• 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.

• Journal of Chest Surgery
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• v.43 no.4
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• pp.428-432
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• 2010

A 67 years old male patient was admitted with back pain that had recurred from 6 months previously. Eleven years previously, he underwent stent grafting at the descending thoracic aorta for a chronic Stanford type B aortic dissection. The preoperative computed tomography showed aortic dissection from the origin of the left subclavian artery to the bifurcation of the abdominal aorta, and there was a type I endoleak at the proximal portion of the stent graft and aneurysmal dilatation of the descending aorta. A hybrid endovascular repair was successfully performed, and this involved debranching and rerouting the aortic arch vessels under extracorporeal cardiopulmonary bypass and then this was followed 13 days later by stenting in the ascending aorta, the aortic arch and the descending aorta. The postoperative computed tomography showed complete exclusion of the type I endoleak. After discharge, he has been followed up for 8 months without any problems.

• Journal of Trauma and Injury
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• v.28 no.4
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• pp.232-240
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• 2015

Purpose: Thoracic aortic injury is a life-threatening injury that has been traditionally treated by using surgical management. Recently, thoracic endovascular aortic repair (TEVAR) has been conducted pervasively as a better alternative treatment method. Therefore, this study will focus on analyzing the outcome of TEVAR in patients suffering from a blunt thoracic aortic injury. Methods: Of the blunt thoracic aortic injury patients admitted to Eulji University Hospital, this research focused on the 11 patients who had received TEVAR during the period from January 2008 to April 2014. Results: Seven of the 11 patients were male. At the time of admission, the mean systolic pressure was $105.64{\pm}24.60mm\;Hg$, and the mean heart rate was $103.64{\pm}20.02per$ minute. The median interval from arrival to repair was 7 (4, 47) hours. The mean stay in the ICU was $21.82{\pm}16.37hours$. In three patients, a chimney graft technique was also performed to save the left subclavian artery. In one patient, a debranching of the aortic arch vessels was performed. In two patients, the left subclavian artery was totally covered. In one patient whose proximal aortic neck length was insufficient, the landing zone was extended by using a prophylactic left subclavian artery to left common carotid artery bypass before TEVAR. There were no operative mortalities, but a patient who was covered of left subclavian artery died from ischemic brain injury. Complications such as migration, endovascular leakage, collapse, infection and thrombus did not occur. Conclusion: Our short-term outcomes of TEVAR for blunt thoracic aorta injury was feasible. Left subclavian artery may be sacrificed if the proximal landing zone is short, but several methods to continue the perfusion should be considered.

• KOREAN JOURNAL OF CROP SCIENCE
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• v.36 no.6
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• pp.560-567
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• 1991

Photosynthetic variation in field grown soybean [Glycine max (L.) Merr. cv Hodgson78］ was studied in relation to leaf anatomical variation. Variations in mesophyll morphology were accentuated by manipulating source and sink size. At R3 stage, two treatments were started: one was thinning and continu-ous debranching(6. 5 plants rather than 26 plants per m of row and remaining plants were debranched weekly), and the other was continuous partial depodding (allowing only one pod to develop at each mainstem node). Gas exchange characteristics, mesophyll cell volume and surface area per unit leaf surface, and microclimatic parameters were measured on the intact terminal leaflet at the 10th node. Observations were made 5 times with 3 to 4 day intervals starting R4 stage. Two models were used to compute leaf photosynthetic rates: one considered no effect of mesophyll morphology on photosynthesis, and the other considered potential effects of variations in mesophyll cell volume and surface area on diffusion and biochemical processes. Seventy nine percent of total photosynthetic variations observed in the experiment was explained by the latter, while 69% of the same variations was explained by the former model. By incorporating the mesophyll morphology concept, the predictability was improved by 14.6% in the field condition. Additional Index Words: photosynthesis model, leaf anatomy, Glycine max (L.) Merr., mesophyll surface area, mesophyll cell volume.

• Korean Journal of Food Science and Technology
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• v.22 no.6
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• pp.603-610
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• 1990

The structural properties of cold water extractable ${\alpha}-D-glucan$ in rice flours obtained by various milling methods were investigated. The blue value, ${\lambda}_{max}$ values of the iodine complex and ${\beta}-amylolysis$ limit of the cold water extractable ${\alpha}-D-glucan$ were in the range of $0.026{\sim}0.030,\;518{\sim}522\;nm$ and $52.7{\sim}59.6%$, respectively, indicating these materials were composed mainly of amylopectin-like polymer. The gel chromatography on Sepharose CL-2B indicated that the cold water extractable ${\alpha}-D-glucan$ had lower molecular weight but wider molecular weight distribution than that of the original rice amylopectin. The Sephadex G-50 gel chromatography showed that the unit chain distribution of cold water extratable ${\alpha}-D-glucan$ after debranching with pullulanase was similar to that of rice amylopectin. And the ion chromatography of the distribution pattern of the chain length below D.P.30 in the debranched ${\alpha}-D-glucan$ indicated only blade-milled flour was similar to the amylopectin, but in the amount of unit chain below D.P.10, all the ${\alpha}-D-glucan$ in rice flours was higher than that of amylopectin.

