• Maxillofacial Plastic and Reconstructive Surgery
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• 제27권4호
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• pp.381-387
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• 2005

Malignant hyperthermia is a catastrophic, hypermetabolic syndrome that arises in susceptible individuals when they are exposed to certain inhalational anesthetics or muscle relaxants. It is characterized by hyperthermia, tachycardia, acidosis, and muscle rigidity. It has been noted that the majority of cases of malignant hyperthermia are fatal unless early diagnosis and treatment are performed. We experienced a 24 year old male Malignant hyperthermia presented for orthognathic surgery under $O_2-N_2O$-sevoflurane anesthesia without succinylcholine. Two half hours after induction, tachycardia developed and was followed by unstable blood pressure and hyperpyrexia. Anesthesia was terminated and vigorous emergency treatment was attempted. The patient was treated by the intravenous administration of dantrolene sodium. The diagnosis of an acute malignant hyperthermia reaction by clinical criteria can be difficult because of the nonspecific nature and variable incidence of many of the clinical signs and laboratory findings. So the malignant hyperthermia clinical grading scale is recommended for use as an aid to the objective definition of this disease. This clinical grading system provides a new and comprehensive clinical case definition for the malignant hyperthermia syndrome. We recently encountered a case of delayed malignant hyperthermia during sevoflurane anesthesia that was successfully treated by the intravenous administration of dantrolene sodium. In conclusion, exposure to sevoflurane should be avoided in patients thought to be susceprible to malignant hyperthermia.

• Tuberculosis and Respiratory Diseases
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• 제52권3호
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• pp.283-287
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• 2002

Scrub typhus is an acute, febrile disease of humans that is caused by Orientia Tsutsugamushi. It is transmitted through the bite of chiggers. The spectrum of the clinical severity for scrub typhus ranges from mild to severe with fatal complication such as meningoencephalitis, pneumonitis, myocarditis. Severe pulmonary involvement e.g. acute respiratory distress syndrome(ARDS) and meningoencephalitis have rarely been observed since the introduction of specific antibiotic therapy. We experienced a case of scrub typhus manifested with ARDS and meningoencephalitis. The patient was treated with doxycycline, anticonvulsant and mechanical ventilator therapy.

• Biomolecules & Therapeutics
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• 제28권4호
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• pp.311-319
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• 2020

Middle East Respiratory Syndrome Coronavirus (MERS-CoV) is a newly emerging viral disease with fatal outcomes. However, no MERS-CoV-specific treatment is commercially available. Given the absence of previous structure-based drug discovery studies targeting MERS-CoV fusion proteins, this set of compounds is considered the first generation of MERS-CoV small molecule fusion inhibitors. After a virtual screening campaign of 1.56 million compounds followed by cell-cell fusion assay and MERS-CoV plaques inhibition assay, three new compounds were identified. Compound numbers 22, 73, and 74 showed IC₅₀ values of 12.6, 21.8, and 11.12 µM, respectively, and were most effective at the onset of spike-receptor interactions. The compounds exhibited safe profiles against Human embryonic kidney cells 293 at a concentration of 20 µM with no observed toxicity in Vero cells at 10 µM. The experimental results are accompanied with predicted favorable pharmacokinetic descriptors and drug-likeness parameters. In conclusion, this study provides the first generation of MERS-CoV fusion inhibitors with potencies in the low micromolar range.

• Journal of Microbiology and Biotechnology
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• 제29권8호
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• pp.1316-1323
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• 2019

