신경섬유종증-제 2형의 진단과 치료 : 16례의 임상경험 Experience of 16 Cases

김정은,백선하,김종수,이상형,오창완,김동규,정희원,김현집,조병규,한대희,최길수 대한신경외과학회 1996 Journal of Korean neurosurgical society Vol.25 No.12

Neurofibromatosis type 2(NF-2) is a dominantly inherited disorder characterized by the occurrence of bilateral acoustic neurinomas and the frequent association of other central nervous system tumors. We present a retrospective review of 16 patients with NF-2 who were treated at our hospital from 1984 to 1995 in 13 cases, the diagnoses of Nf-2 were based on the criteria developed at the Consensus Development Conference of National Institute of Health in the United States, and in another 3 cases. the criterias of NF-2 were not fully satisfied, but the diagnoses of NF-2 were highly suspected. The average age of the patients was 27.6 years, ranging from 13 years to 56 years. The most common symptom was hearing difficulty : intervals between symptom onset and deafness ranged from 8 months to 6 years(mean : 2.9 years) One family of NF-2 was documented consisting of a sister a brother and their mother. Nine patients underwent operations on unilateral acoustic neurinomas : these were subtotally removed in eight patients and totally in one patient Among these patients five were deaf on the ipsilateral side at surgery. Among the other four patients with useful hearing before surgery, hearing was preserved to preoperative status in two patients. Four patients with diagnoses of meningioma, received operations to relieve mass effect with subtotal removal in two patients and total removal in the other two. Early diagnosis and treatment are the most important in the management planning of patients with NF-2 for reasons of early manifestation and rapid progression of the disease.

조혈모세포이식 환자에서 침습성 진균 감염에 대한 Micafungin의 예방 효과 및 안전성

김시현,이동건,최수미,권재철,박선희,최정현,유진홍,이성은,조병식,김유진,이석,김희제,민창기,조석구,김동욱,이종욱,민우성,박종원 대한감염학회 2010 감염과 화학요법 Vol.42 No.3

Background: Micafungin, a potent inhibitor of 1,3-β-D-glucan synthase, is a novel antifungal agent of the echinocandin class. In vitro study showed that micafungin was effective against Aspergillus species as well as Candida species, but clinical data on the prophylactic efficacy against invasive fungal infections (IFIs) other than candidiasis are still lacking. Materials and Methods: We identified 60 consecutive adult hematopoietic stem cell transplantation (HSCT) recipients who received at least 3 doses of micafungin during neutropenic period. Micafungin was started as an alternative in patients who were intolerant or had adverse events (AEs) to primary prophylactic antifungal agents. We retrospectively reviewed the medical records and analyzed the efficacy and safety of micafungin for prophylaxis against IFIs. Results: The patients either had autologous (n=9) or allogeneic (n=51: 1 syngeneic, 24 sibling, 26 unrelated donor) HSCT. Itraconazole oral solution (n=58) was the most frequently used first line antifungal agent for prophylaxis and was administered for median 11 days. The most frequent cause of switch to micafungin was vomiting (n=42). The duration of neutropenia and micafungin administration was median 13 and 12 days, respectively. A successful outcome was achieved in 45 (75%) patients. Empirical antifungal therapy was initiated in 13 (22%) patients. There were 2 cases (3.3%) of breakthrough fungal infections which comprised a probable invasive pulmonary aspergillosis and a possible invasive fungal sinusitis. There was no case of invasive candidiasis. A total of 53 (88%) patients experienced at least one AE regardless of causality during micafungin administration. The most frequent AEs were hypokalemia, vomiting, diarrhea, and elevated serum aspartate aminotransferase or alanine aminotransferase. Among the aforementioned AEs, only 1 case of diarrhea could be classified as a probable relation with micafungin when causality was assessed. There was no AEs that caused discontinuation of micafungin. Conclusions: Micafungin seems to be a safe and effective agent for prophylaxis of IFIs including aspergillosis as well as candidiasis in HSCT recipients. However, further large, prospective, and randomized comparative studies are warranted for aspergillosis.

