Coronary artery aneurysm is a rare disorder. It is defined as abnormal dilatation of coronary artery with diameter exceeding 1.5 times the adjacent normal segments. The incidence of coronary aneurysm is 2.6% in Caucasians and 0.25% in Asians. Over half of the former were associated with atherosclerotic coronary artery disease. However, 70 percents of the latter were nonobstructive coronary artery aneurysms. Coronary artery fistula is a rare disorder. It has been identified in only 0.2% of routine cardiac angiographic studies conducted over a 10-year period. The clinical spectrums are various, asymtomatic, asymptomatic murmur, dyspnea on exertion, fatigue, and congestive heart failure. The right coronary artery (56%) and left coronary artery(36%) are mainly involved in the origin site of congenital coronary artery fistula. The draining site of fistula are right ventricle(39%), right atrium(33%), and pulmonary artery(20%) and so on. This 54 years-old woman had intermittent chest tightness and an abnormal mediastinal shadow on chest roentgenogram and chest C-T examination, which was diagnosed as a mediastinal mass such as teratoma. We performed the operation under left anterolateral thoracotomy for mass excision. However, we knew the mass had the pulsating arterial blood flow through a fine needle puncture of the mass and that it was attached to the left ventricle. We believed the excision of mass on beating heart would be very dangerous. Therefore, we closed the wound without excising the mass. After several days, we performed an echocardiography and coronary angiography, We knew it was cardiac tumor. Incidentally, the patient had a tortuous coronary fistula from the right coronary artery to pulmonary trunk. Using cardiopulmonary bypass with moderate systemic hypothermia, the mass was resected and the fistula was clipped with surgical clips. Pathology of the specimen was a giant coronary arterial aneurysm.
Orthodontic treatment is more complicated when both soft and hard tissues must be considered because an impacted maxillary canine has important effects on function and esthetics. Compared with extraction of impacted maxillary canines, exposure followed by orthodontic traction can improve esthetics and better protect the patient's teeth and alveolar bone. Therefore, in order to achieve desirable tooth movement with minimal unexpected complications, a precise diagnosis is indispensable to establish an effective and efficient force system. In this report, we describe the case of a 31-year-old patient who had a labio-palatal horizontally impacted maxillary left canine with a severe occlusal alveolar bone defect and a missing maxillary left first premolar. Herein, with the aid of three-dimensional imaging, sequential traction was performed with a three-directional force device that finally achieved acceptable occlusion by bringing the horizontally impacted maxillary left canine into alignment. The maxillary left canine had normal gingival contours and was surrounded by a substantial amount of regenerated alveolar bone. The 1-year follow-up stability assessment demonstrated that the esthetic and functional outcomes were successful.
Park, Su Jin;Ahn, Moon Bae;Jang, Woori;Cho, Won Kyung;Chae, Hyo Jin;Kim, Myung Shin;Suh, Byung Kyu
Journal of The Korean Society of Inherited Metabolic disease
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v.17
no.3
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pp.103-108
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2017
Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.
Most brachytherapy treatment planning systems employ a dosimetry formalism based on the AAPM TG-43 report which does not appropriately consider tissue heterogeneity. In this study we aimed to set up a simple Monte Carlo-based intracavitary high-dose-rate brachytherapy (IC-HDRB) plan verification platform, focusing particularly on the robustness of the direct Monte Carlo dose calculation using material and density information derived from CT images. CT images of slab phantoms and a uterine cervical cancer patient were used for brachytherapy plans based on the Plato (Nucletron, Netherlands) brachytherapy planning system. Monte Carlo simulations were implemented using the parameters from the Plato system and compared with the EBT film dosimetry and conventional dose computations. EGSnrc based DOSXYZnrc code was used for Monte Carlo simulations. Each $^{192}Ir$ source of the afterloader was approximately modeled as a parallel-piped shape inside the converted CT data set whose voxel size was $2{\times}2{\times}2\;mm^3$. Bracytherapy dose calculations based on the TG-43 showed good agreement with the Monte Carlo results in a homogeneous media whose density was close to water, but there were significant errors in high-density materials. For a patient case, A and B point dose differences were less than 3%, while the mean dose discrepancy was as much as 5%. Conventional dose computation methods might underdose the targets by not accounting for the effects of high-density materials. The proposed platform was shown to be feasible and to have good dose calculation accuracy. One should be careful when confirming the plan using a conventional brachytherapy dose computation method, and moreover, an independent dose verification system as developed in this study might be helpful.
