• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.25-30
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• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

• Journal of Intelligence and Information Systems
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• v.17 no.4
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• pp.143-155
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• 2011

Lately electronic tagging policy for the sexual offenders was introduced in order to reduce and prevent sexual offences. However, most sexual offences against children happening these days are committed by the tagged offenders whose identities have been released. So, for the crime prevention, we need measures with which we could minimize the suffers more promptly and actively. This paper suggests a new system to relieve the sexual abuse related anxiety of the children and solve the problems that electronic bracelet has. Existing bracelets are only worn by serious criminals, and it's only for risk management and positioning, there is no way to protect the children who are the potential victims of sexual abuse and there actually happened some cases. So we suggest also letting the students(children) wear the LBS(Location Based Service) and USN(Ubiquitous Sensor Network) technology based electronic bracelets to monitor and figure out dangerous situations intelligently, so that we could prevent sexual offences against children beforehand, and while a crime is happening, we could judge the situation of the crime intelligently and take swift action to minimize the suffer. And by checking students' attendance and position, guardians could know where their children are in real time and could protect the children from not only sexual offences but also violent crimes against children like kidnapping. The overall system is like follows : RFID Tag for children monitors the approach of offenders. While an offender's RFID tag is approaching, it will transmit the situation and position as the first warning message to the control center and the guardians. When the offender is going far away, it turns to monitoring mode, and if the tag of the child or the offender is taken off or the child and offender stay at one position for 3~5 minutes or longer, then it will consider this as a dangerous situation, then transmit the emergency situations and position as the second warning message to the control center and the guardians, and ask for the dispatch of police to prevent the crime at the initial stage. The RFID module of criminals' electronic bracelets is RFID TAG, and the RFID module for the children is RFID receiver(reader), so wherever the offenders are, if an offender is at a place within 20m from a child, RFID module for children will transmit the situation every certain periods to the control center by the automatic response of the receiver. As for the positioning module, outdoors GPS or mobile communications module(CELL module)is used and UWB, WI-FI based module is used indoors. The sensor is set under the purpose of making it possible to measure the position coordinates even indoors, so that one could send his real time situation and position to the server of central control center. By using the RFID electronic roll book system of educational institutions and safety system installed at home, children's position and situation can be checked. When the child leaves for school, attendance can be checked through the electronic roll book, and when school is over the information is sent to the guardians. And using RFID access control turnstiles installed at the apartment or entrance of the house, the arrival of the children could be checked and the information is transmitted to the guardians. If the student is absent or didn't arrive at home, the information of the child is sent to the central control center from the electronic roll book or access control turnstiles, and look for the position of the child's electronic bracelet using GPS or mobile communications module, then send the information to the guardians and teacher so that they could report to the police immediately if necessary. Central management and control system is built under the purpose of monitoring dangerous situations and guardians' checking. It saves the warning and pattern data to figure out the areas with dangerous situation, and could help introduce crime prevention systems like CCTV with the highest priority. And by DB establishment personal data could be saved, the frequency of first and second warnings made, the terminal ID of the specific child and offender, warning made position, situation (like approaching, taken off of the electronic bracelet, same position for a certain time) and so on could be recorded, and the data is going to be used for preventing crimes. Even though we've already introduced electronic tagging to prevent recurrence of child sexual offences, but the crimes continuously occur. So I suggest this system to prevent crimes beforehand concerning the children's safety. If we make electronic bracelets easy to use and carry, and set the price reasonably so that many children can use, then lots of criminals could be prevented and we can protect the children easily. By preventing criminals before happening, it is going to be a helpful system for our safe life.

• The Korean Journal of Air & Space Law and Policy
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• v.30 no.2
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• pp.403-425
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• 2015

The Chicago Convention and Annexes have become the basis of aviation safety regulations for every contracting state. Generally, the State's aviation safety regulations refer to the Standards and Recommended Practices(SARPs) provided in the Annexes of the Chicago Convention. In order to properly reflect international aviation safety regulations, constant studies of the aviation fields are of paramount importance. This Paper is intended to identify the main differences between korean and foreign regulation and suggest a few amendment proposals on Mixed-fleet Flying(at or more two aircraft type operation) of flight crew. Comparing with these regulations, the korean regulations and implementations have some insufficiency points. I suggest some amendment proposals of korean regulations concerning Mixed-fleet Flying that flight crew operate aircraft of different types. Basically an operator shall not assign a pilot-in-command or a co-pilot to operate at the flight controls of a type of airplane during take-off and landing unless that pilot has operated the flight controls during at least three take-offs and landings within the preceding 90 days on the same type of airplane or in a flight simulator. Also, flight crew members are familiarized with the significant differences in equipment and/or procedures between concurrently operated types. An operator shall ensure that piloting technique and the ability to execute emergency procedures is checked in such a way as to demonstrate the pilot's competence on each type or variant of a type of airplane. Proficiency check shall be performed periodically. When an operator schedules flight crew on different types of airplanes with similar characteristics in terms of operating procedures, systems and handling, the State shall decide the requirements for each type of airplane can be combined. In conclusion, it is necessary for flight crew members to remain concurrently qualified to operate multiple types. The operator shall have a program to include, as a minimum, required differences training between types and qualification to maintain currency on each type. If the Operator utilizes flight crew members to concurrently operate aircraft of different types, the operator shall have qualification processes approved or accepted by the State. If applicable, the qualification curriculum as defined in the operator's Advanced Qualification Program could be applied. Flight crew members are familiarized with the significant differences in equipment and/or procedures between concurrently operated types. The difference among different types of airpcrafts decrease and standards for these airpcrafts can be applied increasingly because function and performance have been improved by aircraft manufacture company in accordance to basic aircraft system in terms of developing new aircrafts for flight standard procedure and safety of flight. Also, it becomes more necessary for flight crews to control multi aircraft types due to various aviation business and activation of leisure business. Nevertheless, in terms of flight crew training and qualification program, there are no regulations in Korea to be applied to new aircraft types differently in accordance with different levels. In addition, it has no choice different programs based on different levels because there are not provisions to restrict or limit and specific standards to operate at or more than two aircraft types for flight safety. Therefore the aviation authority introduce Flight Standardization and/or Operational Evaluation Board in order to analysis differences among aircraft types. In addition to that, the aviation authority should also improve standard flight evaluation and qualification system among different aircraft types for flight crews to apply reasonable training and qualification efficiently. For all the issue mentioned above, I have studied the ICAO SARPs and some state's regulation concerning operating aircraft of different types(Mixed-fleet flying), and suggested some proposals on the different aircraft type operation as an example of comprehensive problem solving. I hope that this paper is 1) to help understanding about the international issue, 2) to help the improvement of korean aviation regulations, 3) to help compliance with international standards and to contribute to the promotion of aviation safety, in addition.