대두단백질 가수분해물의 특성

김동수,양승택,문윤희,류병호,김희숙,염동민,김진목 慶星大學校 1998 論文集 Vol.19 No.1

The present study was conducted to elucidate some characteristics of soy protein hydrolysates. The soy protein was obtained from defatted soy power by the treatment of n-hexan. The hydrolysates of soy protein were prepared from hydrolysis by bromelian and pepsin with the two kinds of samples of precooking for 30 min in steam oven and noncooking, and fractionated through Bio-gel P-4 column chromatography. Fractions of hydrolysates separated on the Bio-gel column chromatography were carried out to SDS-PAG electrophoresis, resulting to show some different bends from 66kD to 14.2kD of molecular weight. In addition, the fractions showed different activities of superoxide dismutase. From these results, it was found that the hydrolysates could be available to use in a lot of food industrial fields as new functional food materials because the hydrolysates had different characteristics in electrophoresis and could increase the activity of superoxide dismutase.

알쯔하이머형 치매와 혈관성 치매환자에서의 ALDH2와 APOE 유전자의 다형성에 관한 연구

김동홍,김영돈,이창화,엄기춘,김은식,윤경식,김동희 대한생물치료정신의학회 2002 생물치료정신의학 Vol.8 No.2

연구목적: 본 연구는 국내 치매환자들을 대상으로 치매발병의 위험요소가 될 수있는 ALDH2와 APOE 유전자형이 어떤 양상으로 나타나는지 알아보기 위하여 시행되었다. 방 법: 충남 부여군에 위치한 부여노인병원에 입원중인 65세 이상의 알쯔하이머형 치매와 혈관성 또는 혼재성 치매환자 59명(남:19, 녀:40)을 대상으로 ALDH2와 APOE 유전자형을 제한효소법에 의하여 조사하였고 치매유형에 따른 유전자형과 대립유전자의 빈도를 비교하였다. 결 과: 1) 알쯔하이머형 치매환자군에서 혈관성 또는 혼재성 치매환자군보다 ALDH2*(-/2)유전자형의 빈도가 높았으나 통계학적으로 의미있는 차이는 없었다. 2) 알쯔하이머형 치매화자군에서 혈관성 또는 혼재성 치매환자군보다 APOE*(-/ε4) 유전자형의 빈도가 높았으나 통계학적으로 의미있는 차이는 없었다. 3) APOE*ε4와 ALDH2*2를 알쯔하이머병의 균등한 위험대립유전자로 가정하고 각 유전자형의 대립유전자의 수를 점수화하여 각 치매환자군의 위험유전자 전체점수를 비교한 결과 알쯔하이머형 치매환자군에서 점수가 더 높게 나왔으나 통계학적으로 의미있는 차이는 없었다. 결 론: 알쯔하이머형 치매환자군에서 혈관성 또는 혼재성치매치매환자군보다 ALDH2*2와 APOE*ε4 대립유전자의 빈도가 높게 나왔으나 통계학적으로 의미있는 차이는 없었다. A study on gene polymorphism of ALDH2 and APOE in patients with alzheimer's disease and vascular or mixed dementia. Objective : This study was designed to investigate gene polymorphism of ALDH2 and APOE, reported the risk factor of Alzheimer's disease, in korean dementia patients. Method : 59 Patients(male:19, female:40) with dementia, aged 65 and older in Buyeo geriatric hospital located in Buyeo-gun, chungnam, were finally included in this study. gene polymorphism of ALDH2 and APOE was typed with polymerase chain reaction in patients with Alzheimer's desease and vascular or mixed dementia. Results : 1) There was no difference in allelic frequency of ALDH2 gene polymorphism between patients with Alzheimer's disease and vascular or mixed dementia. 2) There was no difference in allelic frequency of APOE gene polymorphism between patients with Alzheimer's disease and vascular or mixed dementia. 3) Supposing APOE*ε4 and ALDH2*2 were equal risk allele of Alzheimer's disease, the sum of score by counting each risk allele was higher in patients with Alzheimer's disease than vascular mixed dementia, however there was no significant difference. Conclusion : The genotype frequency of ALDH2*(-/2) and APOE*(-/ε4) was higher in patients with Alsheimer's disease than vascular or mixed dementia, however there was no significant difference in allelic frequency of gene polymorphism of ALDH2 and APOE between patients with Alzheimer's disease and vascular or mixed dementia.

