Journal of The Korean Society of Inherited Metabolic disease
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v.16
no.1
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pp.34-41
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2016
Adult-onset type II citrullinemia (CTLN2) is characterized by episodes of neurologic symptoms associated with hyperammonemia leading to disorientation, irritability, seizures, and coma. CTLN2 is distinct from classical citrullinemia, which is caused by a mutation of the argininosuccinic acid synthetase (ASS) gene. The serum citrulline level is elevated, while the activity of ASS in liver tissue is decreased. CTLN2 is known to have a poor prognosis if the proper treatment is not taken. We reported a female aged 37 years who developed recurrent attacks of altered consciousness, aberrant behavior, and vomiting. We initially suspected the patient had CTLN2 because of the signs of hyperammonemic encephalopathy, such as altered mentality, memory disturbance, and aberrant behaviors provoked by exercise-induced stress and excessive intravenous amino acid administration. Through her peculiar diet preferences and laboratory findings that included hyperammonemia and citrullinemia, we diagnosed the patient as CTLN2, and SLC25A13 sequencing revealed known compound heterozygous mutations (IVS11+1G>A, c.674C> A). Her parents were heterozygous carriers, and we identified that her older sister had the same mutations. The older sister had not experienced any episodes of hyperammonemia, but she had peculiar diet preferences. The patient and her sister have been well with conservative management. When considering the clinical course of CTLN2, it was meaningful that the older sister could be diagnosed early in an asymptomatic period and that preemptive treatment was employed. Through this case, CTLN2 should be considered in adults who present symptoms of hyperammonemic encephalopathy without a definite etiology. Because of its rare incidence and similar clinical features, CTLN2 is frequently misdiagnosed as hepatic encephalopathy, and it shows a poor prognosis due to the lack of early diagnosis and proper treatment. A high-carbohydrate diet, which is usually used to treat other urea cycle defects, can also exaggerate the clinical course of CTLN2, so proper metabolic screening tests and genetic studies should be performed.
Xenotransplantation in discordant species results in immediate and irreversible hyperacute rejection due to natural antibodies, IgM. With this, antibody depletion is one option to reduce hyperacute rejection, we investigated the effect of PCPP (postcentrifugal plasmapheresis) on the depletion of natural antibodies and the effect of antibody titer on xenograft survival. Material and Method: Outbred swines (n=4) weighing 10∼20 kg were used as donors and mongrel dogs (n=4) weighing 25∼30 kg were used as recipients. Recipient canines underwent plasmapheresis (COBE TPE Laboratories, Lakewood. CO, USA). Pre-transplantation PCPP was peformed on day -2 and day 0. There were three groups (Group 0: no PCPP, Group 1: 1 pla sma-volume (PV) at day -2 and 2 PV at day 0, Group 2: 2 PV at day -2 and 2 PV at day 0). A swine heart was heterotopically transplanted into a recipient's abdominal infrarenal aorta and inferior vena cava. Mean percent depletion of total IgM and IgG in plasma of the recipients was calculated. Serum albumin, electrolyte, complement activity and coagulation factors were measured. Histopathologic examination of heart specimens was performed. Result: Mean percent depletion of IgM and IgG were 95.7$\pm$1.2%, 80.5$\pm$2.4% in the group 2 at the end of PCPP. The percent depletion of serum albumin concentration was decreased from 2.8 to 1.4 g/㎗ in the group 1 and 3.0 to 1.5 g/㎗ in the group 2. Complement hemolytic activity was decreased in group 1 and 2, but returned to normal level within 24 hours. Complement hemolytic activity was reduced to 10% of pre-PCPP level in group 2. Serum fibrinogen decreased to 20% or less and was recovered within 24 hours in group 2. Antithrombin III decreased but less than fibrinogen. PT and aPTT were sometimes but not always prolonged during plasmapheresis. After plasmapheresis, PT and aPTT were prolonged beyond the measurable level. D-dimer was not found during PCPP, but appeared and maintained from 10 minutes after trasplantation. Graft Survival time was 5 min in group 0, and it was 90$\pm$0 min in the group 2. Histopathologic changes were more typically characterized by edema, hemorrhages, thrombosis in all groups at the end of experiment. Conclusion: PCPP effectively removed immuoglobulins and reduced the titer of natural antibodies, as a result, significantly prololonged swine heart xenograft survival.