Middle East respiratory syndrome coronavirus (MERS-CoV) is a newly emerging coronavirus which is zoonotic from bats and camels. Its infection in humans can be fatal especially in patients with preexisting conditions due to smoking and chronic obstructive pulmonary disease (COPD). Among the 25 proteins encoded by MERS-CoV, 5 accessory proteins seem to be involved in viral evasion of the host immune responses. Here we report that ORF4a, ORF4b, and ORF8b proteins, alone or in combination, effectively antagonize nuclear factor kappa B ($NF-{\kappa}B$) activation. Interestingly, the inhibition of $NF-{\kappa}B$ by MERS-CoV accessory proteins was mostly at the level of pattern recognition receptors: melanoma differentiation-associated gene 5 (MDA5). ORF4a and ORF4b additively inhibit MDA5-mediated activation of $NF-{\kappa}B$ while that of retinoic acid-inducible gene 1 (RIG-I) is largely not perturbed. Of note, ORF8b was found to be a novel antagonist of MDA5-mediated $NF-{\kappa}B$ activation. In addition, ORF8b also strongly inhibits Tank-binding kinase 1 (TBK1)-mediated induction of $NF-{\kappa}B$ signaling. Taken together, MERS-CoV accessory proteins are involved in viral escape of $NF-{\kappa}B$-mediated antiviral immune responses.

• 항공우주의학회지
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• 제30권2호
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• pp.75-79
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• 2020

This study focuses on the comparative and quantitative analysis of the epidemiologic trends and aspects of severe fever with thrombocytopenia syndrome (SFTS) outbreaks between Korea and Japan from 2013 to 2017. The following factors were analyzed; cumulative incidence rate (CIR), cases-fatality rate (CFR), and the epidemic aspects, including cases related to gender, male-to-female morbidity ratio (MFMR), age, seasonal, and geographical distributions. We observed 607 SFTS cases with CIR in Korea during the period 2013 to 2017 were as 0.24 per 100,000 populations and with a 127 fatal-cases (F.C.s), corresponding to a CFR of 20.9%, respectively. During the same period in Japan, 319 SFTS cases with a CIR of 0.05 and with 60 F.C.s to a CFR of 18.8% observed. When compared, the CIR of SFTS in Korea was significantly higher than in Japan (P<0.01), but there were no significant differences levels of the CFR and MFMR between Korea and Japan. Also, a higher incidence of SFTS was observed in people aged over 50-years or elders in Korea and those of 60-years or elders in Japan (P<0.01). The seasonal distribution of SFTS outbreak cases showed that the incidence in summer through autumn in Korea (92.4% of total cases) was higher than in Japan (65.2%), while the outbreaks of SFTS in spring was much higher in Japan (31.0%) than in Korea (7.4%), (P<0.01). The regional distribution revealed no significant difference between the eastern area (44.8%) and the western area (46.8%) of the Korean peninsula except Jeju-island (8.4%). However, in Japan, the incidence only occurred in Chubu-Kinki-Chugoku (30.3%), Shikoku (25.7%), Kyushu (42.6%) and Okinawa (0.3%), which are the western and southern areas of Japan. These differences in SFTS occurrence may reflect the influences of vector/hosts, climate, and geographical and cultural characteristics between the two countries.

• Clinical and Experimental Pediatrics
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• 제46권10호
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• pp.996-1002
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• 2003