Several isotopomers of cyclooctanone were prepared by selective deuterium substitution. Intrinsic isotope effects on $^{13}C$ NMR chemical shifts of these isotopomers were investigated systematically at low temperature. These istope effects were discussed in relation to the preferred boat-chair conformation of cyclooctanone. Deuterium isotope effects on NMR chemical shifts have been known for a long time. Especially in a conformationally mobile molecule, isotope perturbation could affect NMR signals through a combination of isotope effects on equilibria and intrinsic effects. The distinction between intrinsic and nonintrinsic effects is quite difficult at ambient temperature due to involvement of both equilibrium and intrinsic isotope effects. However if equilibria between possible conformers of cyclooctanone are slowed down enough on the NMR time scale by lowering temperature, it should be possible to measure intrinsic isotope shifts from the separated signals at low temperature. $^{13}C$ NMR has been successfully utilized in the study on molecular conformation in solution when one deals with stable conformers or molecules were rapid interconversion occurs at ambient temperature. The study of dynamic processes in general requires analysis of spectra at several temperature. Anet et al. did $^1H$ NMR study of cyclooctanone at low temperature to freeze out a stable conformation, but were not able initially to deduce which conformation was stable because of the complexity of alkyl region in the $^1H$ NMR spectrum. They also reported the $^1H$ and $^{13}C$ NMR spectra of the $C_9-C_{16}$ cycloalkanones with changing temperature from $-80^{\circ}C$ to $-170^{\circ}C$, but they did not report a variable temperature $^{13}C$ NMR study of cyclooctanone. For the analysis of the intrinsic isotope effect with relation to cylooctanone conformation, $^{13}C$ NMR spectra are obtained in the present work at low temperatures (up to $-150^{\circ}C$) in order to find the chemical shifts at the temperature at which the dynamic process can be "frozen-out" on the NMR time scale and cyclooctanone can be observed as a stable conformation. Both the ring inversion and pseudorotational processes must be "frozen-out" in order to see separate resonances for all eight carbons in cyclooctanone. In contrast to $^1H$ spectra, slowing down just the ring inversion process has no apparent effects on the $^{13}C$ spectra because exchange of environments within the pairs of methylene carbons can still occur by the pseudorotational process. Several isotopomers of cyclooctanone were prepared by selective deuterium substitution (fig. 1) : complete deuterium labeling at C-2 and C-8 positions gave cyclooctanone-2, 2, 8, $8-D_4$ : complete labeling at C-2 and C-7 positions afforded the 2, 2, 7, $7-D_4$ isotopomer : di-deuteration at C-3 gave the 3, $3-D_2$ isotopomer : mono-deuteration provided cyclooctanone-2-D, 4-D and 5-D isotopomers : and partial deuteration on the C-2 and C-8 position, with a chiral and difunctional case catalyst, gave the trans-2, $8-D_2$ isotopomer. These isotopomer were investigated systematically in relation with cyclooctanone conformation and intrinsic isotope effects on $^{13}C$ NMR chemical shifts at low temperature. The determination of the intrinsic effects could help in the analysis of the more complex effects at higher temperature. For quantitative analysis of intrinsic isotope effects, the $^{13}C$ NMR spectrum has been obtained for a mixture of the labeled and unlabeled compounds because the signal separations are very small.
Park, Sang-Jin;Kim, Sook-Ryung;Baek, Kum-Nyeo;Yoon, Joon-No;Jeong, Eun-Jeong;Kown, Ji-Eun;Kim, Hyon-J.
