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A Case of Hunter Syndrome Diagnosed at 7 Months of Age by Exome Sequencing (엑솜시퀀싱을 통해 생후 7개월에 진단된 헌터증후군)

  • Song, Ari;Lee, Jin Sung;Im, Minji;Park, Hyung Doo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.2
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    • pp.62-67
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    • 2018
  • Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.

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Proanthocyanidins Suppresses Lipopolysaccharide-stimulated Inflammatory Responses via Heme Oxygenase-1 Induction in RAW264.7 Macrophages (프로안토시아니딘의 항염증효과)

  • Cheon, Hye-Jin;Park, Sun Young;Jang, Hee-Ji;Cho, Da-Young;Jung, Jiwon;Park, Gimin;Jeong, Kyeong Mi;Kim, Jin-Kyung
    • Journal of Life Science
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    • v.29 no.4
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    • pp.484-491
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    • 2019
  • Proanthocyanidins are naturally occurring polyphenolic compounds abundant in many vegetables, plant skins (rind/bark), seeds, flowers, fruits, and nuts. Numerous in vitro and in vivo studies have demonstrated myriad effects potentially beneficial to human health, such as antioxidation, immunomodulation, DNA repair, and antitumor activity. Among immune cells, macrophages are crucial players in a variety of inflammatory responses to environmental conditions. However, it has been widely reported that macrophages cause chronic inflammation and are involved in a variety of diseases, such as obesity, diabetes, metabolic syndrome, and cancer. In this study, we report the suppressive effect of proanthocyanidins via the heme oxygenase-1 (HO-1)-related system, on the immune response of the LPS-stimulated mouse macrophage cell line RAW264.7. Increased HO-1 expression at mRNA and protein levels were found in proanthocyanidins-treated RAW264.7 cells. Further, proanthocyanidins enhanced nuclear factor-erythroid 2-related factor 2 translocation into the nucleus. RAW264.7 cells were treated with lipopolysaccharide (LPS) with or without proanthocyanidins, and inflammatory mediator expression levels were assessed. Proanthocyanidins treatment resulted in the attenuation of nitric oxide production and inducible nitric oxide synthase expression in LPS-stimulated RAW264.7 cells. In addition, mRNA and protein expression of proinflammatory cytokines, such as tumor necrosis factor-${\alpha}$ and interleukin-6, was inhibited by proanthocyanidins treatment in LPS-stimulated RAW264.7 cells. These findings support proanthocyanidins as a promising anti-inflammatory agent.

Isolation and characterization of cellulolytic yeast belonging to Moesziomyces sp. from the gut of Grasshopper (메뚜기의 내장에서 분리한 Moesziomyces 속에 속하는 셀룰로오스 분해 효모의 분리 및 특성)

  • Kim, Ju-Young;Jung, Hee-Young;Park, Jong-Seok;Cho, Sung-Jin;Lee, Hoon Bok;Sung, Gi-Ho;Subramani, Gayathri;Kim, Myung Kyum
    • Korean Journal of Microbiology
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    • v.55 no.3
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    • pp.234-241
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    • 2019
  • An intensive interaction between yeasts and insects has highlighted their relevance for attraction to food and for the insect's development and behavior. Yeast associated in the gut of insects secretes cellulase which aided in the food digestion (cellulose degradation). Three strains of cellulose-degrading yeast were isolated from the gut of adult grasshoppers collected in Gyeonggi Province, South Korea. The strains $ON22^T$, $G10^T$, and $G15^T$, showed positive cellulolytic activity in the carboxymethyl cellulose (CMC)-plate assay. The phylogenetic tree based on sequence analysis of D1/D2 domains of the large subunit rRNA gene and the internal transcribed spacer (ITS) regions revealed that the strains $ON22^T$ (100 and 98.4% sequence similarities in D1/D2 domains and ITS) and $G10^T$ (99.8 and 99.5% in D1/D2 domain and ITS region) were most closely related to the species Moesziomyces aphidis JCM $10318^T$; $G15^T$ (100% in D1/D2 domains and ITS) belongs to the species Moesziomyces antarcticus JCM $10317^T$, respectively. Morphology and biochemical test results are provided in the species description. Cellulase with its massive applicability has been used in various industrial processes such as biofuels like bioethanol productions. Therefore, this is the first report of the cellulolytic yeast strains $ON22^T$, $G10^T$, and $G15^T$ related to the genus Moesziomyces in the family Ustilaginaceae (Ustilaginales), in Korea.

