1.The efficacy of SPA0355 in protecting beta cells in isolated pancreatic islets and in a murine experimental model of type 1 diabetes.
Ui Jin BAE ; Mi Young SONG ; Hyun Young JANG ; Hyo Jin GIM ; Jae Ha RYU ; Sang Myeong LEE ; Raok JEON ; Byung Hyun PARK
Experimental & Molecular Medicine 2013;45(11):e51-
Cytokines activate several inflammatory signals that mediate beta-cell destruction. We recently determined that SPA0355 is a strong anti-inflammatory compound, thus reporting its efficacy in protecting beta cells from various insults. The effects of SPA0355 on beta-cell survival were studied in RINm5F cells and primary islets. The protective effects of this compound on the development of type 1 diabetes were evaluated in non-obese diabetic (NOD) mice. SPA0355 completely prevented cytokine-induced nitric oxide synthase (iNOS) expression and cytotoxicity in RINm5F cells and isolated islets. The molecular mechanism of SPA0355 inhibition of iNOS expression involves the inhibition of nuclear factor kappaB and Janus kinase signal transducer and activator of transcription pathways. The protective effects of SPA0355 against cytokine toxicity were further demonstrated by normal insulin secretion and absence of apoptosis of cytokine-treated islets. In experiments with NOD mice, the occurrence of diabetes was efficiently reduced when the mice were treated with SPA0355. Therefore, SPA0355 might be a valuable treatment option that delays the destruction of pancreatic beta cells in type 1 diabetes.
Animals
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Apoptosis
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Benzoxazines/pharmacology/*therapeutic use
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Cell Line
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Cell Survival
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Cells, Cultured
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Diabetes Mellitus, Experimental/*prevention & control
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Insulin-Secreting Cells/*drug effects/metabolism
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Janus Kinases/genetics/metabolism
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Mice
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Mice, Inbred NOD
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NF-kappa B/genetics/metabolism
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Nitric Oxide Synthase Type II/genetics/metabolism
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Rats
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Thiourea/*analogs & derivatives/pharmacology/therapeutic use
2.A Multicenter Study on von Willebrand Disease Realities in Yeungnam Region
Hyun Ju KIM ; Ye Jee SHIM ; Jae Min LEE ; Young Tak LIM ; Eu Jeen YANG ; Kyung Mi PARK ; Hee Won CHUEH ; Eun Sil PARK ; Hyo Sun KIM ; Ji Kyoung PARK ; Eun Jin CHOI ; Seom Gim KONG ; Ji Yoon KIM ; Sang Kyu PARK
Clinical Pediatric Hematology-Oncology 2019;26(1):46-54
BACKGROUND: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. METHODS: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. RESULTS: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). CONCLUSION: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.
Adult
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Child
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Diagnosis
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Hemophilia A
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Hemorrhage
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Hospitals, University
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Humans
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Korea
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Medical Records
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Prevalence
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Retrospective Studies
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von Willebrand Diseases