치매 환자에서 행동·정신 증상에 대한 Risperidone의 치료 효과

김영돈,김동홍,이창화,김동희 대한노인병학회 2001 대한노인병학회지 Vol.5 No.3

정상인에서 베타3-아드레날린 수용체 유전자의 변이가 복부 비만도 및 지질대사에 미치는 영향

김영선,윤석기,김철희,서교일,김학선,김극배,변동원,유명희 순천향의학연구소 1998 Journal of Soonchunhyang Medical Science Vol.4 No.1

The β₃-adrenergic receptor is expressed in visceral adipose tissue in humans and is thought to contribute to the regulation of the resting metabolic rate and lipolysis. We studied the influence of a mutation in the β₃ -adrenergic receptor gene(Trp64Arg) on body fat distribution, central obesity, lipid metabolism in 65 healthy young male adults. One out of 65 subjects were homozygous (Arg/Arg) for the trp64Arg mutation, 17 subjects were heterozygous (Trp/Arg), and 47 lacked the mutation(Trp/Trp). The body weight, height, body mass index, waist-to-hip ratio were similar between the subjects with- and without the mutation. Total body fat, abdominal fat amount, body fat distribution, serum total- and HDL-cholesterol, triglyceride, glucose, and insulin concentrations were also not different according to the presence or absence of the mutation. These results suggested that Trp64Arg mutation in β₃-adrenergic receptor gene is not a major contributing factor for central obesity or change in lipid metabolism in Korean young adults.

주물공장 근로자에서 발생한 국한성 석면폐증 1예

김태우,고동희,김민기,유승원,류향우,한정희,정순희 大韓産業醫學會 2009 대한직업환경의학회지 Vol.21 No.2

배경: 주물공장 근로자에서 발생한 석면폐증의 작업 관련성을 평가하고 그 증례를 보고하고자 한다. 증례: 30년간 주물공장에서 근무한 환자로 인후통, 기침, 노란 가래가 있어 검사한 결과 결핵이 의심되어 치료 하였으며, 치료 중 석면폐증으로 진단되었다. 작업 관련성 평가를 위해 실시한 폐 조직의 조직학적 검사에서 1 cm^(2)당 3개의 석면소체가 발견되었고, 투과전자현미경으로 분석한 석면 함유량은 23.26x10^(6) fibers/g이었다. 결론: 작업환경조사, 폐 조직의 석면 분석 등을 통해 작업 관련성이 높다고 판단하였다. Background: Foundry workers are known to be exposed to silica and have an increased risk of Silicosis. There are also reports that state these foundry workers were associated with asbestos-related change in the X-ray results of the lungs. This report discusses a case of localized asbestosis developed in a foundry worker who worked in 12 Korean foundries. Case report: A 49-year-o1d man who worked at molding and melting for 30 years, Presented with sore throat, cough and yellow sputum for 1 month. From physical and clinical examination, Pulmonary tuberculosis was highly suspicious; therefore, he was medicated for 6 months. After tuberculosis medication, the radiological finding was reactivation of tuberculosis, and an asbestos body was detected at a trans-bronchial lung biopsy. During an operation, operators found multiple palpable masses and bullae 10calized in the right upper lobe (RUL) and performed right upper lobectomy and right middle lobe wedge resection. Using hematoxylin-eosin stain and iron stain, we could detect asbestos bodies by light microscopy. We analyzed the asbestos fiber burden in dry lung tissue by transmission electron microscopy with energy dispersive X-ray spectrometer (TEM-EDX). The fiber type was chrysotile and the fiber burden was 23.26 x 10^(6) fiber/g dry lung. Discussion: This case study of a foundry worker with localized asbestosis is uncommon in Korea. We found an occupational relationship between the foundry worker and localized asbestosis through various investigative techniques such as measurements taken at his working environment, clinical, radiological and pathological examination of the foundry worker and mineralogical examination of the asbestos fiber.