The Infuence of Venture Club Activity by University Student's Goal-Oriented Behavior Model on Self-determination and Startup Intention: Focused on the Medaiation Effects of Big 5 (벤처동아리활동 대학생의 목표 지향적 행동모델이 자기결정성 및 창업의지에 미치는 영향: 성격 5요인의 매개효과)

  • Park, Hwa Soon;Byun, Sang Hea
    • Asia-Pacific Journal of Business Venturing and Entrepreneurship
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    • v.16 no.2
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    • pp.79-93
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    • 2021
  • The question of why do you want to start a "start?" Is the most basic step in trying to do something. In other words, previous studies have shown that the degree of confidence in an individual's decision affects the setting of a specific purpose. Based on this, this study aims to provide basic data for deriving the direction of entrepreneurship education in college students by analyzing the effects of goal-oriented behavioral model on college students' self-determination and intention to start a business through the 5 factor model. To achieve the purpose of the study, a self-report questionnaire was conducted from October 01 to November 11, 2019 for university students attending located in Gyeonggi-do, Seoul. A total of 150 questionnaires were distributed, and 125 parts were used for the final analysis, except 25 parts with insincere responses or errors. Data were analyzed using SPSS Win 24, and reliability, validity analysis, frequency analysis, One-way ANOVA and regression analysis were performed, and three-step regression analysis and Sobel verification were performed for mediating effects. The summary of the study is as follows. First, the influence of university students' goal-oriented behavioral model on self-determination showed that attitudes, subjective norms, and perceived behavioral controls had statistically significant positive effects, and positive and negative expectations were statistically significant. Did not affect. Therefore, the higher the attitude, subjective norms, and perceived behavioral control, the higher the university students' self-determination. Second, the influence of college students' goal-oriented behavioral model on the intention to start a business was as follows.). As a result, the higher the perceived behavioral control and positive expectation, the higher the intention to start up. Third, regression model 1 showed that the behavioral control and positive expectation sentiment among the goal-oriented behavioral model had a significant positive influence on the college students' intention to start a business. Affected. Regression model II added the parameters of the 5 factor model, which increased 2.5% of explanatory power than the first regression model. Perceived behavioral control and positive expectations had a statistically significant positive effect, negative expectations had a statistically significant negative effect, and among the 5 factor model, openness had a statistically significant positive (+) Affected. From these results, it can be seen that the Big Five personality factors have a mediating effect on the relationship between goal-oriented behavior model and intention to start up. This study confirmed that the goal-oriented behavioral model of college students is an important variable in implementing self-determination and intention to start a business. In addition, by using his Big 5 personality factors as positive feedback, he has proved to play an important role by identifying the mediation role that can be set, planned and utilized to plan and achieve his life. The result of this study is that college students are interested in the intention of individual start-ups, so they are not freed from difficult employment difficulties. It is intended to provide basic data useful in the age of creation of government.

