Journal of The Korean Society of Inherited Metabolic disease
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v.18
no.2
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pp.43-49
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2018
Maple syrup urine disease (MSUD, OMIM#248600) is a rare and autosomal recessively-inherited metabolic disorder that is caused by mutations in the branched-chain ${\alpha}$-ketoacid dehydrogenase (BCKDH) genes. It prevents the normal breakdown of branched-chain amino acids (BCAAs), such as leucine, isoleucine, and valine, and leads to poor feeding, lethargy, abnormal movements, seizure, and death if untreated. Here, we report the case of a Korean newborn of biochemically- and genetically-confirmed MSUD manifesting lethargy and central apnea, the acute state of which was successfully treated. The molecular genetic investigation revealed two novel heterozygous mutations (p.Ala32Phefs*48 and p.Val 130Phe) in BCKDHB, and both parents were confirmed as carriers. We emphasize the importance of early diagnosis and prompt introduction of specific treatment for MSUD in life saving and prognosis.
This study was carried out unmanned aerial photography and terrestrial laser scanning to establish digital database on original wall of Gyejoksanseong fortress, and measured ground control points for continuity of the monitoring. It also performed precise examination with the naked eye, unmanned aerial photogrammetry, endoscopy, total station and handy measurement to examine the structural stability of the original walls. The ground control points were considered as a point where visual field can be secured, 3 points were selected around each of the south and north walls. For the right side of the south original wall, aerial photogrammetry was conducted using drones and a deviation analysis of 3-dimensional digital models was performed for short-term monitoring. As a result, the two original walls were almost matched in range within 5mm, and no difference indicating displacement of stones was found, except for partial deviation. Regular monitoring of the areas with structural deformation such as bulging, weak and fracture zone by precisely examining with the naked eye and using high-resolution photo data revealed no distinct change. The inner foundation observed through endoscopy found out that filling stones of the original walls were still remained, while most filling soil was lost. As a result of measuring the total station focusing around the points with structural deformation on the original walls, the maximum displacements of the north and south walls were somewhat high with 6.6mm and 3.8mm, respectively, while the final displacements were relatively stable at below 2.9mm and 1.4mm, respectively. Handy measurement also did not reveal clear structural deformation with displacements below 0.82mm at all points. Even though the results of displacement monitoring on the original walls are stable, it is hard to secure structural stability due to the characteristics of ramparts where sudden brittle fracture occurs. Therefore, it is necessary to conduct conservational scientific diagnosis, precise monitoring, and structural analysis based on the 3-dimensional figuration information obtained in this research.
In this study, a real-time storage level and capacity monitoring and forecasting system for Yongdam Dam watershed was developed using high resolution satellite image. The drought indices such as Standardized Precipitation Index (SPI) from satellite data were used for storage level monitoring in case of drought. Moreover, to predict storage volume we used a statistical method based on Principle Component Analysis (PCA) of Singular Spectrum Analysis (SSA). According to this study, correlation coefficient between storage level and SPI (3) was highly calculated with CC=0.78, and the monitoring and predictability of storage level was diagnosed using the drought index calculated from satellite data. As a result of analysis of principal component analysis by SSA, correlation between SPI (3) and each Reconstructed Components (RCs) data were highly correlated with CC=0.87 to 0.99. And also, the correlations of RC data with Normalized Water Surface Level (N-W.S.L.) were confirmed that has highly correlated with CC=0.83 to 0.97. In terms of high resolution satellite image we developed a water detection algorithm by applying an exponential method to monitor the change of storage level by using Multi-Spectral Instrument (MSI) sensor of Sentinel-2 satellite. The materials of satellite image for water surface area detection in Yongdam dam watershed was considered from 2016 to 2018, respectively. Based on this, we proposed the possibility of real-time drought monitoring system using high resolution water surface area detection by Sentinel-2 satellite image. The results of this study can be applied to estimate of the reservoir volume calculated from various satellite observations, which can be used for monitoring and estimating hydrological droughts in an unmeasured area.
Moon, Na Hyun;Shin, Man Yong;Moon, Ga Hyun;Chun, Junghwa
Korean Journal of Agricultural and Forest Meteorology
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v.21
no.3
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pp.121-134
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2019
This study was conducted to review the trends of forest meteorological studies based on the publications for last 20 years in Korean Journal of Agricultural and Forest Meteorology (KJAFM), and to provide insight for future prospect for researches in the field of forest meteorology. A total of 220 papers related to forest meteorology were published in KJAFM for the last 20 years. That corresponds to 33.5% out of all the papers including agricultural meteorology papers. To review the trends of forest meteorology studies, the 220 published papers were classified into seven categories. They are forest meteorology and forest fire, forest meteorology and tree physiology, forest meteorology and forest protection, micrometeorology in mountain area, climate and forest growth, climate and forest vegetation distribution, and climate change and forest ecosystem. Even if there were differences in paper numbers among the seven categories, it was found that various and very specific studies were conducted in the field of forest meteorology for the last 20 years. It was also expected that the accumulation and utilization of various and accurate forest meteorological information would bring remarkable progress of forest meteorological studies in the near future.
