Kim, Min-Sun;Park, Esther;Song, Ari;Im, Minji;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-Kyu
Journal of The Korean Society of Inherited Metabolic disease
/
v.18
no.3
/
pp.99-106
/
2018
Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.
An analytical method was developed for the determination of sedaxane in agricultural products using liquid chromatograph-tandem mass spectrometry (LC-MS/MS). The samples were extracted with acetonitrile and partitioned with dichloromethane to remove the interference, and then purified by using silica SPE cartridges to clean up. The analytes were quantified and confirmed by using LC-MS/MS in positive ion mode using multiple reaction monitoring (MRM). The matrix-matched calibration curves were linear over the calibration ranges ($0.001-0.25{\mu}g/mL$) into a blank extract with $r^2$>0.99. For validation, recovery tests were carried out at three different concentration levels (LOQ, 10LOQ, and 50LOQ, n=5) with five replicates performed at each level. The recoveries were ranged between 74.5 to 100.8% with relative standard deviations (RSDs) of less than 12.1% for all analytes. All values were consistent with the criteria ranges requested in the Codex guidelines (CAC/GL 40, 2003) and Food Safety Evaluation Department guidelines (2016). The proposed analytical method was accurate, effective and sensitive for sedaxane determination in agricultural commodities.
Hanwoo young bulls are selected based on performance test using the weight at 12 months and pedigree index comprising marbling score. Pedigree index was not based on the progeny tested data but the breeding value of the proven bulls; resulting a lower accuracy. The progeny testing of the young bulls was categorized into testing at farm and at the test station. The farm tested data was difficult to compare with those from test station data. Farm tested bulls had different slaughter ages than those for test station bulls. Therefore, this study had considered a different age at slaughter for respective records on ultrasound traits. Records on body weight at 12 months, ultrasound measures at 12 and 24 months(uIMF, uEMA, uBFT, and uRFT), and carcass traits(CWT, EMA, BFT, and MS) were collected from steers and bulls of Hanwoo national improvement scheme between 2008 and 2013. Fixed effects of batch, test date, test station, personnel for measurement, personnel for judging, and a linear covariate of weight at measurement were fitted in the animal models for ultrasound traits. The ranges of heritability estimates of the ultrasound traits at 12 and 24 months were 0.21-0.43 and 0.32-0.47, respectively. Ultrasound traits at 12 and 24 months between similar carcass traits was genetically correlated at 0.52-0.75 and 0.86-0.89, respectively.
The study aims to investigate the clinical utility of Bayley-III using US norm in Korea. A total of 98 preterm infants and 93 term infants were assessed with the K-Bayley-III. The performance pattern of preterm infants was analyzed with mixed design ANOVA which examined the differences of scaled scores and composite scores of Bayley-III between full term- and preterm- infant group and within preterm infants group. Then, We have investigated agreement between classifications of delay made using the BSID-II and Bayley-III. In addition, ROC plots were constructed to identify a Bayley-III cut-off score with optimum diagnostic utility in this sample. The results were as follows. (1) Preterm infants have significantly lower function levels in areas of 5 scaled scores and 3 developmental indexes compared with infants born at term. Significant differences among scores within preterm infant group were also found. (2) Bayley-III had the higher scores of the Mental Development Index and Psychomotor Developmental Index comparing to the scores of K-BSID-II, and had the lower rates of developmental delay. (3) All scales of Bayley-III, Cognitive, Language and Motor scale had the appropriate level of discrimination, but the cut-off composite scores of Bayley-III were adjusted 13~28 points higher than 69 for prediction of delay, as defined by the K-BSID-II. It explains the lower rates of developmental delay using the standard of two standard deviation. This study has provided empirical data to inform that we must careful when interpreting the score for clinical applications, identified the discriminating power, and proposed more appropriate cut-off scores. In addition, discussion about the sampling for making the Korean norm of Bayley-III was provided. It is preferable that infants in Korea should use our own validated norms. The standardization process to get Korean normative data must be performed carefully.