Natural Baseline Groundwater Quality in Shingwang-myeon and Heunghae-eup, Pohang, Korea (포항시 신광면 및 흥해읍 일대 지하수의 배경수질 연구)

  • Lee, Hyun A;Lee, Hyunjoo;Kwon, Eunhye;Park, Jonghoon;Woo, Nam C.
    • The Journal of Engineering Geology
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    • v.30 no.4
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    • pp.469-483
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    • 2020
  • The results of long-term groundwater level and quality monitoring can be used not only as the basic data for evaluating the impact of various disasters including climate change and establishing responses, but also as key data for predicting and managing geological disasters such as earthquakes. Some countries use groundwater level and quality monitoring for researches to predict earthquakes and to assess the impacts of the earthquake disaster. However, a few cases in Korea report on individual groundwater quality factors (i.e., dissolved ions) observed before and after the earthquakes, being different from other countries. To establish the abnormality criteria for groundwater quality in Pohang, groundwater samples were collected and analyzed five times from 14 agricultural or private wells existing in Shingwang-myeon and Heunghae-eup. As a result of the analysis, it was found that Ca2+ was the dominant cation in Shingwang-myeon, while Na+ was the dominant cation in Heunghae-eup. The elevated NO3- concentration in Shingwang-myeon is contributed to the agricultural activity in the area. A high concentration of Fe was detected in a well on Heunghae-eup; the concentration exceeded the drinking water standard by nearly 100 times. Relatively higher dissolved ions were observed in the groundwater of Heunghae-eup, and it is considered as the result of the flow velocity difference and water-rock reaction accompanying the difference in bedrock and sediment characteristics. The groundwater of Shingwang-myeon appeared to be most affected by the weathering of granite and silicates, while that of Heunghae-eup was mainly affected by the weathering of silicates and carbonate. The background concentrations (baselines) of groundwater Shingwang-myeon and Heunghae-eup was identified through the survey; however, the continuous monitoring is required to monitor the possible changes and the repeatability of seasonal variation.

Verification of the upper limit of results through dilution tests for RIA test (RIA 검사별 희석실험을 통한 결과의 상한치 검증)

  • LEE, Geun Ui;CHOI, Jin Ju;LEE, Young Ji;YOO, Seon Hee;LEE, Sun Ho
    • The Korean Journal of Nuclear Medicine Technology
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    • v.26 no.1
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    • pp.42-46
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    • 2022
  • Purpose In the meantime, there have been not many samples that require dilution, and it has been difficult for the examiner to set an appropriate dilution multiple for RIA test item and report the results. Accordingly, it was judged that it was necessary to set the maximum dilution multiple for each test and to verify the upper limit of the clinical reportable range. Therefore, in this study, the maximum dilution multiple for each RIA test was set and the upper limit of the clinical reportable range was verified accordingly Materials and Methods Among all RIA tests conducted at Asan Medical Center, the study treated on 30 types of tests which also conduct the dilution test. Data from March to July 2021 were collected and analyzed. The study was conducted on samples subjected to serial dilutions such as X2, X4 or X10, X102, X103, X104, X105. Results Among a total of 30 test types, 18 test types have more than 5 N values in the tolerance range of 80~120%. As a result of the verification of maximum dilution multiples, the test set to 104 is 𝛼-fetoprotein and thyroglobulin, and the test set to 103 is CA-125, CEA, and 𝛽-hCG, and the test set to 102 is Free PSA, PSA, CA15-3, SCC, Ferritin, PTH, Cortisol, and Calcitonin. Tests set to 10 include three categories: 𝛽2-Microglobulin, C-peptide, and Testosterone. Conclusion It is expected that it will contribute to improving the quality of nuclear medicine blood tests as the results of dilution experiments can be reported quickly and accurately through the verification of the clinical reportable range.

Performance State and Improvement Countermeasure of Primary Health Care Posts (보건진료소(保健診療所)와 업무실태(業務實態)와 개선방안(改善方案))