각종 난치성 혈액 질환에서의 비혈연간 골수이식

김동욱,한훈,김정아,김희제,민창기,엄현석,최정현,이종욱,한치화,홍영선,최일봉,신완식,민우성,김학기,김춘추,김원일,김동집 대한조혈모세포이식학회 1997 대한조혈모세포이식학회지 Vol.2 No.1

목적: 비혈연간 골수이식은 혈연내에 적절한 골수공여자가 없는 만성골수성백혈병, 고위험군의 급성별혁병, 면역억제치료에 실패한 재생불량성빈혈 및 각종 난치성 조혈모세포질환의 완치를 위한 표준적인 치료방법으로 정착되고 있다. 혈연간 표준적인 동종 이식에 비하여 비혈연간 이식시에는 생착부전, 이식편대숙주반응과 감염이 더 빈번하게 발생하며, 국내에서는 아직까지 체계적인 임상연구결과가 보고된 바 없었다. 이에 본 센터에서는 1995년 10월 이후로 약 20개월간 26예의 비혈연간 골수이식을 시행하였으며 3개월 이상의 추적관찰이 가능하여 이식초기 합병증의 관찰 및 분석이 가능하였던 20예의 환자를 대상으로 이식성적 및 문제점을 보고함으로써 새롭게 확대되고 있는 이 분야의 임상연구 및 진료의 활성화를 꾀하고자 한다. 방법: 각종 혈액 종양질환으로 비혈연간 이식을 시행한 총 26예의 환자중 3개월이상의 추적관찰이 가능하였던 20예를 대상으로 후향적으로 임상경과를 분석한 후 생존 분석을 시행하였고, 환자의 연령, 성별, 질병의 상태, 조식 적합 항원의 일치정도, 이식편대 숙주 반응의 유무와 생존기간과의 상관관계를 살펴보았다. 또한 표준위험군과 고위험군으로 나누어 생존율을 비교하였고 이식과 관계된 생착 부전, 이식편대숙주반응, 감염의 발생과 양상 그리고 그 합병증을 관찰하였다. 결과: 1. 환자와 공여자간에 HLA 불일치가 20예 중 4예에서 있었으며, 생착여부의 확인이 가능했던 17예 중 16예에서 생착이 확인되어 94.1%의 생착율을 보였다. 2. 급성이식편대숙주반응은 62.5%(10/16예)에서 발생하였으며 111도 이상의 급성의 이식편대숙주 반응은 25%(4/16예)에서 발생하였다. 만성이식편대숙주반응은 40%(2/5예)의 환자에서 발생하였으며 이들 모두 국소형으로 중증의 진행형 만성이식편대숙주반응이 관찰된 환자는 없었다. 3. 호흡기 합병증은 10예(50%)에서 발생하였으며 감염성 폐렴을 포함한 호흡기 합병증이 가장 흔한 일차적인 사망 원인이었다. 호흡기 합병증이 발생했던 10예중 6예가 감염에 의한 폐렴이 의심되었고 나머지 4예는 특발성 간질성 폐렴이었다. 4. 8.5개월의 중앙추적기간 중 35%의 생존율을 관찰할 수 있었고, 생존기간은0.5개월에서 15개월 (중앙치:4개월)이었다. 한편 고위험군은 25%(3/12예), 표준위험군은 50%(4/8)의 생존율을 관찰할 수 있었다. 5. 가장 흔한 사망 원인은 감염성 폐렴을 포함한 호흡기 합병증(6예)이었고, 이외의 사망 원인으로는 급성 이식편대숙주반응과 다장기부전이 각각 2예, 생착 부전, 간정맥 폐쇄, 그리고 재발이 각각 1예였다. Unrelated bone marrow transplantation(UBMT) has been increasingly recognized as the standard treatment for cure of chronic myelogenous leukemia, high risk acute leukemia, aplastic failed on immunotherapy, and the variety of refractory hematologic diseases in patients lacking a related donor. However, as compared to HLA identical sibing transplantation, UBMT carries higher incidence of graft failure, graft versus host disease(GVHD), and infection. In our center, 26 patients underwent UMBT between October 1995 and June 1997. The minimum follow-up of 3 months was possible in 20 patients, for whom early complications and clinical outcomes were assessed. The median age of the 20 patients was 24 years. 8 patients had standard risk disease and 12 patients had high risk disease. All patients received various preparative regimens including total body irradiation according to disease and disease status. 