Journal of Genetic Medicine
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v.4
no.2
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pp.133-141
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2007
Purpose : Cri-du-Chat syndrome (CdCs) is a rare but clinically recongnizable condition with an estimated incidence of 1:50,000 live births. The clinical characteristics of the syndrome include severe psychomotor and mental retardation, microcephaly, hypertelorism, hypotonia, and slow growth. Also the size of the chromosome 5p deletion ranges were known from the region 5p13 to the terminal region. In this study, we report the spectrum of 5p deletion in Korean 20 pts. with CdCs and genotype-phenotype associations in CdCs. Methods : In order to delineate genotype-phenotype correlation, molecular cytogenetic studies including GTG banding and clinical characterization were performed on Korean 20 pts with CdCs including parents. CGH array and Fluorescence in situ hybridization (FISH) analysis were used to confirm a terminal deletion karyotype and map more precisely the location of the deletion breakpoint. Results : Molecular analysis of the spectrum of 5p deletion revealed 9 pts (45%) with a del (5)(p14), 7 pts. (35%) a del (5)(p13), 3 pts. (15%) a del (5)(p15.1) and 1 pt. (5%) a del (5)(p15.2) in 20 pts with CdCs. 4(20%)pts were identified to have additional chromosome abnormalites of deficiency and duplication involving chromosomes of 6, 8, 18, & 22. Parental study identified 3 familial case (2 paternal and 1 maternal origin) showing parents being a balanced translocation carrier. And the comparison study of the deletion break points among these 20 pts. with their phenotype has showed the varying clinical pheno-types in the CdCs critical region. Conclusion : The characterization of 5p deletion including parental study may help to delineate the genotypephenotype correlation in CdCs. Also these molecular cytogenetic analyses will be able to offer better information for accurate genetic diagnosis in CdCs and further make possible useful genetic counseling in pts. and family.
With the rapid evolution of technology, the size, number, and the type of databases has increased concomitantly, so data mining approaches face many challenging applications from databases. One such application is discovery of fraud patterns from agricultural product wholesale transaction instances. The agricultural product wholesale market in Korea is huge, and vast numbers of transactions have been made every day. The demand for agricultural products continues to grow, and the use of electronic auction systems raises the efficiency of operations of wholesale market. Certainly, the number of unusual transactions is also assumed to be increased in proportion to the trading amount, where an unusual transaction is often the first sign of fraud. However, it is very difficult to identify and detect these transactions and the corresponding fraud occurred in agricultural product wholesale market because the types of fraud are more intelligent than ever before. The fraud can be detected by verifying the overall transaction records manually, but it requires significant amount of human resources, and ultimately is not a practical approach. Frauds also can be revealed by victim's report or complaint. But there are usually no victims in the agricultural product wholesale frauds because they are committed by collusion of an auction company and an intermediary wholesaler. Nevertheless, it is required to monitor transaction records continuously and to make an effort to prevent any fraud, because the fraud not only disturbs the fair trade order of the market but also reduces the credibility of the market rapidly. Applying data mining to such an environment is very useful since it can discover unknown fraud patterns or features from a large volume of transaction data properly. The objective of this research is to empirically investigate the factors necessary to detect fraud transactions in an agricultural product wholesale market by developing a data mining based fraud detection model. One of major frauds is the phantom transaction, which is a colluding transaction by the seller(auction company or forwarder) and buyer(intermediary wholesaler) to commit the fraud transaction. They pretend to fulfill the transaction by recording false data in the online transaction processing system without actually selling products, and the seller receives money from the buyer. This leads to the overstatement of sales performance and illegal money transfers, which reduces the credibility of market. This paper reviews the environment of wholesale market such as types of transactions, roles of participants of the market, and various types and characteristics of frauds, and introduces the whole process of developing the phantom transaction detection model. The process consists of the following 4 modules: (1) Data cleaning and standardization (2) Statistical data analysis such as distribution and correlation analysis, (3) Construction of classification model using decision-tree induction approach, (4) Verification of the model in terms of hit ratio. We collected real data from 6 associations of agricultural producers in metropolitan markets. Final model with a decision-tree induction approach revealed that monthly average trading price of item offered by forwarders is a key variable in detecting the phantom transaction. The verification procedure also confirmed the suitability of the results. However, even though the performance of the results of this research is satisfactory, sensitive issues are still remained for improving classification accuracy and conciseness of rules. One such issue is the robustness of data mining model. Data mining is very much data-oriented, so data mining models tend to be very sensitive to changes of data or situations. Thus, it is evident that this non-robustness of data mining model requires continuous remodeling as data or situation changes. We hope that this paper suggest valuable guideline to organizations and companies that consider introducing or constructing a fraud detection model in the future.
