Kim, Seong Wan;Jang, Ju Young;Lee, Jang Hoon;Sohn, Young Bae;Jang, Ja-Hyun
Journal of The Korean Society of Inherited Metabolic disease
/
v.20
no.1
/
pp.24-28
/
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.
Kim, Kwan-Wook;Cho, Sang-Ho;Shim, Won-Heum;Youn, Young-Nam
Journal of Chest Surgery
/
v.43
no.4
/
pp.428-432
/
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.
Ku, Gwan Woo;Choi, Jin Ho;Choi, Min Suk;Park, Sang Soon;Sul, Young Hoon;Go, Seung Je;Ye, Jin Bong;Kim, Joong Suck;Kim, Yeong Cheol;Hwang, Jung Joo
Journal of Trauma and Injury
/
v.28
no.4
/
pp.232-240
/
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.
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.
Park, Yong-Kon;Seog, Ho-Moon;Nam, Young-Jung;Choi, Kwang-Soo
Korean Journal of Food Science and Technology
/
v.22
no.6
/
pp.603-610
/
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.
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.
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.
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.
본 웹사이트에 게시된 이메일 주소가 전자우편 수집 프로그램이나
그 밖의 기술적 장치를 이용하여 무단으로 수집되는 것을 거부하며,
이를 위반시 정보통신망법에 의해 형사 처벌됨을 유념하시기 바랍니다.
[게시일 2004년 10월 1일]
이용약관
제 1 장 총칙
제 1 조 (목적)
이 이용약관은 KoreaScience 홈페이지(이하 “당 사이트”)에서 제공하는 인터넷 서비스(이하 '서비스')의 가입조건 및 이용에 관한 제반 사항과 기타 필요한 사항을 구체적으로 규정함을 목적으로 합니다.
제 2 조 (용어의 정의)
① "이용자"라 함은 당 사이트에 접속하여 이 약관에 따라 당 사이트가 제공하는 서비스를 받는 회원 및 비회원을
말합니다.
② "회원"이라 함은 서비스를 이용하기 위하여 당 사이트에 개인정보를 제공하여 아이디(ID)와 비밀번호를 부여
받은 자를 말합니다.
③ "회원 아이디(ID)"라 함은 회원의 식별 및 서비스 이용을 위하여 자신이 선정한 문자 및 숫자의 조합을
말합니다.
④ "비밀번호(패스워드)"라 함은 회원이 자신의 비밀보호를 위하여 선정한 문자 및 숫자의 조합을 말합니다.
제 3 조 (이용약관의 효력 및 변경)
① 이 약관은 당 사이트에 게시하거나 기타의 방법으로 회원에게 공지함으로써 효력이 발생합니다.
② 당 사이트는 이 약관을 개정할 경우에 적용일자 및 개정사유를 명시하여 현행 약관과 함께 당 사이트의
초기화면에 그 적용일자 7일 이전부터 적용일자 전일까지 공지합니다. 다만, 회원에게 불리하게 약관내용을
변경하는 경우에는 최소한 30일 이상의 사전 유예기간을 두고 공지합니다. 이 경우 당 사이트는 개정 전
내용과 개정 후 내용을 명확하게 비교하여 이용자가 알기 쉽도록 표시합니다.
제 4 조(약관 외 준칙)
① 이 약관은 당 사이트가 제공하는 서비스에 관한 이용안내와 함께 적용됩니다.
② 이 약관에 명시되지 아니한 사항은 관계법령의 규정이 적용됩니다.
제 2 장 이용계약의 체결
제 5 조 (이용계약의 성립 등)
① 이용계약은 이용고객이 당 사이트가 정한 약관에 「동의합니다」를 선택하고, 당 사이트가 정한
온라인신청양식을 작성하여 서비스 이용을 신청한 후, 당 사이트가 이를 승낙함으로써 성립합니다.
② 제1항의 승낙은 당 사이트가 제공하는 과학기술정보검색, 맞춤정보, 서지정보 등 다른 서비스의 이용승낙을
포함합니다.
제 6 조 (회원가입)
서비스를 이용하고자 하는 고객은 당 사이트에서 정한 회원가입양식에 개인정보를 기재하여 가입을 하여야 합니다.
제 7 조 (개인정보의 보호 및 사용)
당 사이트는 관계법령이 정하는 바에 따라 회원 등록정보를 포함한 회원의 개인정보를 보호하기 위해 노력합니다. 회원 개인정보의 보호 및 사용에 대해서는 관련법령 및 당 사이트의 개인정보 보호정책이 적용됩니다.
제 8 조 (이용 신청의 승낙과 제한)
① 당 사이트는 제6조의 규정에 의한 이용신청고객에 대하여 서비스 이용을 승낙합니다.
② 당 사이트는 아래사항에 해당하는 경우에 대해서 승낙하지 아니 합니다.
- 이용계약 신청서의 내용을 허위로 기재한 경우
- 기타 규정한 제반사항을 위반하며 신청하는 경우
제 9 조 (회원 ID 부여 및 변경 등)
① 당 사이트는 이용고객에 대하여 약관에 정하는 바에 따라 자신이 선정한 회원 ID를 부여합니다.
② 회원 ID는 원칙적으로 변경이 불가하며 부득이한 사유로 인하여 변경 하고자 하는 경우에는 해당 ID를
해지하고 재가입해야 합니다.