19 patients received CsA + short course MTX for GVHD prophylaxis. One patient received marrow that was depleted of T cells ex vivo using avidinbiotin column. The class I loci were typed by serological methods and HLA-A, HLA-B and HLA-DRB1. 3 additional pairs were one minor mismatched at the HLA-B locus. Another one patients was one major mismatched at the DRBI alleles. 17 patients were evaluable for engraftment. Successful enfraftment was confirmed in 16 patients(94.1%). Only one patient who was performed one major DRBI mismatched transplants experienced graft rejection. 16 patients were evaluable for acute GVHD. The overall incidence of acute GVHD developed in 4 patients(25%). Five patients were evaluable for the development of Ⅳ acute GVHD developed in 4 patients (25%). Five patients were evaluanle for the development of chronic GVHD. 2 patients(40%) developed limited chronic GVHD. Respiratory complications including pulmonary infection developed in 10 patients(50%) and these complications were the most common primary cause of death. Of these 10patients, 6 had pneumonia due to fungus(4 patients), pacterial (1 patient), and CMV infection (1 patient) and 4(20%) had idiopathic interstitial pneumonitis and/or adult respiratory distress syndrome. The duration of median follow- up was 8.5 months and 7 of 20 patients(35%) are alive at the time of this analysis with survival duration of 0.5 to 15 months(median survival duration: 4 months). The overall survival was 25% (3/12 patients) in high risk group and 50%(4/8 patients) in standard group. From these results, we can predict that the incidence and severity of GVHD in Korea are lesser than multiracial countries and the long-term survival of patients with standard risk disease can approach that of HLA matched sibling transplants. For the past two years, the performance of UBMT has been rapidly increasing and it will be possible to analyze much larger number of patients soon in Korea. In the future the problems of graft failure, GVHD, and infection due to long lasting immunocompromised status will need to be overcome by continued medical research. In addition, the volunteer donor pool will have to be expanded by the promotion of the national awareness of its need.