Chang Mu Lee;Chang Ho Jeon;Rang Lee;Hoon Kwon;Chang Won Kim;Jin Hyeok Kim;Jae Hun Kim;Hohyun Kim;Seon Hee Kim;Chan Kyu Lee;Chan Yong Park;Miju Bae
Journal of the Korean Society of Radiology
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v.82
no.4
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pp.923-935
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2021
Purpose We aimed to assess the clinical efficacy of transcatheter arterial embolization (TAE) for treating hemothorax caused by chest trauma. Materials and Methods Between 2015 and 2019, 68 patients (56 male; mean age, 58.2 years) were transferred to our interventional unit for selective TAE to treat thoracic bleeding. We retrospectively investigated their demographics, angiographic findings, embolization techniques, technical and clinical success rates, and complications. Results Bleeding occurred mostly from the intercostal arteries (50%) and the internal mammary arteries (29.5%). Except one patient, TAE achieved technical success, defined as the immediate cessation of bleeding, in all the other patients. Four patients successfully underwent repeated TAE for delayed bleeding or increasing hematoma after the initial TAE. The clinical success rate, defined as no need for thoracotomy for hemostasis after TAE, was 92.6%. Five patients underwent post-embolization thoracotomy for hemostasis. No patient developed major TAE-related complications, such as cerebral infarction or quadriplegia. Conclusion TAE is a safe, effective and minimally invasive method for controlling thoracic wall and intrathoracic systemic arterial hemorrhage after thoracic trauma. TAE may be considered for patients with hemothorax without other concomitant injuries which require emergency surgery, or those who undergoing emergency TAE for abdominal or pelvic hemostasis.
For the past 20 years, the growth and development of university-design-educational institutes contributed to the industrial development of our country. Due to the technological fluctuation and changes in the industrial structure in the latter half of the 20th century, the enterprise is demanding professionally-oriented design manpower. The principle which appears from instances of the advanced nations is to accommodate the demands in social changes and apply them to educational design programs. In order to respond promptly to the industrial demand especially, the advanced nations adopted "multidisciplinary design education programs" to lead innovation in the area of design globally. The objective of the research consequently is to suggest an educational system and a program through which the designer can be educated to obtain complex knowledge and the technique demanded by the industry and enterprise. Nowadays in order to adapt to a new business environment, designers specially should have both the knowledge and techniques in engineering and business administration. We suggest that the IPDI, a multidisciplinary design educational system and program is made up of the coordinated operation of major classes, on-the-job training connection, educational system for research base creation, renovation design development program for the application and the synthesis of alternative proposals about the training facility joint ownership by connecting with the education of design, business administration and engineering.
Kim, Yong Kuk;Im, Hae Ra;Lee, Gwang Hoon;Han, Soo Jin;Sun, Yong Han;Ryoo, Eell;Cho, Kang Ho;Tchah, Hann;Lee, Hak Soo
Clinical and Experimental Pediatrics
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v.46
no.1
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pp.37-41
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2003
Purpose : Although air enema reduction has been known as a good method of diagnosis and treatment of intussusception, it could develop colon perforation. However, there have been few studies about this complication. So we analyzed the risk factors of colon perforation during air enema reduction in patients with intussusception. Methods : We reviewed the charts of 12 colon perforation patients during air enema reduction of intussusception, who were admitted to Gil Medical Center from Jan. 1990 to Dec. 2001. Their age, sex, major symptoms, length of time till hospital visit, types of intussusception, operative findings and pathologic reports were reviewed. Results : Among 657 cases, 596 patients(90.7%) were successfully treated, but 12 patients(1.83%) failed in air enema reduction and had colon perforation. In patients with colon perforation the male to female ratio was 11 : 1, and average age was 5.3 months. The most common symptom at the time of hospital visit was vomiting(91.7%). Cyclic irritability(75.0%), bloody stool(75.0%) and abdominal mass(41.7%) were also noted. The average length of time between symptom onset and hospital visit was 44.7 hours. Types of intussusception were predominantly ileocolic, ileocecal, and ileoileocolic. The site of perforation was most commonly found at the proximal part of intussusception including ascending colon(50%) and transverse colon(50%). Most cases were uncomplicated, and had a single perforation. Pathologic reports showed hemorrhagic necrosis and mesenteric laceration at the site of colon perforation. Complications of colon perforation were tension pneumoperitonium(58.3%), requiring immediate decompression. Conclusion : The chance of colon perforation during air enema reduction increases in cases with small bowel obstruction on simple abdominal x-ray of a patient younger than 6 months, delay in time till hospital visit and higher air pressure during reduction. Therefore more careful investigation is needed in these cases.