목 적 : Rett 증후군 환자에서는 일반인에서 보다 돌연사의 확률이 높은 것으로 알려져 있으며, 그 원인으로 자율신경계 기능 부전에 의한 치명적인 부정맥 같은 심장의 전기 생리적 불안정성이 가장 의심되고 있다. 본 연구는 Rett 증후군 환자에서 심박수 변이도와 교정 QT 간격을 측정하여 실제로 자율신경 활성도에 문제가 있는지의 여부를 확인하기 위하여 실시하였다. 방 법 : 분자유전학적 염색체검사나 임상적 진단기준에 의하여 진단된 Rett 증후군 환자 12례를 대상군으로, 나이가 비슷한 정상아 30례를 대조군으로 하여 이들에서의 자율신경 활성도인 심박수 변이도와 교정 QT 간격을 측정하여 비교하였다. 심박수 변이도는 24시간 보행 심전도를 기록한 후 디지털화 된 심전도를 자동적으로 분석하여 power spectrum을 만들고 그 중 주파수영역(고주파 성분, 저주파 성분, 극소저주파 성분, LF/HF 비율)과 시간 영역(평균 RR 간격, SDNN, rMSSD, pNN50), 교정 QT 간격을 측정하여 비교하였다. 비교는 세 그룹으로 나누어서 전체 Rett 증후군과 정상 대조군 사이, 전형적인 Rett 증후군과 정상 대조군 사이, 비전형적 Rett 증후군과 정상 대조군 사이에서 실시하였다. 결 과 : 1) Rett 증후군 환자에서는 대조군에서 보다 시간영역의 rMSSD이 통계적으로 의미있게 낮았고(P<0.005), LF/HF 비율이 의미있게 높았으며(P<0.005), 교정 QT 간격도 의미있게 연장되어(P<0.005) 있었다. 2) 전형적인 Rett 증후군 환자에서도 대조군에서 보다 rMSSD이 통계적으로 의미있게 낮았고(P<0.005), LF/HF 비율도 의미있게 높았으며(P<0.05), 교정 QT 간격도 의미있게 연장되어 있었다(P<0.001). 3) 비전형적 Rett 증후군에서도 마찬가지로 rMSSD이 통계적으로 의미있게 낮았고(P<0.05), LF/HF 비율도 의미있게 높았으며(P<0.001), 교정 QT간격도 의미있게 연장되어 있었다(P<0.05). 4) 이상의 결과에서 Rett 증후군에서는 전형적인 환자나 비전형적인 환자 모두 미주신경긴장의 변화와 호흡에 의해 민감한 영향을 받는 rMSSD의 저하가 있었고, 교감-부교감신경 균형성을 나타내는 LF/HF 비율의 증가가 있었으며, 자율신경계에 영향을 받는 교정 QT 간격이 연장되었던 것으로 보아, 교감-부교감신경의 균형성에 이상이 있는 것으로 사료된다. 결 론 : Rett 증후군 환자에서 자율신경 활성도의 지표인 심박수 변이도의 의미있는 저하와 교정 QT 간격의 의미있는 연장이 나타나는 것으로 미루어 보아, 이 질환에서 자주 동반되는 돌연사가 일어나는 원인 중의 하나로 이러한 교감-부교감신경의 불균형을 의심할 수 있겠다.

• Tuberculosis and Respiratory Diseases
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• 제52권4호
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• pp.367-375
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• 2002

연구배경: 발진열은 벼룩에 의해 매개되며 전세계적으로 발생되는 리케치아질환으로 대개 경한 임상경과를 보이나 간혹 중증으로 발현된다. 저자들은 발진열로 유발된 급성호흡곤란증후군 6예를 경험하게 되어 그 임상상을 분석하고자 하였다. 방 법: 1991년 이후 서울중앙병원에 발열을 주소로 내원하여 발진열로 진단된 환자들을 찾아 호흡곤란증후군을 동반한 군과 그렇지 않은 군으로 나누어 각군에 대한 연령, 성별, 혈액학적 검사 및 미생물학적 검사에 대해 조사하여 비교하였다. 발진열의 진단은 특이적 면역형 광항체검사법을 시행하여 초회 항체역가가 1:512 이상이거나 추적 항체가의 4배이상 증가가 있으면서 발진열에 합당한 임상양상이 있는 경우로 한정하였고 급성호흡곤란증후군은 American-European Consensus Conference에 제시된 기준에 따라 정의하였다. 결 과: 호흡곤란증후군이 동반된 6례를 포함 모두 15예가 있었다. 호흡곤란증후군이 동반된 6명의 환자 중 2명이 여자였고 3명이 도시에 거주하였다. 발진은 모두에서 관찰되지 않았다. 한명이 치료도중 사망하였다. 급성호흡곤란증후군과 연관된 인자로 진단 및 치료시까지의 지연시간과 저알부민혈증, 혈중 bilirubin의 증가, 높은 APACHE III 점수와 MOD 점수가 통계적 유의성을 보였다. 결 론: 발진열은 풍토병의 성격을 띤 지역에서 원인미상의 급성호흡곤란증후군의 한 원인으로 고려될 수 있고 최근 도시거주자에게도 발병되고 있다. 발진열에 의한 급성호흡곤란증후군의 예후는 다른 원인에 의한 경우보다 좋지만 조속한 진단 및 치료가 중요할 것으로 생각된다.

