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반복적인 폐구균 감염을 보인8세 남아와 친형에서의 범저감마글로불린혈증
정성훈,안용주,김수연,조병수,김성도,Chung, Sung-Hoon,Ahn, Yong-Joo,Kim, Su-Yeon,Cho, Byoung-Soo,Kim, Sung-Do 대한소아감염학회 2007 Pediatric Infection and Vaccine Vol.14 No.1
범저감마글로불린혈증은 X 염색체에 의해 유전되는 질환으로 주로 1세 이전에 진단이 가능하지만, 진단이 늦어질 경우 반복된 감염 및 중증 감염으로 인한 심각한 후유증을 남길 수 있으므로 조기 진단과 치료가 매우 중요하다. 증상이 다양하게 나타날 수 있으므로 진단을 위한 각별한 주의가 요구된다. 저자들은 반복적이고 심각한 감염의 병력에도 8세가 돼서야 범저감마글로불린혈증 진단을 받은 환아와 그로 인해 함께 진단 받은 친형의 사례를 경험하였기에 보고하는 바이다. Patients with primary immunodeficiencies often have symptoms of their disease for months or years before diagnosis and treatment. This is partly because these disorders are relatively uncommon and the infections typical of immunodeficienciey, for example otitis, sinusitis, and pneumonia, are common. We report a case of agammaglobulinemia in an 8-year-old boy with recurrent and severe infection. He was first seen in our hospital for bacterial meningitis in 2006. His immune status revealed panhypogammaglobulinemia and deficiency in mature B lymphocyte. His elder brother also showed deficiency in mature B lymphocyte but mild hypogammaglobulinemia. Some X-linked agammaglobulinemia (XLA) cases may remain undiagnosed because they only show mild hypogammaglobulinemia and they lack repeated infections in childhood.
악골에 발생한 양성 치성종양의 임상 및 병리조직학적 연구
정성훈(Sung Hoon Chung),이의웅(Eui Wung Lee) 대한구강악안면외과학회 1991 대한구강악안면외과학회지 Vol.17 No.2
The author studied and analyzed on the 56 cases of benign oodontogenic tumor in the jaws which had been diagnosed with biopsy for the period from Jan 1980 to June 1990. in the Deparment of Oral and Maxillofacial surgery, Yonsei Dental hospital. This study contained the clinicostatiscal analysis of the frequency in relation to sex, age, location, chief complaints, duration, radiographic findings, histopathilogic findings and treatment methods. the result of this study were as foollows : 1. Benign odontogenic tumors of 56 cases disclosed 43 cases(76%) of ameloblastomas(28 cases, 50%), odontomas(15 cases, 26%), and other 6 types lesions(13 cases, 24%). 2. Benign odontogenic tumors occured mainly in the first and second decades(61%), male to female ratio oof the study being 1m5:1. Also the majority of cases occured in the mandible than in the maxilla(3:1). 3. In ameloblastoma 1) total 28 cases(50 %) of ameloblastoma occured in the average age of 35.8 years and had a 3:1 male predominance, mainly in the first, second and third decades(19 cases, 68%). The majority of the cases occured in the mandible, especially 14 cases(50%) in the body ramus-angle and body-angle-r4amus-coronoid process. The most common complaint was the swelling without pain(13 cases, 46). As regards duration, 16 cases(57%) was less than 1 year. 2) Radiographically, the unilocular(15 cases, 54%) annd multilocular lesions(13 cases, 46%) were similar distribution, 18 cases(64%) showed the apparent root resorption. 3) histologically, plexiform type was predominantly(10 cases), most of plexiform type was found in the young ages, radiographically it was unilocular type. 4) Radical resection was performed in 12 cases(58%). 6 cases recurredc after surgery and matastasis was absent. 4. In odontoma 1) Total 15 case(26%) of odontoma occured in the less than 30 years of age, the average age was 15.3 years, and sex distribution was not remarkable. The incidence was similar between maxilla and mandible, but more common in the premaxilla, posterior mandible. The most common chief complaint was the delayed eruption of tooth(5 cases, 33%) and followed by the routine radiographic examination(4 cases, 27%). The 8 cases(53%) were of complex type and 7 cases(47%) were of compound type. 5. 6 types of other benign odontogenic tumor, occured in the first and second decades(10 cases, 77%) and shwed similar sexual distribution. Also the incidence was similar beteen maxilla and mandible, but AOT was presented only in the maxilla and odontogenic myxima in the mandible.
리빙랩과 린 스타트업을 활용한 스타트업 지원 비즈니스 모델에 관한 연구
정성훈(Chung, Sung Hoon),김은희(Kim, Eun Hee),김세진(Kim, Se Jin) 한국창업학회 2017 한국창업학회지 Vol.12 No.1
Recently the importance of start-up increases and a government policy for supporting start-up is actively being made. However, the success rate of start-up is not very high. The purpose of this study is to suggest a start-up support business model to increase the success rate of start-up. In this research, failure factors of start-up business was analyzed based on literature reviews, and several cases of start-up support business model were studied. Finally we developed a web-based business model for start-up support was developed applying ‘Living lab’ platform and ‘Lean start-up’ process, and analyzed differences between previous models and the new model.