Jangdo Island (area $1.54km^2$) located in the western end of Dadohae Haesang National Park has been recognized as an prominent ecoregion possessing high moor and national biodiversity hotspot. In terms of the Z$\ddot{u}$rich-Montpellier School's phytosociology, we investigate the diversity of plant communities on the island and reevaluate the Jangdo wetland designated as Ramsar site. Ten physiognomic types of the Jangdo's vegetation were classified into 22 syntaxa (3 associations, 15 communities and 4 subcommunities). Jangdo wetland was actually denominated as 'eutrophic wetland' by Pharagmitetea and Orizetea rather than 'high moor'. Nevertheless, existence value of the Jangdo wetland is evaluated very high as a stepping stone for migratory birds and even plant dispersions. A new site of the northernmost distribution of Arachniodo-Castanopsietum sieboldii, which is a kind of cold-resistant phytocoenosis among the Camellietea japonicae of the warm-temperate broad-leaved forests, was described. Hosta yingeri-Carpinus turczaninovii var. coreana community and Carex wahuensis var. robusta-Juniperus chinensis var. procumbens community were described specifically as an endemic and an edaphic vegetation type, respectively. The unique Jangdo's vegetation reflects regional environmental conditions such as much higher frequency of frost-free days and the highest number of annual average foggy days in Korea and a well-developed aquifer in the depressed basin formed by differential erosion. We identified that human interventions (pasture, logging, forest fire, cultivation, etc.) has been involved intensively on every vegetation types, even though a rugged and inaccessible topography of the island. Particularly the Jangdo wetland has been recently threatened by fundamental distortion on hydrological system. We request an immediate establishment of the conservation prescription manual.
Transportation enterprises should maintain constant and qualitative operation. Thus, in short period, transportation enterprises don't change supply in accordance with demand. In the result, transportation enterprises don't reduce operation in spite of management deficit at will. In freight transportation type, less-than-truckload(LTL) has more relation with above transportation feature than truckload(TL) does. Because freight transportation supply of TL is more flexible than that of LTL in correspondence of freight transportation demand. Relating to above mention, it appears that shortage of road and freight terminal of LTL is larger than that of TL. Especially in road and freight terminal comparison, shortage of freight terminal is larger than that of road. Shortage of road is the largest in 1990, and improved after-ward. But shortage of freight terminal is serious lately. So freight terminal needs more expansion than road, and shows better investment condition than road. Freight terminal expansion brings road expansion in LTL, on the contrary, freight terminal expansion substitutes freight terminal for road in TL. In transportation revenue, freight terminal's contribution to LTL is larger than that to TL. However, when we adjust quasi-fixed factor - road and freight terminal - to optimal level in the long run, in TL, diseconomies of scale becomes large, but in LTL, economies of scale becomes large. Consequently, it is necessary for TL to make counterplans to activate management of small size enterprises and owner drivers. And LTL should make use of economies of scale by solving the problem, such as nonprofit route, excess of rental freight handling of office, insufficiency of freight terminal, shortage of driver, and unpreparedness of freight insurance.