• Journal of Chest Surgery
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• 제20권3호
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• pp.536-543
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• 1987

The Fontan procedure was physiological correction which was initially applied to tricuspid atresia. We had used the modified Fontan operation in 8 cases at National Medical Center, Seoul, from Aug. 1984 to Oct. 1986. Age range was 20 months to 15 years [mean: 9 years] and male: female ratio was 5:3. 5 patients had tricuspid atresia [lb: 2 cases, Ic: 1 case, lib: 1 case, & llc: 1 case], 2 Patients had univentricular heart of left ventricular type, and one patient had transposition of great arteries with complete endocardial cushion defect. The operative principle was direct anastomosis between right atrium and pulmonary artery, whether main pulmonary artery or right pulmonary artery without any conduits. Postoperatively all patients needed high central venous pressure for adequate hemodynamic status in both survival [20-24 CmH2O] and mortality groups [20-24 CmH2O]. The fatal complications were as follows: empyema with bronchopleural fistula [1 case], bleeding tendency & brain damage [1 case], low cardiac output syndrome & acute renal failure [2 cases], and right to left shunt of unknown origin [1 case]. There were 5 hospital deaths; 3 of 5 tricuspid atresia patients, 1 of 2 univentricular heart patients, and 1 of 1 transposition of great arteries with complete endocar4ial cushion defect patient. The overall mortality was 62.5%. 3 survivors were nearly compatible with Choussat & Fontan criteria. Thus appropriate patient selection and experienced surgical technique were required for good results.

• Clinical and Experimental Pediatrics
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• 제57권12호
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• pp.505-513
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• 2014

While the survival of extremely premature infants with respiratory distress syndrome has increased due to advanced respiratory care in recent years, necrotizing enterocolitis (NEC) remains the leading cause of neonatal mortality and morbidity. NEC is more prevalent in lower gestational age and lower birth weight groups. It is characterized by various degrees of mucosal or transmural necrosis of the intestine. Its exact pathogenesis remains unclear, but prematurity, enteral feeding, bacterial products, and intestinal ischemia have all been shown to cause activation of the inflammatory cascade, which is known as the final common pathway of intestinal injury. Awareness of the risk factors for NEC; practices to reduce the risk, including early trophic feeding with breast milk and following the established feeding guidelines; and administration of probiotics have been shown to reduce the incidence of NEC. Despite advancements in the knowledge and understanding of the pathophysiology of NEC, there is currently no universal prevention measure for this serious and often fatal disease. Therefore, new potential techniques to detect early biomarkers or factors specific to intestinal inflammation, as well as further strategies to prevent the activation of the inflammatory cascade, which is important for disease progression, should be investigated.

• Journal of Yeungnam Medical Science
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• 제33권1호
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• pp.68-71
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• 2016

Tumoral calcinosis (TC) is a condition resulting from extensive calcium phosphate precipitation, primarily in the periarticular tissues around major joints. Calciphylaxis is a fatal ischemic vasculopathy mainly affecting dermal blood vessels and subcutaneous fat. This syndrome is rare and predominantly occurs in patients with end-stage renal disease. Here, we report on a rare case involving a patient with TC complicated with calciphylaxis. Our patient was a 31-year-old man undergoing hemodialysis who presented with masses on both shoulders and necrotic cutaneous ulcers, which were associated with secondary hyperparathyroidism, on his lower legs. He underwent subtotal parathyroidectomy, and sodium thiosulfate (STS) was administered for 27 weeks. Twenty months after beginning the STS treatment course, he experienced dramatic relief of his TC and calciphylaxis.