임상 ; 단일 신생아 중환자실에서 경험한 최근 10년간 선천성 기형의 변화
정성훈 ( Sung Hoon Chung ),김희영 ( Hee Young Kim ),김지희 ( Ji Hee Kim ),최용성 ( Yong Sung Choi ),이병섭 ( Byong Sop Lee ),김기수 ( Ki Soo Kim ),김애란 ( Ai Rhan Kim ) 대한주산의학회 2013 Perinatology Vol.24 No.1
목적: 국내 선천성 이상에 대한 신뢰성 있는 통계자료가 부족한 상황에서 단일기관에서의 대략적인 발생 분포를 알아보고 이들의 사망률에 대하여 조사하고자 하였다. 방법: 2001년 1월부터 2010년 12월까지 서울아산병원 신생아 중환자실과 신생아실에 입원한 29,026명을 대상으로 하였다. 2009년 보건복지가족부에서 발간한 ``선천성 이상아 조사 및 분석 연구``을 참고로 7개 계통으로 나누어 총 78개 질환군으로 분류하였다. 결과: 2001년에서 2010년까지 대상 선천성 이상아 수는 3,122명으로 2001년 217명에서 2010년 515명으로 계속 증가하는 추세이다. 대상 환아는 평균 재태연령 38+2±2.3주(미숙아 12.5%), 평균 출생체중 3,030±541 g, 남자 비율이 61.0%였다. 35세 이상의 고령산모는 16.1%였으며 2001년 8.3%에서 2010년 23.0%로 증가하였다. 신체 부위 및 기관별로 순환기계통 선천성 이상의 유병률이 가장 높았으며, 1개 이상의 동반 기형이 있는 비율은 2001년 24.0%에서 2010년 23.7%로 10년간 비슷한 양상이었다. 각 계통별 가장 많은 질환은 각각 심방중격결손증, 선천수신증, 직장항문폐쇄/협착, 선천성 낭종 이형성증, 입천장/입술갈림증, CATCH22 증후군, 다지증, 선천수두증이었다. 2001년도에 비해 2010년도에는 선천성 기형아의 유병률이 6.4%에서 16.4%로 증가하였고, 이 중 순환기계통이 31.5%에서 52.9%, 비뇨생식계통이 18.5%에서 14.8%, 위장관계통이 24.8%에서 12.1%로 변화하였다. 환아들의 2세 때 사망률은 2001년 11.1%에서 2010년 8.0%로 감소하였고, 각 계통별 질환 중 사망률이 가장 높은 질환은 좌심실형성부전증후군, 콩팥무발생증, 선천가로막탈장, 폐의 저하증 및 형성이상, 소안구증, 18번 삼염색체증, 다리의 감소성 결손, 뇌없음증이었으며, 사망률이 높을수록 다른 선천성 기형이 동반되는 경우가 많았다. 결론: 여러 전문 분야적 접근이 용이해짐에 따라 사망률이 점차 감소하고 있으나 최근 선천성 이상아의 출생 빈도와 복합기형의 비율이 증가추세이다. 이들이 속하고 있는 사회인구계층을 알고 예방 가능한 원인을 추적해야 하겠다. Purpose: Congenital anomalies contribute a significant proportion of infant morbidity and mortality, as well as fetal mortality. However, there is the lack of reliable data statistically about congenital anomalies in Korea. The purpose of this study is to investigate the epidemiological information, changing patterns, and mortality of congenital anomalies in a single Neonatal Intensive Care Unit. Methods: We did research targeting 29,026 neonates in Neonatal Intensive Care Unit and nursery of Asan Medical Center Children`s Hospital from January 1st, 2001 to December 31th, 2010. We classified total 76 diseases in 8 organic systems with the reference ``Congenital Anomaly Survey and Statistics`` published by the Ministry of Health and Welfare in 2009. Results: The number of neonates who have congenital anomalies for ten years are 3,464. It is increasing in comparison with 233 in 2001, 559 in 2010. The average gestational age was 38+2±2.3 weeks (prematurity 19.4 %), the average birth weight was 2,940±689 g and 64.1% were men. Mothers older than 35 were 16.7%, it was increased from 8.3% in 2001 to 23.0% in 2010. According to organic systems, anomalies of circulatory systems were most common. Neonates who have multiple anomalies are increased from 38.8% in 2001 to 46.3% in 2010. The most common diseases according to each organic systems are patent ductus arteriosus, congenital hydronephrosis, anorectal atresia/stenosis, congenital cystic adenomatoid malformation, cleft lip and/or palate, CATCH22 syndrome, polydactyly and congenital hydrocephalus. The incidence of congenital anomaly increased from 6.8% in 2001 to 17.8% in 2010. For example, the incidence of circulatory anomalies changed from 36.0% to 56.0%, the incidence of urogenital anomalies changed from 18.2% to 14.2% and the incidence of gastrointestinal anomalies changed from 24.4% to 9.0%. The mortality rate when the patient was 2 years old decreased from 10.3% in 2001 to 7.0% in 2010. The disease with the highest mortality rate according to each organic systems were hypoplastic left heart syndrome, renal agenesis, congenital diaphragmatic hernia, pulmonary hypoplasia or dysplasia, microphthalmos, 18 trisomy, reduction defects of lower limbs, anancephaly. The higher the mortallity rate is, the more often congenital anomalies are associated with other one. Conclusion: With easy accessibility of the area to health care, the mortality rate of neonates decreases. But the incidence of congenital anomalies and the rate of multiple anomalies have increased recently. We have to understand about population who has risk factors and to trace the cause for prevention.