This study was carried out to select the rice variety for high quality rice according to reducing N application level (7 kg/10 a) in medium altitude and plain of southern Korea. It was cultivated to seven varieties and ten varieties in medium altitude and plain region respectively in this study. In medium altitude region, the yield of head rice was higher in most varieties except for Borami (526 kg/10 a) than the control variety, Chucheongbyeo (533 kg/10 a). On the other hand, Borami, Gopumbyeo, and onnuri were lower in head rice rate than the other varieties. In addition, Borami and Chucheongbyeo shown a lodging risk but the other varieties were safe against lodging. The milled rice protein content of Chucheongbyeo was 6.1% and the other varieties protein content was similar or lower than that of control except for Borami(6.3%). In plain region, the head rice yield of Nampyeongbyeo (527 kg/10 a) was higher than that of control variety, Ilmibyeo (503 kg/10 a). Head rice rate was lower in Hopumbyoe, Sindongjin, and Chilbobyeo than the other varieties. The varieties contained lower protein content compared Ilmibyeo (6.1%) were Hopumbyeo (5.9%), Jinsumi (6.0%), Sindongjin (5.9%), Chilbobyeo (6.0%), Hwangeumnodeul (5.8%), Hopyeongbyeo (6.0%). Amylose content, whiteness, and palatability (Toyo value) were no significant difference among varieties in two different regions. In conclusion, considering head rice rate, yield of head rice, protein content, lodging resistance, ripening rate and palatability, Pungmi1ho, Saenuri, Chilbobyeo in medium altitude region and Jinsumi, Hwanggumnodul in plain region were selected as proper rice varieties for high quality rice production in southern Korea.
Journal of Dental Rehabilitation and Applied Science
/
v.36
no.4
/
pp.254-261
/
2020
Purpose: The purpose of this study was to assess the marginal and internal fit of interim crowns fabricated by two different manufacturing method (subtractive manufacturing technology and additive manufacturing technology). Materials and Methods: Forty study models were fabricated with plasters by making an impression of a master model of the maxillary right first molar for ceramic crown. On each study model, interim crowns (n = 40) were fabricated using three types of 3D printers (Meg-printer 2; Megagen, Zenith U; Dentis, and Zenith D; Dentis) and one type milling machine (imes-icore 450i; imes-icore GmbH). The internal of the interim crowns were filled with silicon and fitted to the study model. Internal scan data was obtained using an intraoral scanner. The fit of interim crowns were evaluated in the margin, absolute margin, axial, cusp, and occlusal area by using the superimposition of 3D scan data (Geomagic control X; 3D Systems). The Kruskal-wallis test, Mann-Whitney U test and Bonferroni correction method were used to compare the results among groups (α = 0.05). Results: There was no significant difference in the absolute marginal discrepancy of the temporary crown manufactured by three 3D printers and one milling machine (P = 0.812). There was a significant difference between the milling machine and the 3D printer in the axial and occlusal area (P < 0.001). The temporary crown with the milling machine showed smaller axial gap and higher occlusal gap than 3D printer. Conclusion: Since the marginal fit of the temporary crown produced by three types of 3D printers were all with in clinically acceptable range (< 120 ㎛), it can be sufficiently used for the fabrication of the temporary crown.
In this study, an electronics industrial wastewater activated sludge model (e-ASM) to be used as a Water Digital Twin was calibrated based on real high-tech electronics industrial wastewater treatment measurements from lab-scale and pilot-scale reactors, and examined for its treatment performance, effluent quality prediction, and optimal process selection. For specialized modeling of a high-tech electronics industrial wastewater treatment system, the kinetic parameters of the e-ASM were identified by a sensitivity analysis and calibrated by the multiple response surface method (MRS). The calibrated e-ASM showed a high compatibility of more than 90% with the experimental data from the lab-scale and pilot-scale processes. Four electronics industrial wastewater treatment processes-MLE, A2/O, 4-stage MLE-MBR, and Bardenpo-MBR-were implemented with the proposed Water Digital Twin to compare their removal efficiencies according to various electronics industrial wastewater characteristics. Bardenpo-MBR stably removed more than 90% of the chemical oxygen demand (COD) and showed the highest nitrogen removal efficiency. Furthermore, a high concentration of 1,800 mg L-1 T MAH influent could be 98% removed when the HRT of the Bardenpho-MBR process was more than 3 days. Hence, it is expected that the e-ASM in this study can be used as a Water Digital Twin platform with high compatibility in a variety of situations, including plant optimization, Water AI, and the selection of best available technology (BAT) for a sustainable high-tech electronics industry.
