• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.12 no.1
• /
• pp.42-48
• /
• 2012

Pompe disease is a rare lysosomal glycogen storage disorder caused by a total or partial deficiency of the acid ${\alpha}$-glucosidase (GAA) enzyme due to the GAA gene mutations. The classic infantile form of Pompe disease is a rapidly progressive multi-organ disease with hypotonia, generalized muscle weakness, and hypertrophic cardiomyopathy, usually leading to death in the first 2 years of life. Enzyme replacement therapy with recombinant human GAA has been shown to be effective and subsequently yielded promising results. Here, we present clinical and genetic characteristics of three Korean non-classic infantile Pompe patients, and the short term efficacy of enzyme replacement therapy. Considering that enzyme replacement therapy can change the natural course of infantile Pompe disease, early diagnosis and early initiation of treatment is critical to improving patient outcomes.

• Journal of Chest Surgery
• /
• v.31 no.1
• /
• pp.46-51
• /
• 1998

Treatment of esophageal perforation when diagnosed late remains controversial. Ten consecutive patients since 1990 were treated late(later than 24 hours) for esophageal perforation with primary repair. Four perforations were iatrogenic, 3 were spontaneous, 2 were foreign body aspiraton and 1 was trauma. The interval from perforation to operation was 116 hours in mean and 48 hours in median value. The principles of repair included (1) a local esophagomyotomy proximal and distal to the tear to expose the mucosal defect and intact mucosa beyond, (2) debridement of the mucosal defect and closure, (3) reapproximation of the muscle, and (4) adequate drainage. The repair was buttressed with parietal pleura or pericardial fat in 9 patients. Associated distal obstruction was treated with dilation and esophagomyotomy intraoperatively. There was one mortality and cause of death was massive gastric bleeding due to gastric ulcer on 33rd day after operation. Five patients had leak at the site of repair and these cases were treated completely with conservative treatment except a mortality case. In conclusion, in the absence of malignant or irreversible distal obstruction, meticulous repair of perforated esophagus and adequate drainage are preferred approach, regardless of the duration from the injury to the operation.

• Proceedings of the Korea Water Resources Association Conference
• /
• 2010.05a
• /
• pp.952-956
• /
• 2010

저수지에 설치된 물순환장치는 저수지 수문상황과 자연조건의 변화에 따라 유동특성이 상이하므로 연구자가 고려하는 다양한 조건에서 현장 측정하기는 불가능하다. 이런 문제를 대체하는 방안으로 전산유체유동(CFD) 모형을 적용한 모사를 실시함으로서 다양한 조건에 따른 효과를 평가할수 있게 된다. 본 연구에서는 전산유체유동을 통한 대류식 물순환장치의 유동영향범위와 수질변화 등을 평가하고, 다양한 조건에서 모사를 실시하여 최적운영방안을 도출하고 실제운영에 활용토록 하고자 한다. 수체거동을 모사하기 위해 실제 저수지를 형상화한 Domain을 3가지로 구성하였다. 첫번째는 반경 20m, 깊이 40m Domain에 물순환장치를 중앙에 설치한 것이며(D20), 두 번째는 반경 40m, 깊이 40m에 두 개의 물순환장치를 양쪽에 설치하였고(D40), 세 번째는 반경 100m, 깊이 40m로 설정(D100)하였고 양쪽에 두 개의 물순환장치를 설치한 것으로 구성하였다. CFD에 의한 개별 대류식 순환장치의 유동모사결과 D20은 시간의 경과에 따라 수온성층이 하강하는 현상이 나타났으며 이러한 결과로 판단할때 40m 간격으로 대류식 순환장치를 설치하여 운영하면 탈성층이 나타날 수 있을것으로 판단되었다. D40에 대하여 CFD에 의한 유동모사를 실시하였으며 시간의 경과에 따라 수온성층의 변화는 나타나지 않았다. 이러한 결과로 판단할때 40m 간격으로 한줄로 대류식 순환장치를 설치하여 운영하면 성층을 깨는 현상은 나타나지 않을 것으로 평가되었다. 반면 Dye테스트시 심층에서 상승한 수체는 수온성층 표면에서 수평방향으로 계속 퍼져 나가면서 옆장치에서 상승된 수체와 혼합이 활발히 이루어 지는 현상을 나타내었다. 장치간 거리가 100m인 대류식 물순환장치에 의한 유동모사시 수온성층의 변화는 전혀 나타나지 않았으며, Dye테스트시 심층에서 상승한 수체는 수온성층 위에서 수평방향으로 퍼져 나가면서 옆에서 가동되는 장치에서 상승된 수체와 혼합 현상을 나타내기는 하나 D40보다 혼합시간이 더 걸리는 것으로 나타났다.

