• Title/Summary/Keyword: GSD

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Antitumor Effect of the Cotreatment of Gagamsibjeondaebo-tang and $As_2O_3$ in Human Lung Cancer Cell Line H-460 (인간 폐암세포주 H-460 세포에서 가감십전대보탕과 $As_2O_3$의 병용처리에 의한 항종양 증진효과)

  • Hur Jong Chan;Won Jin Hee;Kim Dong Woung;Han Se Hee;Moon Goo
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.18 no.4
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    • pp.1089-1097
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    • 2004
  • This study was designed to elucidate the synergistic cytotoxic mechanisms of the cotreatment of Gagamsibjeondaebo-tang (GSD) and AS₂O₃ in human lung cancer cell line, H-460. The combination of GSD and AS₂O₃ synergistically augmented the cytotoxicity of GSD and AS₂O₃ in H-460 cells. The nature of cytotoxicity was revealed as apoptosis which was characterized by chromatin condensation and fragmentaton in DAPI staining. The apoptotic cytotoxicity of GSD and AS₂O₃ was accompanied by the cleavage of PARP. Of note, the expression of pro-apoptotic BclXS protein was increased, but the expressions of Sax and BclXL was not affected in H-460 cells treated with GSD and AS₂O₃. In addition, antioxidant NAC completely blocked the apoptotic death of H-460 cells by GSD and AS₂O₃. In conclusion, this results suggest that the cotreatment of GSD and AS203 induces the synergistic apoptotis of human large cell lung cancer cell line, H-460 via the induction of BclXS and reactive oxygen species (ROS).

Does Type I Truly Dominate Hepatic Glycogen Storage Diseases in Korea?: A Single Center Study

  • Jeong, Yu Ju;Kang, Ben;Choi, So Yoon;Ki, Chang-Seok;Lee, Soo-Youn;Park, Hyung-Doo;Choe, Yon Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.17 no.4
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    • pp.239-247
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    • 2014
  • Purpose: There are no studies of hepatic glycogen storage diseases (GSDs) other than type I and III in Korea. We aimed on investigating the characteristics of hepatic GSDs in Korea diagnosed and followed at a single center. Methods: We retrospectively analyzed patients who were diagnosed as GSD and followed at Samsung Medical Center from January, 1997 to December, 2013. Clinical manifestations, laboratory results, treatment, and prognosis were investigated. Results: Twenty-one patients were included in the study. The types of 17 patients were confirmed by enzyme activity tests and/or gene analysis. GSD Ia was diagnosed in 7 patients (33.3%), Ib in 1 patient (4.8%), III in 2 patients (9.5%), IV in 1 patient (4.8%), and IX in 6 patients (28.6%). Types other than GSD I constituted 52.9% (9/17) of the patients diagnosed with a specific type of hepatic GSD. The median age at presentation was 2 years. Hepatomegaly was observed in 95.2%, elevated liver transaminases in 90.5%, and hyperlactacidemia in 81.0% of the patients. The duration for follow-up was $77{\pm}62.0$ months. Uncooked corn starch was initiated in all the patients. No mortality was observed during the follow-up period, and liver transplantation was performed in 14.3%. Conclusion: Types other than GSD I comprised more than half of the patients diagnosed with a specific type of hepatic GSD. Clinical suspicion and thorough evaluation of hepatic GSDs in Korea should be focused not only on GSD I, but also on other types.

Effects of Gamisinsunbulo-dan on Learning and Memory Function in the Dementia Rat by Ibotenic acid Damage (가미신선부노단이 ibotenic acid손상에 의해 유도된 치매 백서의 학습 및 기억장애에 미치는 영향)

  • Eom Hyun Sup
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.16 no.6
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    • pp.1151-1156
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    • 2002
  • This research was done to make the effective prescription and cope with various senile dementia. Sprague-Dawley rats were injured by ibotenic acid to make a damage on learning and memory functions of model rats. At first acquisition test and retention rest were done in the Morris water maze. And to evaluate the effects of the sample drug(GSD) on choline acetyltranferase and acetylcholine esterase, immunoreactive measurement and enzymatic activity measuring were carried out. The ibotenic acid were injected to hippocampus CA1 and CA3 area. Conclusion : GSD improved the learning ability in the acquisition test and memory function in the retention test significantly. And GSD increased the level of ChAT which is synthesizing acetylcholine in CA1 area, and at the same time it increased the level of AChE which is resolving acetylcholine. These results show that GSD improved the cholinergic catabolism and anabolism, and the increment of metabolic activity of cholinergic system. In other words, it contributes to the recovery of damaged learning and memory function by ibotenic acid. So it can be concluded that GSD will be helpful to cholinergic brain damage induced by primary or senile reduction of acetylcholine secretive activity.