  • Park, Young-Hee;Kam, Sin;Han, Chang-Hyun;Cha, Byung-Jun;Kim, Tae-Woong;Gie, Jung-Aie;Kim, Byong-Guk
    • Journal of agricultural medicine and community health
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    • v.25 no.2
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    • pp.353-377
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    • 2000
  • This study was performed to investigate the performance state and improvement countermeasure of Primary Health care Posts(PHPs). The operation reports of PHPs(1996 330 PHPs, 1999 313 PHPs) located in Kyongsangbuk-Do and data collected by self-administered questionnaire survey of 280 community health practitioners(CHPs) were analyzed. The major results were as follows: Population per PHP in 1999 decreased in number compared with 1996. But population of the aged increased in number. The performance status of PHP in 1999 increased compared with 1996. A hundred forty one community health practitioners(50.4%) replied that the fiscal standing of PHP was good. Only 1.4% replied that the fiscal standing of PHP was difficult. For the degree of satisfaction in affairs, overall of community health practitioners felt proud. The degree of cooperation between PHP and public health institutions was high and the degree of cooperation of between PHP and private medical institutions was high. The degree of cooperation between PHP and Health Center was significantly different by age of CHP, the service period of CHP, and CHP's service period at present PHP. Over seventy percent of CHPs replied that they had cooperative relationship with operation council, village health workers, community organization. CHPs who drew up the paper on PHP's health activity plan were 96.4 % and only 11.4% of CHPs participated drawing up the report on the second community health plan. CHPs who grasped the blood pressure and smoking status of residents over 70% were 88.2%, 63.9% respectively and the grasp rate of blood pressure fur residents were significantly different according to age and educational level of CHP. CHPs received job education in addition continuous job education arid participated on research program in last 3 years were 27.5%, respectively. CHPs performed the return health program for residents in last 3years were 65.4%. Over 95% of CHPs replied that PHPs might be necessary and 53.9% of CHPs replied that the role of PHPs should be increased. CHPS indicated that major reasons of FHPs lockout were lack of understanding for PHP and administrative convenience, CHPs were officials in special government service governors intention of self-governing body. CHPs suggested number of population in health need such as the aged and patients with chronic disease, opinion of residents, population size, traffic situation and network in order as evaluation criteria for PHP and suggested results of health performance, degree of relationship with residents, results of medical examination anti treatment, ability for administration and affairs in order as evaluation criteria for CHP. CHPs replied that the important countermeasures for PHPs under standard were affairs improvement of PHPs and shifting of location to health weakness area in city. Over 50% of CHPs indicated that the most important thing for improvement of PHPs was affairs adjustment of CLIP. And CHPs suggested that health programs carried out in priority at PHP were management of diabetes mellitus and hypertention. home visiting health care, health care for the aged. The Affairs of BLIP should be adjusted to satisfy community health need and health programs such as management of diabetes mellitus and hypertention, home visiting health care, health care for the aged should be activated in order that PHPs become organization reflecting value system of primary health care.

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Development and Research into Functional Foods from Hydrolyzed Whey Protein Powder with Sialic Acid as Its Index Component - I. Repeated 90-day Oral Administration Toxicity Test using Rats Administered Hydrolyzed Whey Protein Powder containing Normal Concentration of Sialic Acid (7%) with Enzyme Separation Method - (Sialic Acid를 지표성분으로 하는 유청가수분해단백분말의 기능성식품 개발연구 - I. 효소분리로 7% Siailc Acid가 표준적으로 함유된 유청가수분해단백분말(7%)의 랫드를 이용한 90일 반복경구투여 독성시험 평가 연구 -)