Purpose Testosterone is a steroid hormone synthesized by the Leydig cells of the testes in men, and by the adrenal cortex and ovaries in women. Testosterone production is regulated by luteinzing hormone secreted by the anterior pituitary gland. In this experiment, the effectiveness of testosterone radioimmunoassay (RIA) kits produced by three companies was evaluated and compared in case the production of testosterone kits was stopped or supply problems occurred. Materials and Methods In October 2021, samples were collected from the patients (n=49) who requested the testosterone RIA test. The experiment was conducted by dividing the patient's sample into low concentration (1.0 ng/mL or less), medium concentration (2.0-4.0 ng/mL) and high concentration (6.0 ng/mL or more). The Testosterone RIA test compared and evaluated the validity of Company A kits used in this hospital and those of Company B and C used in other hospitals. The precision, sensitivity, recovery, linearity and correlation were evaluated for each kit. The testosterone RIA test was carried out in accordance with the insert kit manual for each manufacturer. Results As a result of measuring the precision of the intra assay, the Coefficient of Variation (CV) value of the company A kit was high at 11.4% only in the low concentration sample, and in the case of the company B and C kits, the CV value was less than 10% at low, medium, and high concentrations. In the inter-assay precision measurement, the CV value was less than 15% in both A and C kits, but in the case of the B kit, the CV value exceeded 15% at low and medium concentrations. Sensitivity was 0.13 ng/mL for company A, 0.01 ng/mL for company B, and 0.01 ng/mL for company C, and the linearity of all three kits showed excellent linearity. In the case of recovery rate, all of the A, B, and C company kits showed results that were out of 90-110%. In the case of correlation test, when compared with the company A kit currently use in here, the correlation coefficient (R2) value for the company B kit was 0.9508, and for the company C kit was 0.9352 Conclusion As a result, there was a slight difference in precision at the low concentration sample. The correlation test showed an excellent correlation coefficient. However, it was difficult to secure samples of various concentrations because there were not many tests of testosterone requested at this hospital. So, additional experiments should be carried out by acquiring samples of various concentrations on each laboratory later.
Sulfur-oxidizing, ammonium-oxidizing, and nitrogen-oxidizing media were used to isolate bacteria to degrade malodor gas effectively in piggery manure or soil. Twelve different strains were isolated: Paenibacillus amylolyticus, Rhodococcus jostii, Rhodococcus qingshengii, Rhodococcus opacus, Alcaligenes faecalis, Alcaligenes faecalis, Kastia adipate, Kastia adipata, Microbacterium oxydans, Halomonas campisalis, Acinetobacter oleivorans, and Micrococcus luteus. By inoculating each strain in the piggery liquid manure by 1%, the pH in most strain treatments was maintained at 8.0. Total bacterial counts were maintained at 7.3~7.9 log CFU/ml until 15 days, and then they dropped dramatically down to 5.1~5.5 log CFU/ml. On the 30th day, the treatment group inoculated with Rhodococcus opacus SK2659 showed a relatively high level of ammonium nitrogen removal, which was 39% of that of the control group. When Rhodococcus opacus SK2659 was inoculated, H2S concentration after 100 days was 3.23% compared with the control (no inoculation), suggesting that Rhodococcus opacus SK2659 is an excellent strain for removing malodor gas. The gas production of the treatments was lower than that of the control. The total accumulated amount of gas production in most strain treatments was a quarter of the gas production compared to the control throughout the experimental periods. Acinetobacter oleivorans SK2675 showed the lowest level at 12.39% compared to the control in gas production. In conclusion, the use of mixture strains, such as Rhodococcus opacus SK2659 and Acinetobacter oleivorans SK2675 isolated in this study could increase the efficacy of malodor gas reduction in the biological treatment of piggery manure.
Gwangyeon Kim;Sangmin Ha;Seongjun Lee;Boseong Lim;Min-Cheol Kim;Moon Son
Korean Journal of Mineralogy and Petrology
/
v.36
no.1
/
pp.55-72
/
2023
This study aims to identify the fault zone architecture and geometric and kinematic characteristics of the Yeongdeok Fault, based on the geometry and kinematic data of various structural elements obtained by detailed field survey and anisotropy of magnetic susceptibility (AMS) of the fault rocks. The Yeongdeok Fault extends from Opo-ri, Ganggu-myeon, Yeongdeok-gun to Gilgok-ri, Maehwa-myeon and Bangyul-ri, Giseong-myeon, Uljin-gun, and cuts various rock types from the Paleo-proterozoic to the Mesozoic with a range of 4.6-5.0 km (4.77 km in average) of right-lateral offset or forms the rock boundaries. The fault is divided into four segments based on its geometric features and shows N-S to NNW strikes and dips of an angle of ≥ 54° to the east at most outcrops, even though the outcrops showing the westward dipping (a range of 54°-82°) of fault surface increase as it goes north. The Yeongdeok Fault shows the difference in the fault zone architecture and in the fault core width ranging from 0.3 to 15 m depending on the bedrock type, which is interpreted as due to differences in the physical properties of bedrock such as ductility, mineral composition, particle size, and anisotropy. Combining the results of paleostress reconstruction and AMS in this and previous studies, the Yeongdeok Fault experienced (1) sinistral strike-slip under NW-SE maximum horizontal principle stress (σHmax) and NE-SW minimum horizontal principle stress (σHmin) in the late Cretaceous to early Cenozoic, and then (2) dextral strike-slip under NE-SW maximum horizontal principle stress (σHmax) and NW-SE minimum horizontal principle stress (σHmin) in the Paleogene. It is interpreted that the deformation caused by the Paleogene dextral strike-slip movement was the most dominant, and the crustal deformation was insignificant thereafter.
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.