The purpose of the study was to investigate how the use of graphing calculators influence on forming students' mathematical concept of algebra, students' mathematical connection, and attitude toward mathematics. First, graphing calculators give instant feedback to students as they make students compare their written answers with the results, which helps students learn equations and linear inequalities for themselves. In respect of quadratic inequalities they help students to correct wrong concepts and understand fundamental concepts, and with regard to functions students can draw graphs more easily using graphing calculators, which means that the difficulty of drawing graphs can not be hindrance to student's learning functions. Moreover students could understand functions intuitively by using graphing calculators and explored math problems volunteerly. As a result, students were able to perceive faster the concepts of functions that they considered difficult and remain the concepts in their mind for a long time. Second, most of students could not think of connection among equations, equalities and functions. However, they could understand the connection among equations, equalities and functions more easily. Additionally students could focus on changing the real life into the algebraic expression by modeling without the fear of calculating, which made students relieve the burden of calculating and realize the usefulness of mathematics through the experience of solving the real-life problems. Third, we identified the change of six students' attitude through preliminary and an ex post facto attitude test. Five of six students came to have positive attitude toward mathematics, but only one student came to have negative attitude. However, all of the students showed positive attitude toward using graphing calculators in math class. That's because they could have more interest in mathematics by the strengthened and visualization of graphing calculators which helped them understand difficult algebraic concepts, which gave them a sense of achievement. Also, students could relieve the burden of calculating and have confidence. In a conclusion, using graphing calculators in algebra and function class has many advantages : formulating mathematics concepts, mathematical connection, and enhancing positive attitude toward mathematics. Therefore we need more research of the effect of using calculators, practical classroom materials, instruction models and assessment tools for graphing calculators. Lastly We need to make the classroom environment more adequate for using graphing calculators in math classes.
Hemoptysis which is comparatively common symptom in respiratory disease patients is a clinical symptom which has high risk of death in spite of many curative means. Bronchial tube embolization is a very useful medical procedure when hemoptysis does not stop for treatment of internal medicine or surgery and in addition to the purpose of instant hemostasis, for patients for whom surgical operation is impossible or for the purpose of gaining time to improve the state of the patient before surgical operation. In relation to this, this study is to know of the usefulness of bronchial tube artery embolization. The objects were 60 persons for whom bronchial tube artery embolizations are conducted because of large hemoptysis occurred from March 2007 to December 2009 in J hospital. They had large hemoptysis of 400ml or more per day and 200ml or more at a time or though the quantity of hemoptysis was less than 400ml they did not respond to the treatment of internal medicine for 10 days or longer. The average age was 60.5 years and cause diseases were tuberculosis, bronchiectasis, and pneumonia and lung cancer. Embolus parts were Rt bronchial artery 19 examples, Lt bronchial artery, both bronchial artery, Rt Intercostobronchial artery, and they were the case where embolization for many blood vessels were simultaneously carried out. As embolus materials, PVA (conteour) and microcoil were used. In 76.6% of 60 persons of patients hemorrhage stopped with the lapse of time after the procedure and in 4 examples (6.6) re-embolization was carried out due to re-bleeding after the procedure. Bronchial tube artery embolization has high early success rate and effectively controls hemoptysis in the treatment of hemoptysis and is an effective emergency remedy for hemorrhage due to large hemoptysis and will be a good medical procedure which reduces death rate. In addition, primarily if it is conducted together with the treatment of internal medicine it will be, as an effective curative means for hemorrhage due to large hemoptysis, a good mediate radial rays medical procedure which reduces death rate.
Alien turtles belonging to Genus Trachemys have been designated as Invasive Alien Species since 2001 and their import has been banned in Korea. However, current status of import and distribution of the other alien turtles have not been reported. In this study, we aimed to investigate the taxa of alien turtles introduced into Korea, to assess their potential risks to the natural ecosystems and to suggest the future management directions of them in Korea. We identified 73 species of alien turtles belonging to 9 families. Since 2008, more than 6,000 kg of turtles have been imported annually and widely distributed through the pet shops, traditional markets and individual transactions. From the survey of natural habitats, we found that 8 species belonging to 3 families including Chrysemys picta, Pseudemys concinna, P. nelsoni, P. peninsularis, P. rubriventris, Mauremys sinensis, Macrochelys temminckii and Trachemys scripta have inhabited in 12 study sites. Out of 73 alien turtles, the potential adverse impacts of 13 species to ecosystems are serious when we considered status of designation of invasive alien species in other countries. For the management of alien turtles, it is required to register alien turtles in the import list and share general information such as import purpose, distribution and management condition among relevant authorities. The breeders and distributors must be obliged to identify turtles and to record management. The government must check transfer and migration of turtles periodically to prevent their introduction and spread into natural environments. The change of alien turtle populations in natural habitats should be monitored and their management plan should be developed to control the alien turtles in areas where the impacts are significant.
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