• Proceedings of the Korean Society of Disaster Information Conference
• /
• 2017.11a
• /
• pp.329-330
• /
• 2017

재난은 인류의 발전과 더불어 다양하게 발생되어왔다. 최근 21세기 들어 발생하는 재난은 그 현상이 더욱 다양하고 복잡하며 그 피해의 정도가 대규모로 진행되는 경우가 많아 졌다. 태풍, 집중호우, 강풍, 폭설, 지진, 황사 등의 자연재난은 지구온난화현상으로 국내뿐만 아니라 전 세계적으로 커다란 영향력을 미치고 있으며, 화재, 구축물의 붕괴, 지하철사고, 테러 및 감염병 등의 사회 재난도 더욱더 증가하고 있는 추세이다. 나아가 사스, 메르스, 지카 바이러스 및 대형 테러 등 전 세계적 차원의 사회 재난의 발생 또한 급증하고 있다. 이와 같이 현대사회에서 발생하는 재난는 개인이나 국가의 정책만으로 예방하고 관리할 수 없는 특성이 있다. 따라서, 자본주의 경제의 한축을 이루고 있는 기업의 재난을 관리하여 연속성을 확보하고 더나아가 리질리언스(Resilence)를 보장하기 위한 일환으로 2007년 7월 재해경감을 위한 기업의 자율 활동 지원에 관한 법률(이하, 기업재해경감법 이라함.)이 공포된 지 약 10여년이 경과하였다. 이는 법 제도화를 통하여 기업의 재해경감활동을 지원하기 취지에서 출발한 것 이다. 그 후 현재 까지 어느 정도의 발전이 있었지만 경과 년 수에 비해 아직 미흡한 실정이다. 이후 그 기업들이 좀 더 원활하게 재해경감활동을 위한 계획을 수립, 실행, 검토 및 유지하기 위한 지침으로서 기업재난관리표준을 2010년 4월 2일 제정하였고 이후 재해경감활동 수립계획을 제정하여 시행하기에 이르렀다. 또한, 기업재난관리표준은 제정된 이후 2013년 12월 9일 전면 개정작업을 통해 경영관리 프로세스 모델(Plan-Do-Check-Act)이 적용된 관리체계를 접목시키고 용어정의를 국제표준과 일치시켜 기업재해경감활동 상의 혼선을 방지하기 위한 노력을 하였다. 현 정부에서는 주무부처가 바뀌고 조직이 변화되어 행정안전부고시 제2017-1호(2017.07.26.일)로 개정되어 현재에 이르고 있다. 본 논문에서는 기업재난관리표준과 연관된 제반 법령 및 규정 등에 대한 법적성격 과 지위를 규명하고, 기업재난관리표준의 관련분야 적용 시 문제점 및 바람직한 제도개선 방향을 제시하고자 한다.

• KOREAN JOURNAL OF CROP SCIENCE
• /
• v.37 no.4
• /
• pp.372-376
• /
• 1992

This experiment was conducted to determine hay making time on the drying period, hay moisture content, hay loss content, yield and forage quality of Italian ryegrass in paddy field. Plant height increased rapidly from middle of April, and leaf weight rate were 55.6% at April 20 and 18.9% at May 30. Moisture content and loss content of hay were 17.1-20.5% and 10.2-15.0%, respectively, at drying for 4 days from May 10 to May 30. As the growth stage later, crude protein, fat and ash contents reduced, but crude fiber and NFE contents increased. Dry matter and TDN yield at the cutting of May 20 and May 30 increased to 17-18% and 5-11%, respectively, than those of April 20＋May 30.

• Journal of Korean Society of Forest Science
• /
• v.83 no.4
• /
• pp.545-555
• /
• 1994

The objective of the study was to provide the useful scientific data on the early rehabilitation of the legging road after timber harvesting in the forest area. This study was carried out at logging roads which were constructed during 1989 and 1994 in Mt. Baekwoon. The field survey was conducted in July, 1991. Judging from the analysis of soil bulk density, time required for recovery as the undisturbed forest soil condition was more than 10 years in the road which was left, and the regression equation is as follows, $$Y_1=1.4195-0.0744{\cdot}X(R^2=0.91)$$ $$Y_2=1.4673-0.0688{\cdot}X(R^2=0.73)$$ (X : elapsed year after road construction. $Y_1$, $Y_2$ : soil bulk density($g/cm^3$) at 0~7.5cm, and 7.5~15.0cm, respectively) Especially soil bulk density with buffer strip-woods was $0.890-0.903g/cm^3$, so it was 20% lower than that of logging road surface without buffer strip-woods. Among the 7 factors, location, sand content, and soil hardness had statistically significant effect on the soil bulk density in logging road surface. The pioneer species on logging road surface were Rhus cratargifolius, Prunus chinensis, and Lespedeza cyrtobotrya, etc. in woody species, and Pteridium aquilinum, Arundinella hirta, and Lysimachia clethroides, etc. in herb species. So, in process of year, average plant coverage were 70% on cutting and banking slope and 20% on logging road surface which elapsed 6 years after logging road construction. Through this research, buffer strip-woods must be remained for environmental conservation of forest conditions, and from the time to be closed the road, planting, seeding, and grazing works could be effective to the soil condition and vegetation recovery.