Monsoonal sediment transport along the subaqueous Mekong Delta: An analysis of surface sediment grain-size changes

  • Thanh C., Nguyen;An T., Dang;Khuong N.T., Tran
    • Ocean Systems Engineering
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    • v.12 no.4
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    • pp.403-411
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    • 2022
  • Annually, about 48-60% of sediment discharge of the Mekong River is delivered near the mouths of the Mekong River branches which is mostly coinciding with the southwest (SW) monsoon. This sediment budget in turn will be southwestwardly transported along the coast of the Mekong Delta (MD) during the northeast (NE) monsoon. Analysis of monsoonal changes in grain-size distribution (GSD) of surface sediment contributes to a better understanding of erosion and deposition processes along the MD. This study aims to figure out changes in GSD and sediment textures along the MD between SW and NE monsoons based on 183 surficial sediment samples collected along the MD during two field surveys carried-out in October 2016 and February-March 2017. Compared to the GSD during the SW and NE monsoon, the GSD along the MD changed significantly, especially in the estuary areas and along the coast of Bac Lieu and Ganh Hao. Whereas, in the west coast of the MD, GSD seem no changes between the two seasons. These changes in seabed sediment suggest that sediment with grain-sizes ranging from silt to fine sand can be transported during only a NE season.

The Correlation Analysis between Satellite Image Quality and Application Performance (위성임무에 따른 성능지표 간 중요도 결정을 위한 위성영상용 성능지표와 응용분야 성능지표의 상관관계 분석)

  • Kim, Dong-Wook;Kim, Tae-Jung;Kim, Hee-Seob
    • Proceedings of the KSRS Conference
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    • 2009.03a
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    • pp.175-180
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    • 2009
  • 인공위성에 탑재될 영상 시스템에는 영상의 품질을 결정하는 여러 성능지표가 고려된다. 이러한 성능지표는 위성 서비스와 관련된 여러 응용분야의 지표들과 밀접한 상관관계를 가지고 있으며, 각 응용분야의 지표에 따라 그 중요도에 차이점을 두고 있다. 일반적으로, 위성 설계를 위한 성능지표로는 GSD(Ground Sample Distance), MTF(Modulation Transfer Function), SNR(Signal to Noise Ratio)등과 같은 전자광학적 성능지표가 사용되고 있다. 본 논문에서는 이러한 위성설계상의 성능지표와 응용분야 지표간의 상관관계 분석을 위해서 GSD, MTF, SNR과 같은 위성 설계용 성능지표가 반영된 가상영상을 생성한 후 이를 영상의 응용분야인 판독도 분야와 정밀지형정보 분야에 적용하여 위성설계용 성능지표가 응용분야의 성능지표에 미치는 영향에 대해서 분석을 하였다. 전체 실험에 대한 분석결과 판독도와 관련된 응용지표는 MTF, GSD, SNR에 영향을 받는 것으로 나타났고, 정밀지형정보 분야와 관련된 응용지표는 SNR과 MTF에 민감하게 반응하는 것으로 나타났다.

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Anesthetic management for dental surgery in a child with glycogen storage disease type IIIa: a case report

  • Bugra, Aykenar;Nedim, Cekmen
    • Journal of Dental Anesthesia and Pain Medicine
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    • v.22 no.6
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    • pp.451-455
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    • 2022
  • Glycogen storage disease (GSD) is a group of inherited disorders, which result in the deficiency of enzymes involved in glycogen metabolism, leading to an accumulation of glycogen in various organs. Deficiency of amylo-1-6-glicosidase (debranching enzyme) causes glycogen storage disease type III (GSD III). The main problems that anesthesiologists face in patients with GSD III include hypoglycemia, muscle weakness, delayed awakening due to abnormal liver function, possible difficulty in airway, and cardiomyopathy. In the face of these difficulties, airway preparation and appropriate glucose monitoring and support during the fasting period are important. The doses of the drugs to be used should be calculated considering the increased volume of distribution and decreased metabolic activity of the liver. We present the case of a child with GSD IIIa who underwent dental prosedation under general anesthesia. She was also being prepared for liver transplantation. This case was additionally complicated by the patient's serious allergic reaction to eggs and milk.

