34 research outputs found

    Extending Bilingual WordNet via Hierarchical Word Translation Classification

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    PACLIC 23 / City University of Hong Kong / 3-5 December 200

    A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease

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    Multicentric Castleman disease (MCD) is a systemic lymphoproliferative disorder that can lead to mass lesions in various body parts, including the lungs, kidneys, and extranodal sites. Meanwhile, orbital Castleman disease is extremely rare. Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibroinflammatory disorder and is characterized by the formation of tumor-like lesions with lymphoplasmacytic infiltrates, which are enriched in IgG4-positive plasma cells and may present with a characteristic storiform pattern of fibrosis to variable degrees. In this study, we report a case of a 67-year-old Taiwanese man with a 7-year history of bilateral eyelid swelling and proptosis. Orbital magnetic resonance imaging revealed soft tissue lesions in the bilateral intraconal region, demonstrating strong enhancement in the lacrimal glands, and extension into the bilateral infraorbital foramen, suggesting an orbital lymphoproliferative disease. The histopathological results of the intraorbital tumor excision were suggestive of a plasma-cell-predominant mixed-cell variant of MCD. However, the patient also showed definitive signs of IgG4-RD, including lacrimal gland enlargement and histopathological results of plasmacytosis, fibrosis, and germinal centers, with an increased ratio of IgG4 cells and elevated serum IgG4 levels. This case suggests a potential interacting pathway between these two disease entities that needs further studies

    Clinical and Genetic Analysis of Peutz-Jeghers Syndrome Patients in Taiwan

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    Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder that is characterized by intestinal hamartomatous polyps and mucocutaneous pigmentation. Recently, germline mutations in the LKB1 gene have been reported to underlie PJS. The gene that encodes this serine/threonine kinase is located at chromosome 19p13.3. The aim of this study was to investigate the clinical and genetic characteristics of PJS patients in Taiwan. Methods: We searched the patient database of the National Taiwan University Hospital, a tertiary medical center in Taiwan, between January 1990 and November 2005. Patients' clinical information, demographic data, endoscopic pictures, and outcome were reviewed and analyzed. After obtaining informed consent, DNA and RNA were extracted from peripheral blood mononuclear cells and the LKB1 gene was sequenced. Results: A total of 14 unrelated patients who fulfilled the diagnostic criteria of PJS were included, and seven of them had genetic analysis performed. Mucocutaneous pigmentation was the most frequent presentation. Hamartomas occur most commonly in the small intestine (86%). Frequent abdominal complications include intussusception and gastrointestinal bleeding. Four germline mutations were found (57.1%). Three resulted in stop codons at codon 60, 162 (novel mutation), and 308. The fourth mutation was a missense mutation at codon 239 (novel mutation). Conclusion: Compared with other countries, PJS patients in Taiwan tended to have more extensive polyps in the gastrointestinal tract, with intussusception being the most common abdominal symptom. Mutations in the LKB1 gene were identified in 57% of the probands in Taiwan

    Lipopolysaccharide-induced Notch signaling activation through JNK-dependent pathway regulates inflammatory response

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    Abstract Background Notch and TLR pathways were found to act cooperatively to activate Notch target genes and to increase the production of TLR-induced cytokines in macrophages. However, the mechanism of LPS-induced Notch activation and its role in sepsis still remains unclear. Methods We analyzed the expression patterns of Notch components in a LPS-stimulated murine macrophage cell line using real-time PCR and western blotting. The role of DAPT, a gamma-secretase inhibitor that is known to be a potent Notch inhibitor, in LPS-induced cytokine release and experimental sepsis in mice was also explored. Student's t-test was used to analyze the difference between the two groups. Results We found that Notch signaling was activated after LPS stimulation. The expression of Jagged 1, a Notch ligand, induced by LPS occurred in a JNK-dependent manner. In addition, Notch target genes were upregulated by early Notch-independent activation followed by delayed Notch-dependent activation after LPS stimulation. Disruption of Notch signaling by DAPT attenuated the LPS-induced inflammatory responses, including vascular endothelial growth factor (VEGF) and high-mobility group box chromosomal protein 1 (HMGB1), both in vitro and in vivo and partially improved experimental sepsis survival. Conclusions These findings support the existence of a synergistic effect of Notch signaling and the LPS pathway both in vitro and in vivo. Therefore, in the future Notch inhibitors may be utilized as adjunctive agents for the treatment of sepsis syndrome.</p

    The incidence and factors of hip fractures and subsequent morbidity in Taiwan: An 11-year population-based cohort study.

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    Hip fractures are a major problem to elder population, but subsequent morbidity is unclear about environmental factors and socioeconomic conditions. The study aims to investigate the incidence of hip fractures treated by the surgery; to compare the sequelae and temporal trends of hip fractures; to evaluate the seasonal effects in the subsequent short-term and long-term morbidities after hip fractures. A cohort study design is conducted using national health research datasets between 2000 and 2010. The ICD-9-CM diagnostic codes were utilized to investigate the incidence of hip fractures and the corresponding treatments. Hierarchical modeling was used to analyze the factors associated with various types of hip fractures. The results indicated that females had a lower incidence in the 30-44 age group, but a significantly higher incidence than males among those aged 60 years or older (adjusted rates 232.1 vs. 100.3 per 100,000 persons, p<0.001). The incidence of hip fractures in the low-income group showed no significant difference compared to that in the general population. There was a temporal trend of a 8.6% increase in the incidence of all types of hip fractures over the period of 2000-2010. A summer-winter variation is observed among the elderly. Hip fractures and subsequent morbidity are increasing in Taiwan's aging society. Older age, female gender, and time periods were independent risk factors for subsequent morbidities after surgical treatment. The result of this study is useful to the healthcare policy makers and to raise the public awareness of hip fractures
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