  • Noh, Hye-Ji;Cho, Hyang-Hyun;Kim, Hee-Kyong
    • Journal of Dairy Science and Biotechnology
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    • v.34 no.2
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    • pp.99-116
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    • 2016
  • We herein performed animal safety assessment in accordance with Good Laboratory Practice (GLP) regulations with the aim of developing sialic acid from glycomacropeptide (hereafter referred to as "GMP") as an index ingredient and functional component in functional foods. GMP is a type of whey protein derived from milk and a safe food, with multiple functions, such as antiviral activity. A test substance was produced containing 7% (w/w) sialic acid and mostly-hydrolyzed whey protein (hereafter referred to as "7%-GNANA") by enzymatic treatment of substrate GMP. The maximum intake test dose level was selected based on 5,000 mg/kg/day dose set for male NOEL (no-observed-effect-level) and female NOAEL (no-observed-adverse-effect-level) determined by a dose-range finding (DRF) test (GLP Center of Catholic University of Daegu, Report No. 15-NREO-001) that was previously conducted with the same test substance. To evaluate the toxicity of a repeated oral dose of the test substance in connection with the previous DRF study, 1,250, 2,500, and 5,000 mg/kg of the substance were administered by a probe into the stomachs of 6-week-old SPF Sprague-Dawley male and female rats for 90 d. Each test group consisted of 10 male and 10 female rats. To determine the toxicity index, all parameters, such as observation of common signs; measurements of body weight and food consumption; ophthalmic examination; urinalysis, electrolyte, hematological, and serum biochemical examination; measurement of organ weights during autopsy; and visual and histopathological examinations were conducted according to GLP standards. After evaluating the results based on the test toxicity assessment criteria, it was determined that NOAEL of the test substance, 7%-GNANA, was 5,000 mg/kg/day, for both male and female rats. No animal death was noted in any of the test groups, including the control group, during the study period, and there was no significant difference associated with test substance, as compared with the control group, with respect to general symptoms, body weight changes, food consumption, ophthalmic examination, urinalysis, hematological and serum biochemical examination, and electrolyte and blood coagulation tests during the administration period (P<0.05). As assessed by the effects of the test substance on organ weights, food consumption, autopsy, and histopathological safety, change in kidney weight as an indicator of male NOAEL revealed up to 20% kidney weight increase in the high-dose group (5,000 mg/kg/day) compared with the change in the control group. However, it was concluded that this effect of the test substance was minor. In the case of female rats, reduction of food consumption, increase of kidney weight, and decrease of thymus weight were observed in the high-dose group. The kidney weight increased by 10.2% (left) and 8.9% (right) in the high-dose group, with a slight dose-dependency compared with that of the control group. It was observed that the thymus weight decreased by 25.3% in the high-dose group, but it was a minor test substance-associated effect. During the autopsy, botryoid tumor was detected on the ribs of one subject in the high-dose group, but we concluded that the tumor has been caused by a naturally occurring (non-test) substance. Histopathological examination revealed lesions on the kidney, liver, spleen, and other organs in the low-dose test group. Since these lesions were considered a separate phenomenon, or naturally occurring and associated with aging, it was checked whether any target organ showed clear symptoms caused by the test substance. In conclusion, different concentrations of the test substance were fed to rats and, consequently, it was verified that only a minor effect was associated with the test substance in the high-dose (5,000 mg/kg/day) group of both male and female rats, without any other significant effects associated with the test substance. Therefore, it was concluded that NOAEL of 7%-GNANA (product name: Helicobactrol) with male and female rats as test animals was 5,000 mg/kg/day, and it thus was determined that the substance is safe for the ultimate use as an ingredient of health functional foods.

Gender Differences in Pain in Cancer Patients (성별에 따른 암환자의 통증 차이)