Both the child's and defendant's testimonies play crucial roles in the court's ruling of a child abuse case. Thus, empirical studies examining a child's truthful report, that is, disclosure, of his or her experience and recantation of the disclosure have manifold practical implications. The objective of the present study was to examine how easily a child recants his or her testimony after witnessing and disclosing an adult engaging in a small mistake. Furthermore, this study examined whether the child's age and emotional support from his or her caregiver predict the recantation of the child's testimony. Children of age 5-8 years played with dolls with the experimenter and witnessed the experimenter breaking the doll mask. The experimenter asked the children to keep it a secret. Then the children had the first memory interview, during which the interviewer induced the child to disclose the incident. Based on the treatment conditions, some children were provided supportive feedback while other received unsupportive feedback from their primary caregivers (mother) regarding the disclosure, then were interviewed for the second time. First, the author of this study examined whether the children would recant their disclosure (whether they would deny the incident after telling the truth of about what happened to the doll), and also examined the features of the child's voluntary reports, that is, the degree of their honesty. The findings of the experiment indicated that there were age-specific differences in the frequency of recantation, meaning that older children (8-9-year-olds) showed a stronger tendency to maintain their recantation in the second interview than relatively younger children (6-7-year-olds). Furthermore, children who received supportive responses from their mothers regarding the disclosure demonstrated more honest reports compared to those who received unsupportive responses from their mothers. The findings of this study assist the understanding of the effects of social-motivational factors on the process of children disclosing the truth when voluntarily recalling a negative incident that they had experienced and provide practical implications in legal aspects.
The cultural contents industries have moved closer to the centre of the economic action in many countries and across much of the world. For this reason, the concern with the development of glocal cultural contents has also been growing. According to Goldman Sock's BRICs report, Indian economy will be the engine of global economy with China. In addition, India will be a new blue chip country for large consumer market of cultual contents. The most important point for the development of glocal cultural contents is a systematic and in-depth analysis of other culture. India is a complex and multicultural country compared with Korea which is a nation-state. Therefore, this paper is intended as an understanding about India appropriately and suggestion for a strategy to enter cultural industry in India. As the purpose of this paper is concerned, we will take a close look at 9 Indian culture codes which can be classified into three main groups: 1) political, social and cultural codes 2) economic codes 3) cultural contents codes. Firstly, political, social and cultural codes are i) consistent democracy and saving common people, ii) authoritarianism which appears an innate respect for authority of India, iii) Collective-individualism which represents collectivist and individualistic tendency, iv) life-religion, v) carpe diem. Secondly, economic culture codes are vi) 1.2billion Indian people's God which represents money and vii) practical purchase which stands for a reasonable choice of buying products. Lastly, viii) Masala movie and ix) happy ending that is the most popular theme of Masala movies are explained in the context of cultural content codes. In conclusion, 3 interesting cases , , will be examined in detail. From what has been discussed above, we suggest oversea expansion strategy based on these case studies. Eventually, what is important is to understand what Indian society is, how Indian society works and what contents Indian prefers.
Background: Chest wall tumors can classified into soft tissue tumors and bone tissue tumors and can be subclassified into benign and malignant tumors. Materials and methods: We report an analysis of 68 patients with primary chest wall tumors treated at the department of thoracic and cardiovascular surgery at Hanyang University Hospital from January, 1973 to September 1997. Results: Among a total of 68 patients 33(48.5%) were males and 35(51.5%) were females. The ages of the patients ranged from 10 to 79 years with a mean age of 39.3 years. According to the age distribution, 23 patients (33.8%) were from the 4th decade, 12 patients(17.6%) were from the 6th decade, and 10 patients(14.7%) were from the 5th decade. Among the primary chest wall tumors, 53 cases were benign and 15 cases were malignant. Among the benign tumors, 17 cases(32.1%) were in the 4th decade and among the malignant tumors, 6 cases(40%) were in the 4th decade. In both malignant and benign tumors the most common ages were in the 4th decade. The most common tumors were fibrous dysplasia and chondroma, each with a total of 14 cases(26.4%). Osteochondroma and lipoma each had 8 cases(15.1%). Among malignant tumors, osteosarcoma was most common with 8 cases (53.3%). According to location, 49 cases occured in both bone and cartilage tissue, 19 cases occurred in cartilage. Among the presenting symptoms, palpable mass was present in all cases. Fifty-one patients complained of tenderness and among cases with involvement of the lung, 3 patients had complained of respiratory distress. Among the malignant tumors 6 cases underwent a radical operation and 4 cases of benign tumors underwent a radical operation. Postoperativly, there was one case with recurrence from a desmoid tumor. There were no deaths postoperativly and no deaths due to complications(and their postoperative courses were uneventful). Conclusions: Most patients with primary chest wall tumors initially present with mass at admission. Resection is sufficient treatment for benign tumors but in malignant tumors wide resection of the chest wall is needed and mchest wall reconstruction.
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