This paper finds a solution in the social context which cannot be explained thoroughly by well-timed release date, revival of comedy films, and the attraction of Lee Byeong-heon's comedy etc. while it throws question of how the film, Extreme Job captivated 16 million audience. The incredible hits of Extreme Job cannot be explained by analyzing the text alone. After this essay investigates a function and a role of comedy as a public sphere, it examines people's desires and wishes in the comedy and other genres since 2008 when the conservative government has seized power. Since 2008 a series of dark tone's action thriller, social problem film, and disaster film have emerged, these genres showed absence of public security, crisis of democracy and criticism against rulling class. On the other hand, hit comedy films have showed escapism such as weepie, nostalgia, and fantasy at the same time, generally. Although Veteran (2015) is not full-blown comedy, after this film's big success, "comic mode" has gradually revived. A light tone's films which are truer to genre rules has started representing the wishes of people toward social reforms and changes. Meanwhile, "Candlelight Protest" served as a momentum to recover the democracy which has been in crisis, but it could not lead changes in economic and daily lives. Exreme Job can be read as a question how we will survive since "Candlight Protest." The lives of detectives as self-employed workers who has taken over a fried chicken restaurant for going undercover are appearances of ordinary persons who must survive in the edless conpetition. Furthermore, this film shows a dream of a "great success myth" which becomes well-known as a famous restaurant and a self-management such as brand-naming and an exapansion of franchise business. We can read ganster's chicken franchises as a huge distribution industry which disturbs market system by delivering drugs secretly. While applauses that we give to the police having identities of self-employed workers which sweeps the ganster are giving support to oridinary neighborhood like us, they are also wishes of people who long for the restoration of publicness of police in the market which is becoming increasingly privatized today. A significance of this essay is to examine Extreme Job in terms of the geography of film genres and the revival of comic mode sicne 2008 at the macro level, and is to read the film in the perspective of the problems of economic and daily lives which has been still unsolved since "Candlelight Protest" at the micro level.
Purpose: To examine the relationship between total fat infiltration (TFI) rate, which quantifies the reduction of muscles around the spine and is an important factor for sarcopenia, and the factors affecting osteoporotic vertebral compression fracture. Materials and Methods: Patients treated for osteoporotic compression fractures of the lumber spine from January 2012 to December 2016 were analyzed retrospectively. Among them, this study included ninety-eight patients who were 1) diagnosed with osteoporosis with a bone mineral density (BMD) T score of less than 2.5 g/cm2, 2) received vertebroplasty or kyphoplasty for lumbar fractures, 3) involved one segment of the lumbar spine, and 4) were followed-up for more than one year. The TFI rate confirmed by analyzing magnetic resonance imagings with the Image J program was studied. Based on this, the relationship between the TFI of the multifidus and erector spinae muscles and the factors of osteoporosis were analyzed. Results: The mean TFI of the multifidus and erector spinae was 14.66±10.16. The spine BMD showed a positive correlation with the hip BMD, but a negative correlation with the TFI. A positive correlation was observed between the hip BMD and body mass index. In addition, vitamin D was positively correlated with both the hip and spine BMD but negatively correlated with the TFI rate. Conclusion: Muscle growth helps treat osteoporosis, and can prevent fractures that occur frequently in osteoporosis patients. Increasing the vitamin intake can also slow the progression of muscle atrophy.
Purpose: To evaluate the prognostic factors affecting poor functional outcomes in patients with retear after rotator cuff repair. Materials and Methods: From January 2013 to December 2018, among 631 patients who underwent arthroscopic repair of a rotator cuff tear, 42 patients, who could be followed-up for more than one year and showed a retear of the repaired cuff on magnetic resonance imaging (MRI), were collected retrospectively. The preoperative demographic data, range of motion, American Shoulder and Elbow Surgeons (ASES) score, fatty degeneration, and tear progression on postoperative MRI, as well as other factors that could affect the clinical outcomes, were analyzed. Patients who scored <80 points on the ASES score were allocated to the poor function group. The risk factors for poor clinical outcomes were compared with the group with ASES scores of 80 or above. Results: The postoperative functional results in the group with retear (n=42) after arthroscopic rotator cuff repair showed significant improvement. Univariate analysis revealed the preoperative visual analogue scale (VAS) score and tear progression to have associations with a poor shoulder function. In addition, subscapularis repair was found to be associated with a good shoulder function. The preoperative VAS score and tear progression except for subscapularis repair were independent factors associated with poor clinical outcomes according to multivariate logistic regression analysis. Conclusion: In patients with retear after rotator cuff repair, the preoperative VAS and tear progression in postoperative MRI are factors predicting a poor functional outcome.