• Korean journal of food and cookery science
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• v.13 no.1
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• pp.7-15
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• 1997

The composition and polymeric structure of starch are the most important factors to determine the functional properties of rice. In an effort to relate the structural characteristics and its functional properties of starch in rice, molecular structural properties of starch from 6 rice cultivars were analysed. To relate the structure and function of starch the texture of Jeung-pyun made of rice were analyzed during retrogradation. The polymeric structure of rice starch was analyzed by debranching with isoamylase after gelatinization and fractionated with Sephadex G-75 gel filteration. The size fractionated debranched starch was categorized into four groups such as Fraction I, II, III and Intermediate Fraction depending in their max, The fractions with the max higher than 620 nm were designated as Fraction I, while those in the range of 600-620 nm and 540-600 nm as the Intermediate Fraction and Fraction II, respectively. The Fractions with the max lower than 540 were described as Fraction III. The average degree of polymerization (DP) of the Fraction I was estimated to be higher than 200, and those of other fractions, i.e. the Intermediate Fraction I, Fraction II and III were 150,45 and 25, respectively. The levels of Fraction I were varied from II to 35％ of total sugar. The Fraction I showed the linear relationship with the amylse contents, and the Intermediate Fraction, which might contain the mixture of short chain of amylose and debranched long chain of amylopectin, were measured to be in the range of 2.7∼8.4%. The levels of fraction II and III, both to be considered as the branches of amylopectin, were ranged 14.5∼23.6% and 39.7∼73.0%, respectively. The ratio of Fraction III to Fraction II describing the degree of branch or compactness of amylopectin was estimated to be around 4.0 for waxy varieties and around 2.0 for high amylose cultivars. With these results, it can be suggested that the degree of branch of the amylopectin may effect on amylose contents of starch or rice versa, To invertigate processing aptitude of different rice cultivars for the preparation of Jeung-pyun, its texture was analyzed by Instron, hardness, cohesiveness, elasticity, gumminess and chewiness of Jeung-pyun made of rice showed the significant relationship with the amylose content. Hardness was increased during retrogradation of product, but the relationship between hardness and amylose content due to not only difference in amylose content but also difference in structural characteristics of starch. In analysis of relationship between structure of rice starch and mechanical properties of Jeung-pyun during retrogradation elasticity did not show any relationship before retrogradation bur during retrogradation showed significant correlation. With these result, it can be suggested that the degree of branch of the amylopectin may effect on elasticity during retrogradation. However hardness, cohesiveness, gumminess and chewiness which were significant different before retrogradation, showed some correlation with structure of rice starch during retrigradation.

• Journal of Chest Surgery
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• v.43 no.5
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• pp.490-498
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• 2010

Background: A hybrid procedure using an open surgical extra-anatomic bypass of aortic arch vessels and thoracic endovascular aortic repair (TEVAR) is less invasive than open surgery, and provides a suitable proximal landing zone. Here we report our experience with a hybrid TEVAR procedure at a single center. Material and Method: We retrospectively reviewed consecutive patients with thoracic aortic disease who received a hybrid TEVAR procedure between August 2008 and January 2010. Patients' data were prospectively collected and mean follow-up was $10.8{\pm}5.5$ months (range 3~20). Result: Nine patients (7 males and 2 females) with a mean age of $63.8{\pm}15.8$ years (range 38~84) underwent a hybrid procedure. Five patients had an arch or a proximal descending aortic aneurysm, two had a dissecting aneurysm of the descending aorta, and two had an aneurysm of the ascending arch and descending aorta. Mean expected mortality calculated by logistic EuroSCORE was 21%. Six patients underwent debranching and rerouting from ascending aorta to arch vessels, 2 had carotid-carotid bypass grafting, and 1 underwent carotid-axillary bypass grafting. Mean operation time was $221.4{\pm}84.0$ min (range 94~364). Deployment success of endovascular stent grafting was 100% with no endoleak on completion angiography. There was no mortality, and a small embolism in the branch of the right opthalmic artery in one patient. During follow-up, one intervention was required for the endoleak. Actuarial survival at 20 months was 100%. Conclusion: Early and mid-term results are encouraging and suggest that hybrid TEVAR procedures are less invasive and safer and represent an effective technique for treating thoracic aortic disease.

• Korean Journal of Food Science and Technology
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• v.27 no.6
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• pp.1017-1027
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• 1995

This study was attempted to investigate physicochemical properties, molecular structural properties of native and acid-treated chestnut starch and chestnut starch gel. The amylose content was 18.9% and X-ray diffraction pattern showed Cb type. Swelling power was increased abruptly in the range of $65^{\circ}C{\sim}75^{\circ}C$ but increased slowly after that and solubility was increased abruptly until $70^{\circ}C$ but increased slowly after that. In amylograms which have different heating temperatures, cooling viscosity at $50^{\circ}C$ was reduced as heating temperature was increased. In molecular structural properties of amylose, ${\lambda}_{max}$ was 640 nm, ${\beta}-amylolysis$ limit was 84.2% and the degree of polymerization was 951 and in those of amylopectin, ${\lambda}_{max}$ was 570 nm, ${\beta}-amylolysis$ limit was 58.2%, the degree of polymerization was 1371 and average chain length was 22.6. In gel chromatography elution profiles of starch and amylose, 4.0% and 11.5% of low molecular weight-molecules($<5{\times}10^5$) were leached out. In gel chromatography elution profiles of soluble starch, the higher heating temperature was, the more high molecular weight-starches were leached out. The elution profiles after debranching amylopectin with pullulanase showed 2.2 of the ratio of peakIII(DP 10-15) to peakII(DP 35-45). Acid hydrolysis extent of 2.2 N HCI-treated starch at $35^{\circ}C$ for 10 days was 96% and hydrolysis rate showed two step pattern which had border line at 4 days. In elution profiles of acid treated chestnut starch, amylopectin peak was disappeared compeletly after 6 hrs and converted short chains of DP 10-15. Amylose content was increased until 6 hrs but decreased after that. Hardness of starch gel made at $75^{\circ}C$ of heating temperature and cohesiveness of starch gel made at $85^{\circ}C$ of heating temperature were the highest. Retrogradation rate of starch gels were relatively high, especially for the starch gel made at $75^{\circ}C$ of heating temperature.