Effects of Resistant Starch on Metabolism of Beile Acids in College Women (효소저항저분이 인체내 담즙산 대사에 미치는 영향)

  • 김지현;최인선;박소앙;신말식;오승호
    • Journal of Nutrition and Health
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    • v.33 no.8
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    • pp.802-812
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    • 2000
  • The purpose of this study was to examine the effect of resistant starch(RS) in hyperchlesterolemia and colon cancer. The subjects of this study was eight college women participating in the general starch diet(GSD) period for 5 days and resistant starch diet(RSD) period for 7 days. RSD contains 30g or the RS. On the last day of each program blood were collected. And for the last 3 days of each diet period, the amount of all the food consumed by the subjects and feces were collected. Food was measured to determine and compared the energy, protein and fat intakes. The amount of total cholesterol, HDL-cholesterol, LDL-cholesterol and volatile fatty acids in plasma and the amounts of bile acids in feces were measured by gas chromatography. The results obtained were as follows, Daily energy intake was higher in the RSD compared with the GSD, Protein and fat intakes were lower in the RSD compared with the compared with the GSD. Volatile fatty acid contents in plasma, the amounts of acetic acid, propionic acid and valeric acid were higher in the RSD compared with the GSD. The amounts of bile acids in feces, cholic acid, chenodeoxycholic acid and lithocholic acid were higher in the RSD compared with the GSD, But the amount of deoxycholic acid n the RSD period was significantly low. Secondary/primary ratios of bile acids was lower in the RSD compared with GSD, respectively. We speculate that , RS consumption decreases colonic mucosal proliferation as a result of the decreased formation of cytotoxic secondary bile acids. Thus, RS intakes may contribute the prevention of heart disease and colon cancer in humans. (Korean J Nutrition 33(8) : 802-812, 2000)

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A Case of Glycogen Storage Disease Type III Diagnosed by Gene Panel Sequencing (유전자 패널 검사로 진단된 당원병 III형 증례)

  • Kim, Seong Wan;Jang, Ju Young;Lee, Jang Hoon;Sohn, Young Bae;Jang, Ja-Hyun
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.20 no.1
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    • pp.24-28
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    • 2020
  • Type III Glycogen storage disease (Type III GSD, OMIM#232400) is a genetic metabolic disorder in which undigested glycogen accumulates in the organs due to lack of glycogen debranching enzyme caused by AGL mutation. The clinical symptoms of type III GSD include hepatomegaly, delayed growth, hypoglycemia and muscle weakness. These clinical symptoms are similar to those of other types of GSD, making it difficult to distinguish clinically. The authors report a case of type III GSD diagnosed by gene panel sequencing. A 11-month old male patient was presented with hepatomegaly. In liver biopsy, glycogen was accumulated in hepatocytes, suggesting GSDs. For differential diagnosis of types of GSD, gene panel sequencing for GSDs was performed. As a result, two novel pathogenic compound heterozygous variants: c.311_312del (p.His104Argfs*15) and c.3314+1G>A in AGL were detected and the patient was diagnosed as type III GSD. After diagnosis, he started dietary treatment with cornstarch, and has been free from complications. After two years, two same variants were also identified in the chorionic villous sampling of the pregnant mother, and the fetus was diagnosed as type III GSD. Gene panel sequencing is useful for diagnosis of disease which is indistinguishable by clinically and has high genetic heterogeneity, such as GSD. After diagnosis, familial genetic analysis can provide adequate genetic counseling and rapid diagnosis.

Acquisition of Subcentimeter GSD Images Using UAV and Analysis of Visual Resolution (UAV를 이용한 Subcentimeter GSD 영상의 취득 및 시각적 해상도 분석)

  • Han, Soohee;Hong, Chang-Ki
    • Journal of the Korean Society of Surveying, Geodesy, Photogrammetry and Cartography
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    • v.35 no.6
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    • pp.563-572
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    • 2017
  • The purpose of the study is to investigate the effect of flight height, flight speed, exposure time of camera shutter and autofocusing on the visual resolution of the image in order to obtain ultra-high resolution images with a GSD less than 1cm. It is also aimed to evaluate the ease of recognition of various types of aerial targets. For this purpose, we measured the visual resolution using a 7952*5304 pixel 35mm CMOS sensor and a 55mm prime lens at 20m intervals from 20m to 120m above ground. As a result, with automatic focusing, the visual resolution is measured 1.1~1.6 times as the theoretical GSD, and without automatic focusing, 1.5~3.5 times. Next, the camera was shot at 80m above ground at a constant flight speed of 5m/s, while reducing the exposure time by 1/2 from 1/60sec to 1/2000sec. Assuming that blur is allowed within 1 pixel, the visual resolution is 1.3~1.5 times larger than the theoretical GSD when the exposure time is kept within the longest exposure time, and 1.4~3.0 times larger when it is not kept. If the aerial targets are printed on A4 paper and they are shot within 80m above ground, the encoded targets can be recognized automatically by commercial software, and various types of general targets and coded ones can be manually recognized with ease.

Heterogenous Clinical Manifestations in Adult Patients with Late Diagnosis of Glycogen Storage Disease type Ia (성인기에 진단된 당원병 제 Ia형의 다양한 임상 양상)

  • Kim, Yoo-Mi;Cheon, Chong Kun;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.1
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    • pp.9-17
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    • 2015
  • Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.