③ 기타 회원 개인정보 관리 및 변경 등에 관한 사항은 서비스별 안내에 정하는 바에 의합니다.
제 3 장 계약 당사자의 의무
제 10 조 (KISTI의 의무)
① 당 사이트는 이용고객이 희망한 서비스 제공 개시일에 특별한 사정이 없는 한 서비스를 이용할 수 있도록
하여야 합니다.
② 당 사이트는 개인정보 보호를 위해 보안시스템을 구축하며 개인정보 보호정책을 공시하고 준수합니다.
③ 당 사이트는 회원으로부터 제기되는 의견이나 불만이 정당하다고 객관적으로 인정될 경우에는 적절한 절차를
거쳐 즉시 처리하여야 합니다. 다만, 즉시 처리가 곤란한 경우는 회원에게 그 사유와 처리일정을 통보하여야
합니다.
제 11 조 (회원의 의무)
① 이용자는 회원가입 신청 또는 회원정보 변경 시 실명으로 모든 사항을 사실에 근거하여 작성하여야 하며,
허위 또는 타인의 정보를 등록할 경우 일체의 권리를 주장할 수 없습니다.
② 당 사이트가 관계법령 및 개인정보 보호정책에 의거하여 그 책임을 지는 경우를 제외하고 회원에게 부여된
ID의 비밀번호 관리소홀, 부정사용에 의하여 발생하는 모든 결과에 대한 책임은 회원에게 있습니다.
③ 회원은 당 사이트 및 제 3자의 지적 재산권을 침해해서는 안 됩니다.
제 4 장 서비스의 이용
제 12 조 (서비스 이용 시간)
① 서비스 이용은 당 사이트의 업무상 또는 기술상 특별한 지장이 없는 한 연중무휴, 1일 24시간 운영을
원칙으로 합니다. 단, 당 사이트는 시스템 정기점검, 증설 및 교체를 위해 당 사이트가 정한 날이나 시간에
서비스를 일시 중단할 수 있으며, 예정되어 있는 작업으로 인한 서비스 일시중단은 당 사이트 홈페이지를
통해 사전에 공지합니다.
② 당 사이트는 서비스를 특정범위로 분할하여 각 범위별로 이용가능시간을 별도로 지정할 수 있습니다. 다만
이 경우 그 내용을 공지합니다.
제 13 조 (홈페이지 저작권)
① NDSL에서 제공하는 모든 저작물의 저작권은 원저작자에게 있으며, KISTI는 복제/배포/전송권을 확보하고
있습니다.
② NDSL에서 제공하는 콘텐츠를 상업적 및 기타 영리목적으로 복제/배포/전송할 경우 사전에 KISTI의 허락을
받아야 합니다.
③ NDSL에서 제공하는 콘텐츠를 보도, 비평, 교육, 연구 등을 위하여 정당한 범위 안에서 공정한 관행에
합치되게 인용할 수 있습니다.
④ NDSL에서 제공하는 콘텐츠를 무단 복제, 전송, 배포 기타 저작권법에 위반되는 방법으로 이용할 경우
저작권법 제136조에 따라 5년 이하의 징역 또는 5천만 원 이하의 벌금에 처해질 수 있습니다.
제 14 조 (유료서비스)
① 당 사이트 및 협력기관이 정한 유료서비스(원문복사 등)는 별도로 정해진 바에 따르며, 변경사항은 시행 전에
당 사이트 홈페이지를 통하여 회원에게 공지합니다.
② 유료서비스를 이용하려는 회원은 정해진 요금체계에 따라 요금을 납부해야 합니다.
제 5 장 계약 해지 및 이용 제한
제 15 조 (계약 해지)
회원이 이용계약을 해지하고자 하는 때에는 [가입해지] 메뉴를 이용해 직접 해지해야 합니다.
제 16 조 (서비스 이용제한)
① 당 사이트는 회원이 서비스 이용내용에 있어서 본 약관 제 11조 내용을 위반하거나, 다음 각 호에 해당하는
경우 서비스 이용을 제한할 수 있습니다.
- 2년 이상 서비스를 이용한 적이 없는 경우
- 기타 정상적인 서비스 운영에 방해가 될 경우
② 상기 이용제한 규정에 따라 서비스를 이용하는 회원에게 서비스 이용에 대하여 별도 공지 없이 서비스 이용의
일시정지, 이용계약 해지 할 수 있습니다.
제 17 조 (전자우편주소 수집 금지)
회원은 전자우편주소 추출기 등을 이용하여 전자우편주소를 수집 또는 제3자에게 제공할 수 없습니다.
제 6 장 손해배상 및 기타사항
제 18 조 (손해배상)
당 사이트는 무료로 제공되는 서비스와 관련하여 회원에게 어떠한 손해가 발생하더라도 당 사이트가 고의 또는 과실로 인한 손해발생을 제외하고는 이에 대하여 책임을 부담하지 아니합니다.
제 19 조 (관할 법원)
서비스 이용으로 발생한 분쟁에 대해 소송이 제기되는 경우 민사 소송법상의 관할 법원에 제기합니다.
[부 칙]
1. (시행일) 이 약관은 2016년 9월 5일부터 적용되며, 종전 약관은 본 약관으로 대체되며, 개정된 약관의 적용일 이전 가입자도 개정된 약관의 적용을 받습니다.