  • Kim, Hyun-Sook;Lee, So-Woo;Yun, Young-Ho;Yu, Su-Jeong;Heo, Dae-Seog
    • Journal of Hospice and Palliative Care
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    • v.4 no.1
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    • pp.14-25
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    • 2001
  • Purpose : To determine whether there exist gender differences in pain in Korean cancer patients and whether the depression and performance that are often expressed differently between men and women with cancer interact with pain. Method : The results of survey were collected from 140 in- and out-patients (78 male and 62 female) who had cancer treatment at one of the university hospital in Seoul for four months from February of 1999. The severity and interference of pain were examined with the self-reported survey based on Korean version of Brief Pain Inventory (BPI-K). Demographic and clinical information for all patient were compiled by reviewing their medical records, and the level of depression was examined with the Korean version of Beck Depression Inventory (BDI-K). Usual statistical methods, e.g., frequences, means and SDs were used to characterize the sample. The chi-square tests for categorical data and t-test for numerical data were used for group comparison. And the correlation between variables were performed using Pearson correlation coefficient. Resuts : 1) The mean scores of the worst pain for last 24-hours measured with the pain severity of BPI-K were 5.77 in male and 6.45 in female. The pain interference of BPI-K in men was in the order of mood (5.49), enjoy (5.36), and work (5.00), and in women were work (7.48), enjoy (7.16), and mood (6.53). 2) In pain severity, significant difference was found between men and women in the average pain for last 24-hours (t=-2.130, P=.035). In pain interference, significant difference was found between men and women in activity (t=-2.450, P=.015), mood (t=-2,321, P=.022), walk (t=-2.762, P=.007), work (t=-4.946, P=.000), relate (t=-2.595, P=.010), sleep (t=-2.071, P=.040), enjoy (t=-3.198, P=.001). 3) It was found that the items of pain and depression are significantly correlated in men but not in women. Men also exhibited higher correlation in the items of pain and performance status than women. Conclusions : Women report significantly greater average pain for last 24-hours and for all items of pain interference than men. Pain and depression are significantly correlated in men. The results of this study suggest that gender differences in pain should be considered for planning effective pain management program.

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A Study on the System of Aircraft Investigation (항공기(航空機) 사고조사제도(事故調査制度)에 관한 연구(硏究))