• Clinical and Experimental Pediatrics
• /
• v.51 no.9
• /
• pp.987-991
• /
• 2008

Purpose : Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis (HNL), is a self-limited disease characterized by cervical lymphadenopathy and fever. The etiology of KFD remains unknown; however, the self-limiting nature of HNL suggests the cause of this disease could be viral infection. For this reason, several viruses have been evaluated as possible etiologies of HNL, including Epstein-Barr virus (EBV), human herpesvirus 6 (HHV6), human herpesvirus 8 (HHV8), and cytomegalovirus (CMV). The aim of this study was to examine the relationship of EBV and HHV6 to HNL. Methods : Data pertaining to 51 cases with biopsy-confirmed HNL were collected between January 1999 and December 2005, from the Department of Pathology, College of Medicine, Pusan National University, Busan, Korea. The clinical records-including data regarding age, gender, duration of fever, and lymph node involvementwere reviewed retrospectively. The in situ hybridization (ISH) assay was performed by EBER PNA probe (Dako, Capinteria, CA, USA), and immunohistochemistry testing was performed with anti-HHV type 6 monoclonal antibodies (Chemicon, Temecula, CA, USA). Results : The HNL patients in this study were 24 males and 27 females, ranging in age from seven to 61 years (median: 25.9). ISH for EBV was positive in 8/51 (15.7%) biopsies, and immunohistochemistry for HHV6 was positive in 15/51 (29.4%) biopsies. Serologic analysis of EBV IgM was performed in 23 cases; only one patient was positive for EBV IgM and EBV ISH. Conclusion : Our study could not provide supportive evidence of a viral pathogenesis for HNL; therefore, cases of HNL may not have a dominant viral cause. However, some rare exceptional cases may have been caused by viral infection.

• Journal of the Korean Arthroscopy Society
• /
• v.16 no.1
• /
• pp.92-97
• /
• 2012

Purpose: The reparability of rotator cuff tears is very important to establish the indication of the surgical treatment of rotator cuff tears. We reviewed the factors that effect to reparability of rotator cuff tears, and considered about the surgical indication of rotator cuff tears. Materials and Methods: The reparability was considered by the surgical reparability and the postoperative healing potentials of rotator cuff. The natural history of rotator cuff tear gives us the information to make decision about the surgical treatment or the conservative treatment. Results: There are 3 general categories of factors that help predict the healing potential of a rotator cuff tear. These include surgical technique, biology, and environmental issues. Surgeon can control the surgical technique by himself. The control of biology comes from careful operative indications by age, acute trauma history, chronicity, and tear size. And the control of environmental factor can give better outcomes by cessation of smoking and nonsteroidal anti-inflammatory drug (NSAID). Conclusion: A better understanding of the natural history and the reparability of rotator cuff tear will help us to estabilish the indication of surgical treatement of rotoator cuff tears, and lead to satisfactory clinical outcomes.

• Clinical and Experimental Pediatrics
• /
• v.53 no.4
• /
• pp.585-591
• /
• 2010

Purpose : Graves' disease is the most common cause of hyperthyroidism in children and adolescents. In this study, we investigated the natural course and the prognostic factors of Graves' disease in Korean children and adolescents. Methods : One-hundred thirteen (88 girls and 25 boys) patients were included in this study. A retrospective analysis was made of all patients who were diagnosed with Graves' disease. The following parameters were recorded and analyzed: patient's sex, age at diagnosis, duration of disease, laboratory findings, symptoms and signs, and family history of autoimmune thyroid disease. Results : All patients were initially treated with antithyroid drugs, either methimazole (93.8%) or propylthiouracil (6.2%). Antithyroid drugs had been discontinued in 75 (66.4%) of 113 patients. Of these 75 patients, 23 (20.4%) relapsed after $25.5{\pm}33.7$ months. Thirteen (11.5%) of 23 patients, who experienced the first relapse, showed a second remission. However, 2 (1.8%) of 13 patients relapsed again. Euthyroid state could not be achieved by antithyroid drugs in 1 patient, and radioactive iodine therapy was performed. The older the patient at diagnosis, the greater the likelihood of remission ($P$=0.034). Conclusion : Age at diagnosis seems to be a prognostic factor in Korean children and adolescents with Graves' disease, and should be taken into account in treatment plan determination.

• Food Science and Preservation
• /
• v.9 no.1
• /
• pp.42-45
• /
• 2002

Variations of cyanogenic glucoside was investigated on varieties, picking date, each part and processed flood of Ume. First, variation of amygdalin contents was determined by HPLC during ripening. As a result in case of peels, Oshuku showed most highest content(20.2 mg%) in all varieties. In case of seeds, Native species showed most highest content(562 mg%), and seeds contented more than peels. And then, variation of prunasin contents was determined by HPLC. As a results in case of peels, native species contented most lowest prunasin in all varieties, and its contents slightly decreased with increased storage periods. Other hand, in case of seeds, native species contented most highest prunasin(177 mg%). Contents of amygdalin and prunasin of extracts was determined by HPLC during six month ripening. As a result, in case of freezing storages contents of those not changed hardly during ripening. But, in case of native storage, contents of amygdalin was decreased and prunasin was increased with increased aging periods. Profile of Ume tea was similar to extracts of it.