인천지역 TDI 노출 작업자에서 직업성천식 유병율

김형렬,김철우,홍윤철,전형준,김치년,김현수,이지나,신주연,고동희,노재훈 大韓産業醫學會 2004 대한직업환경의학회지 Vol.16 No.2

목적 : TDI는 직업성천식을 일으키는 대표적인 물질로 알려져 있다. 과거 TDI에 노출된 작업자들의 천식 발생률을 5~6%가량 보고하였고, 국내에서도 2-20%에 이르는 유병율을 보고한바 있다. 작업환경이 많이 개선되고, 노출량이 감소된 최근에도 TDI에 의한 직업성천식에 대한 보고가 많이 이루어지고 있어, 이에 대한 적극적인 조사와 진단과정을 통해 유병율을 구하고 질병발생의 변화양상을 파악해 보고자 하였다. 방법 : 인천지역에서 특수건강진단을 수행하는 2개 기관의 TDI 노출 근로자 400여명 중, 사업장의 규모가 50인 이상이며 사업주가 연구진행을 허락한 사업장에서 근무하는 근로자 중 직접적으로 TDI에 노출되는 작업에 종사하는 근로자 170명을 연구대상으로 하였다. 이들에 대해 설문지와 산업의학의사의 면담을 통한 작업관련성 평가를 근거로 정밀대상자를 선정하고 이들에 대해 메타콜린 유발시험, TDI 유발시험 등을 수행하여 직업성천식을 확인하였다. 결과 : 설문조사 결과 11명이 직업과 관련하여 천식증상을 보이는 것으로 나타났고(6.9%), 이중 7명에서 메타콜린 유발검사 양성소견을 보였다. TDI에 의한 특이유발시험 결과 1명에서 양성반응이 나타나, 이 집단에서 TDI에 의한 직업성천식의 유병율은 0.58%였다. 결론 : 과거 국내연구에서 TDI에 의한 직업성천식의 유병율이 2~20%에 이르렀던 데 비해, 본 연구에서는 0.58%로 나타났다. 이는 국외 논문에서도 나타나는 경향으로 작업장 노출량 감소로 점차 발생률이 감소하는 양상을 보이고 있다. 그러나 이미 증상이 나타난 근로자들이 부서를 옮기거나, 직장을 그만둠으로 인해 그 유병율이 과소평가되었을 가능성도 있다고 판단된다. Objecdives: This study was carried out to estimate the prevalence of isocyanate-induced occupational asthma in toluene diisocyanate (TDI) exposed workers. Methods: We examined 170 workers who had been directly exposed to TDI through a medical questionnaire, physical examination, and pulmonary function test. Based on screening examination, workers with suspected occupational asthma were selected for further evaluation such as methacholine and TDI challenge tests. Results: Eleven (6.9%) among 170 workers complained of symptoms of occupational asthma. and 7 among these 11 symptomatic workers showed positive responses to the methacholine challenge test (4.1 %). One spray painter was confirmed as having the TDI induced occupational asthma following a positive response to TDI challenge test. Conclusions: The prevalence of TDI-induced asthma was at 0.58% was lower than that for former studies (2-20%). Improved workplace environment, lower level of TDI exposure compared to the past, and the healthy workers effect may have contributed to this low rate of asthma prevalence in workers with TDI exposure.

소규모사업장 용접근로자들의 소음·용접흄·망간 노출에 관한 연구

김동구,문덕환,이채관,박명희,함성애,손병철,김대환,이창희,김휘동,이채언 인제대학교 백병원 2002 仁濟醫學 Vol.23 No.5

Objective : This study was conducted for the purpose of investigating the level exposure of welders to noise, welding fume and Manganese(Mn) related to this process. Methods and Material : All subjects in this study were welders engaged in CO2 arc and coated arc welding process especially in small-scale enterprises classified by type of manufacture of ship building, repairing and of other fabricated metal products, which is located in Gimhae and Milyang of Kyeongsang province in south Korea. Air sampling of welding fume was measured, shipped and analyzed on the basis of national institute for occupational safety and health(NIOSH) method in U.S. and work environment measurement method established in south Korea. To measure Noise in the work environment, Noise dosimeter was used on the basis of work environment measurement method established in south Korea. Result : This study was performed during a period from January, 2000 to December, 2001 and Results were as follows: 1. Noise level measured in this work environment was higher than national criteria for permissible exposure limit on health examinations related to hearing loss. 2. The concentration of welding fume and Mn in manufacture of ship building and repairing was higher than those in manufacture of other fabricated metal products. 3. In conditions of existing in ventilation system installed and operated, the concentration of welding fume and Mn was slightly lower than those of not existing. 4. In conditions of existing in sufficient air supply from out door into the work place relied on natural exhaust system, the concentration of welding fume and Mn was slightly lower than those of in not sufficient. 5. In all subjects, a rate of wearing ear plug and respiratory mask for dust and fume was lower than that of not wearing. Conclusion : According to above results, this study suggests that more positive work environment managements such as the use of portable local ventilation system, the supply of highly qualified personal protective equipment, and consecutive education and training related to health protection should be need to acquire suitable health protection program for welders engaged in small-scale enterprises in Korea.