible to Escherichia coli.
Anti-Bacterial Agents↗