25 research outputs found

    The Outcome of Cochlear Implantation for Mitochondrial Disease Patients With Syndromic Hearing Loss.

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    【Objectives】To evaluate the outcome and to confirm the validity of cochlear implantation for syndromic deafness in patients with mitochondrial disease. Study Design: Retrospective case review. 【Setting】Tertiary referral center. 【Patients】We reviewed medical charts of 367 cochlear implantation cases at Kyoto University Hospital between 1987 and 2012. We identified five patients with syndromic mitochondrial disease who underwent cochlear implantation surgery. The mean age of the patients (four women and one man) when they underwent surgeries was 44.4 years (range, 30–64 yr; median, 41 yr). 【Interventions】Therapeutic and rehabilitative. 【Main Outcome Measure】In four of five patients, speech perception performance was measured using Japanese vowels, consonant-vowel syllables, and short sentences. 【Results】Only 1.4% (5 of 367) of cochlear implantation cases at Kyoto University Hospital underwent cochlear implantation surgery because of syndromic mitochondrial diseases. Four of those patients showed significantly improved speech perception outcomes, and the beneficial effects of the intervention continued long after surgery. One patient could not perform speech perception test presumably because of poor cognitive function. 【Conclusion】Mitochondrial disease patients who underwent cochlear implantation surgery sustained gains in hearing performance even long after surgery. A single patient showed poor postoperative speech perception associated with cognitive problems. Cochlear implantation for mitochondrial disease patients seems to be a viable treatment option in the absence of significant cognitive impairment

    A Case of Mycobacterial Skin Disease Caused by Mycobacterium peregrinum, and a Review of Cutaneous Infection

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    An 83-year-old Japanese man presented with a 2-month history of symptomatic nodules on the left hand. He was not in an immunocompromised condition and reported no causal events. A biopsy specimen demonstrated granulomatous tissue with mixed cell infiltration consisting of neutrophils, histiocytes, lymphocytes, and multinuclear giant cells. No bacillus was detected by PAS, acid-fast stain, immunofluorescent stain or polymerase chain reaction analysis. The isolate was found to be a rapidly growing mycobacterium after 4 weeks of incubation at 25°C on an Ogawa egg slant. Mycobacterium peregrinum was isolated by DNA-DNA hybridization analysis, 16S rRNA gene sequence, and by its production of 3-day arylsulfatase. The patient received 200 mg oral minocycline for 28 weeks. The lesion disappeared after 10 weeks of this treatment

    8-Prenylnaringenin tissue distribution and pharmacokinetics in mice and its binding to human serum albumin and cellular uptake in human embryonic kidney cells

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    8-Prenylnaringenin (8-PN), a hop flavonoid, is a promising food substance with health benefits. Compared with nonprenylated naringenin, 8-PN exhibits stronger estrogenic activity and prevents muscle atrophy. Moreover, 8-PN prevents hot flushes and bone loss. Considering that prenylation reportedly improves the bioavailability of flavonoids, we compared the parameters related to the bioavailability [pharmacokinetics and tissue distribution in C57/BL6 mice, binding affinity to human serum albumin (HSA), and cellular uptake in HEK293 cells] of 8-PN and its mother (non-prenylated) compound naringenin. C57/BL6 mice were fed an 8-PN or naringenin mixed diet for 22 days. The amount of 8-PN (nmol/g tissue) in the kidneys (16.8 ± 9.20), liver (14.8 ± 2.58), muscles (3.33 ± 0.60), lungs (2.07 ± 0.68), pancreas (1.80 ± 0.38), heart (1.71 ± 0.27), spleen (1.36 ± 0.29), and brain (0.31 ± 0.09) was higher than that of naringenin. A pharmacokinetic study in mice demonstrated that the Cmax of 8-PN (50 mg/kg body weight) was lower than that of naringenin; however, the plasma concentration of 8-PN 8 h after ingestion was higher than that of naringenin. The binding affinity of 8-PN to HSA and cellular uptake in HEK293 cells were higher than those of naringenin. 8-PN bioavailability features assessed in mouse or human model experiments were obviously different from those of naringenin

    Possible Biosynthetic Pathways for all cis-3,6,9,12,15,19,22,25,28-Hentriacontanonaene in Bacteria

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    A very long chain polyunsaturated hydrocarbon, hentriacontanonaene (C31:9), was detected in an eicosapentaenoic acid (EPA)-producing marine bacterium, which was isolated from the mid-latitude seashore of Hokkaido, Japan, and was tentatively identified as mesophilic Shewanella sp. strain osh08 from 16S rRNA gene sequencing. The geometry and position of the double bonds in this compound were determined physicochemically to be all cis at positions 3, 6, 9, 12, 15, 19, 22, 25, and 28. Although C31:9 was detected in all of the seven EPA- or/and docosahexaenoic acid-producing bacteria tested, an EPA-deficient mutant (strain IK-1Δ8) of one of these bacteria had no C31:9. Strain IK-1Δ8 had defects in the pfaD gene, one of the five pfa genes responsible for the biosynthesis of EPA. Although Escherichia coli DH5α does not produce EPA or DHA inherently, cells transformed with the pfa genes responsible for the biosynthesis of EPA and DHA produced EPA and DHA, respectively, but not C31:9. These results suggest that the Pfa protein complex is involved in the biosynthesis of C31:9 and that pfa genes must not be the only genes responsible for the formation of C31:9. In this report, we determined for the first time the molecular structure of the C31:9 and discuss the possible biosynthetic pathways of this compound

    The Role of Interregional Trade in Generating Change in the Regional Economies of Japan, 1980–1990

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    An earlier analysis (Hewings et al., 1998) revealed the 'hollowing-out' process, a decrease in the level of intraregional intermediation, in the Chicago economy during the period 1975-2011. The main force underlying this structural change has been the change in regional trade patterns in a way that interregional trades across economies has replaced the local purchases of intermediate inputs. The issue addressed in this paper focuses on the decomposition of structural change into changes in interregional trade and in technology, in order to investigate the nature of structural change over time and across sectors. The empirical realization is provided by reference to a series of regional input-output tables for a nine-region division of the Japanese economy (1980-1985-1990). The results revealed that interregional trade has played a key role in determining regional output level while technology itself had a tendency to decrease further.Coefficient Change, Interregional Trade, Decomposition, Japan,

    Isolation and Characterization of a Novel Thraustochytrid-like Microorganism that Efficiently Produces Docosahexaenoic Acid

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    A thraustochytrid-like microorganism (strain 12B) was isolated from the mangrove area of Okinawa, Japan. On the basis of its ectoplasmic net structure and biflagellate zoospores we determined strain 12B to be a novel member of the phylum Labyrinthulomycota in the kingdom Protoctista. When grown on glucose/seawater at 28 °C, it had a lipid content of 58% with docosahexaenoic acid (DHA; 22:6 n−3) at 43% of the total fatty acids. It had a growth rate of 0.38 h(−1). The DHA production rate of 2.8 ± 0.7 gl(−1) day(−1) is the highest value reported for any microorganism

    Calciphylaxis in POEMS Syndrome: A Case Treated with Etidronate

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    ArticleACTA DERMATO-VENEREOLOGICA.91(1):98-99(2011)journal articl
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