  • Kim, Doo-Hwan
    • The Korean Journal of Air & Space Law and Policy
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    • v.9
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    • pp.85-143
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    • 1997
  • The main purpose of the investigation of an accident caused by aircraft is to be prevented the sudden and casual accidents caused by wilful misconduct and fault from pilots, air traffic controllers, hijack, trouble of engine and machinery of aircraft, turbulence during the bad weather, collision between birds and aircraft, near miss flight by aircrafts etc. It is not the purpose of this activity to apportion blame or liability for offender of aircraft accidents. Accidents to aircraft, especially those involving the general public and their property, are a matter of great concern to the aviation community. The system of international regulation exists to improve safety and minimize, as far as possible, the risk of accidents but when they do occur there is a web of systems and procedures to investigate and respond to them. I would like to trace the general line of regulation from an international source in the Chicago Convention of 1944. Article 26 of the Convention lays down the basic principle for the investigation of the aircraft accident. Where there has been an accident to an aircraft of a contracting state which occurs in the territory of another contracting state and which involves death or serious injury or indicates serious technical defect in the aircraft or air navigation facilities, the state in which the accident occurs must institute an inquiry into the circumstances of the accident. That inquiry will be in accordance, in so far as its law permits, with the procedure which may be recommended from time to time by the International Civil Aviation Organization ICAO). There are very general provisions but they state two essential principles: first, in certain circumstances there must be an investigation, and second, who is to be responsible for undertaking that investigation. The latter is an important point to establish otherwise there could be at least two states claiming jurisdiction on the inquiry. The Chicago Convention also provides that the state where the aircraft is registered is to be given the opportunity to appoint observers to be present at the inquiry and the state holding the inquiry must communicate the report and findings in the matter to that other state. It is worth noting that the Chicago Convention (Article 25) also makes provision for assisting aircraft in distress. Each contracting state undertakes to provide such measures of assistance to aircraft in distress in its territory as it may find practicable and to permit (subject to control by its own authorities) the owner of the aircraft or authorities of the state in which the aircraft is registered, to provide such measures of assistance as may be necessitated by circumstances. Significantly, the undertaking can only be given by contracting state but the duty to provide assistance is not limited to aircraft registered in another contracting state, but presumably any aircraft in distress in the territory of the contracting state. Finally, the Convention envisages further regulations (normally to be produced under the auspices of ICAO). In this case the Convention provides that each contracting state, when undertaking a search for missing aircraft, will collaborate in co-ordinated measures which may be recommended from time to time pursuant to the Convention. Since 1944 further international regulations relating to safety and investigation of accidents have been made, both pursuant to Chicago Convention and, in particular, through the vehicle of the ICAO which has, for example, set up an accident and reporting system. By requiring the reporting of certain accidents and incidents it is building up an information service for the benefit of member states. However, Chicago Convention provides that each contracting state undertakes collaborate in securing the highest practicable degree of uniformity in regulations, standards, procedures and organization in relation to aircraft, personnel, airways and auxiliary services in all matters in which such uniformity will facilitate and improve air navigation. To this end, ICAO is to adopt and amend from time to time, as may be necessary, international standards and recommended practices and procedures dealing with, among other things, aircraft in distress and investigation of accidents. Standards and Recommended Practices for Aircraft Accident Injuries were first adopted by the ICAO Council on 11 April 1951 pursuant to Article 37 of the Chicago Convention on International Civil Aviation and were designated as Annex 13 to the Convention. The Standards Recommended Practices were based on Recommendations of the Accident Investigation Division at its first Session in February 1946 which were further developed at the Second Session of the Division in February 1947. The 2nd Edition (1966), 3rd Edition, (1973), 4th Edition (1976), 5th Edition (1979), 6th Edition (1981), 7th Edition (1988), 8th Edition (1992) of the Annex 13 (Aircraft Accident and Incident Investigation) of the Chicago Convention was amended eight times by the ICAO Council since 1966. Annex 13 sets out in detail the international standards and recommended practices to be adopted by contracting states in dealing with a serious accident to an aircraft of a contracting state occurring in the territory of another contracting state, known as the state of occurrence. It provides, principally, that the state in which the aircraft is registered is to be given the opportunity to appoint an accredited representative to be present at the inquiry conducted by the state in which the serious aircraft accident occurs. Article 26 of the Chicago Convention does not indicate what the accredited representative is to do but Annex 13 amplifies his rights and duties. In particular, the accredited representative participates in the inquiry by visiting the scene of the accident, examining the wreckage, questioning witnesses, having full access to all relevant evidence, receiving copies of all pertinent documents and making submissions in respect of the various elements of the inquiry. The main shortcomings of the present system for aircraft accident investigation are that some contracting sates are not applying Annex 13 within its express terms, although they are contracting states. Further, and much more important in practice, there are many countries which apply the letter of Annex 13 in such a way as to sterilise its spirit. This appears to be due to a number of causes often found in combination. Firstly, the requirements of the local law and of the local procedures are interpreted and applied so as preclude a more efficient investigation under Annex 13 in favour of a legalistic and sterile interpretation of its terms. Sometimes this results from a distrust of the motives of persons and bodies wishing to participate or from commercial or related to matters of liability and bodies. These may be political, commercial or related to matters of liability and insurance. Secondly, there is said to be a conscious desire to conduct the investigation in some contracting states in such a way as to absolve from any possibility of blame the authorities or nationals, whether manufacturers, operators or air traffic controllers, of the country in which the inquiry is held. The EEC has also had an input into accidents and investigations. In particular, a directive was issued in December 1980 encouraging the uniformity of standards within the EEC by means of joint co-operation of accident investigation. The sharing of and assisting with technical facilities and information was considered an important means of achieving these goals. It has since been proposed that a European accident investigation committee should be set up by the EEC (Council Directive 80/1266 of 1 December 1980). After I would like to introduce the summary of the legislation examples and system for aircraft accidents investigation of the United States, the United Kingdom, Canada, Germany, The Netherlands, Sweden, Swiss, New Zealand and Japan, and I am going to mention the present system, regulations and aviation act for the aircraft accident investigation in Korea. Furthermore I would like to point out the shortcomings of the present system and regulations and aviation act for the aircraft accident investigation and then I will suggest my personal opinion on the new and dramatic innovation on the system for aircraft accident investigation in Korea. I propose that it is necessary and desirable for us to make a new legislation or to revise the existing aviation act in order to establish the standing and independent Committee of Aircraft Accident Investigation under the Korean Government.

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