Huh, Soon Ho;Choi, Byeong Yeol;Han, Sang Roc;Chung, Woo Chull
Journal of the Korean Orthopaedic Association
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v.56
no.2
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pp.125-133
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2021
Purpose: The clinical outcomes were investigated to determine if arthroscopic management is a useful method for 19 hips with iliopsoas tendon impingement (IPI) after total hip arthroplasty (THA). Materials and Methods: Eighteen patients (19 hips), who complained of groin pain and flexion pain that persisted after THA from September 2013 to December 2019, were the subjects of this investigation. The mean time to manifestation after THA was four months (range, 1-9 months) in patients of an average age of 60 years (range, 50-69 years). Thirteen out of 18 patients underwent THA using the direct anterior approach and five by the lateral approach. IPI was diagnosed by the medical history, physical examination, blood test, radiographic examination using X-ray and computed tomography, and topical injection therapy. All patients underwent arthroscopic treatment and a dynamic arthroscopic physical examination after exposure to the iliopsoas tendon revealed impingement. Tenotomy was then performed on the muscle portion through the total tendon portion. Symptoms and pain levels of preoperative, postoperative and follow-up period were investigated and compared. Results: The Western Ontario and McMaster Universities Osteoarthritis Index score decreased from an average of 58.4 (range, 40-88) before surgery to an average of 35.0 (range, 15-76) after surgery. Similarly, the visual analogue scale decreased from an average of 4.0 (range, 2-6) before surgery to an average of 1.4 (range, 0-4) after surgery. Sixteen patients (88.9%) showed pain relief and improvement in the straight leg raise test, and two patients showed postoperative muscle weakness and sustained pain. In the follow-up period, muscle weakness improved. One patient underwent arthroscopic iliopsoas tenotomy at the lesser trochanteric level but the symptoms persisted. The clinical symptoms were improved after one more tenotomy at the joint level. Conclusion: Arthroscopic iliopsoas tenotomy performed in patients with IPI after THA showed good clinical results.
Suebin Choi;Chankue Park;Jonghyun Shin;Taesung Jeong;Eungyung Lee
Journal of the korean academy of Pediatric Dentistry
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v.50
no.3
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pp.318-333
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2023
The purpose of this study is to analyze changes in dental trauma in children under the age of 12 during the period of Coronavirus Disease 2019 (COVID-19). March 2020, when COVID-19 was officially declared a pandemic by the World Health Organization, was set as the starting point for COVID-19. From March 2018 to February 2020, subjects in the pre-COVID-19 period were classified as the Pre-COVID-19 group, and from March 2020 to March 2022, subjects in the post-COVID-19 period were classified as the COVID-19 group. Information related to trauma was collected through electronic medical records. The number of trauma patients before and after the outbreak of COVID-19 decreased significantly. During the COVID-19 period, there was no significant difference in the male-female ratio or the distribution order of age groups. In the COVID-19 group of permanent teeth, the ratio of trauma caused by personal mobility was higher than trauma caused by sports. In the COVID-19 group of permanent teeth, the ratio of crown fracture with pulp involvement was significantly higher than the ratio of crown fracture without pulp involvement. Changes in trauma patterns caused by COVID-19 were observed more clearly in school-aged children than in preschool children. In a pandemic situation such as COVID-19, it is expected to be used as a good educational basis for knowing that frequent diagnoses can change due to changes in the environment.
Purpose: The purpose of this study was to analyze the radiological and clinical outcomes in elderly patients with unstable intertrochanteric femur fractures in accordance with the length of intramedullary nail. Materials and Methods: Between August 2009 and December 2014, a total of 139 patients-older than 65 years of age with AO/OTA classification of 31-A2 unstable intertrochanteric femur fracture-who has been followed-up for at least 1 year after the treatment with internal fixation by using an intramedullary nail were enrolled for this retrospective control study. The subjects were classified into two groups according to the length of intramedullary nail: 106 patients in the short group (group I) and 33 patients in the long group (group II). For radiological assessments, the reduction state, time to union, and implant related complications were examined. The clinical outcomes were assessed by preoperative hemoglobin, operating time, intraoperative bleeding amount, blood transfusion rate, hospitalization period, and Charnley hip pain scoring system at the final follow-up. Results: The postoperative radiographs showed good or acceptable reduction in all cases. The mean time of radiologic bone union was 4.8 months, and there was no difference between the two groups. With respect to surgical time, the group II was found to take longer (57.87 minutes) than the group I (45.65 minutes) (p=0.003). The bleeding amount during surgery of the group II was greater (288.78 ml) than that of the group I (209.90 ml) (p=0.046). The clinical results at the final follow-up were found to be satisfactory in both groups. Conclusion: In cases of good reduction of the fracture from the treatment of unstable intertrochanteric femur fracture accompanying the posteromedial fragment in elderly patients, both groups-long and short intramedullary nails-showed satisfactory radiological and clinical outcomes.
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