Rhizomelic Chondrodysplasia Punctata I형 1례

김달현,권영세,전용훈,홍영진,손병관,윤혜란,Kim, Dal Hyun,Kwon, Young Se,Jun, Yong Hoon,Hong, Young Jin,Son, Byoung Kwan,Yoon, Hye Ran 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.12

저자들은 신생아 시기에 얼굴의 이형성과 사지 근위부 단축으로 입원한 환아에서 생화학검사, 유전자 검사, 섬유아세포 배양으로 국내 최초로 확진된 RCDP 1형 1례를 보고하는 바이다. Rhizomelic chondrodysplasia punctata(RCDP) is a rare autosomal recessive disorder clinically characterized by symmetrical shortening of the proximal limbs, contractures of joints, a typical dysmorphic face, cataracts, and itchyosis. Patients with RCDP can be subdivided into three subgroups based on biochemical analysis and complementation studies. RCDP type I results from mutations in the PEX7 gene encoding the peroxisomal targeting signal type II(PST2) receptors and presents with both a defect in plasmalogen biosynthesis and phytanic acid oxidation. RCDP type II is deficient in the activity of dihydroxyacetonephosphate acyltransferase(DHAP-AT). RCDP type III is deficient in alkyl-dihydroxyacetonephosphate synthase(alkyl-DHAP). We report a case of RCDP type I which was confirmed with biochemical study, fibroblast culture, and gene study.

청소년에서의 체지방 분포와 대사위험 인자들의 연관성

김달현,성태정,홍영진,손병관,김순기,Kim, Dal Hyun,Seong, Tae Jung,Hong, Young Jin,Son, Byong Kwan,Kim, Soon Ki 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.4

목 적 : 이번 연구의 목적은 다양한 비만지표를 이용하여 인천지역 중고등학교의 비만 유병율을 알아보았으며, 비만지표와 비만의 합병증인 고혈압과 고지혈증의 연관성을 알아보고 중심성 비만을 평가하기 위해 허리둘레 측정치와 WHR의 유효성을 알아보았다. 방 법 : 2000년 4, 5월 인천지역의 3개의 중학교, 5개의 고등학교를 선별하여 11세에서 18세까지의 총 1,382명(남 : 여=690 : 692)을 대상으로 신체계측과 혈청지질농도, 혈압을 측정하였다. 결 과 : 1) 비만의 유병율은 BMI를 기준으로 분류할 때 남자 6.7%, 여자 6.9%이었고, 허리둘레 측정치를 기준시 남자 5.4%, 여자 5.0%이었고, WHR 기준시 남자 7.0%, 여자 5.8%이었다. 2) 남녀 모두에서 BMI, 허리둘레, WHR은 수축기 혈압과 확장기 혈압 모두 양의 유의한 상관관계가 있었다. 3) 남자에서 중성지방은 BMI(r=0.13, P<0.05), 허리둘레 측정치(r=0.34, P<0.05)와 양의 유의한 상관관계가 있었다. 여자에서는 지질농도와 유의한 상관관계가 없었다. 4) 남자에서 BMI는 비만도(r=0.98, P<0.05), 허리둘레 측정치(r=0.89, p<0.05), 체지방(r=0.85, P<0.05)과 높은 상관관계가 있었다. 여자에서 BMI는 비만도(r=0.98, P<0.05), 허리둘레 측정치(r=0.82), 체지방(r=0.89, P<0.05)과 높은 상관관계가 있었다. 결 론 : 이번의 결과는 고혈압의 유병율이 정상체중군에 비해서 남녀 모두 비만할수록 증가하였고 비만지표와 상관관계가 있었다. 소아비만과 관련된 고혈압은 성인 혈압의 가장 뛰어난 예측인자이고 성인 비만으로의 지속과 무관하게 고혈압을 유발시키므로 청소년의 비만을 예방하고 치료하는 것이 필요하다. Purpose : The objectives of this study were to estimate the prevalence of obesity in adolescents; to study the association between obesity, blood pressure, and dyslipidemia; and to evaluate the superiority of waist circumference to waist/hip ratio(WHR) in central obesity. Methods : We selected 1382 adolescents(M : F=690 : 692) aged from 11 to 18 years(mean age; boys 15.0, girls 15.4) in Incheon. We measured weight, height, waist, waist/hip ratio, and blood pressure, with blood sampling to evaluate the dyslipidemia. Results : The prevalence of obesity was 6.7% in boys and 6.9% in girls by body mass index(BMI). The prevalence of systolic hypertension was 15.7% in normal group, 32.4% in the overweight group, and 35.4% in the obese group by BMI. The prevalence of diastolic hypertension was 4.9% in the normal group, 6.9% in the overweight group, and 14.6% the in obese group. In boys and girls, BMI, waist circumference, and WHR were positively correlated with systolic and diastolic blood pressure. respectively. In boys, triglyceride was positively correlated with waist circumference(r=0.34, P<0.05). In boys and girls, BMI was highly correlated with obesity index(r=0.98, each), with waist circumference(r=0.89, r=0.82, respectively), and with body fat(r=0.85, r=0.89) respectively. Conclusion : These data suggest the importance of the screening of blood pressure in obese adolescents, with prevention and intervention of obesity since it may be the most cost-effective way of reducing the complications related with obesity.

발전과정에 있어서 제도개혁의 역할과 역량개발

김달현(Kim, Dal Hyun) 국제개발협력학회 2010 국제개발협력연구 Vol.2 No.2

This paper is to concern with paradigm shift of aid and development, the important role of institutional reform and capacity development to enhance development and aid effectiveness in developing countries. The traditional approach of aid and development was moved away and putting institutional development at the center of aid efforts and development made a radical paradigm shift in the way of doing cooperation. In this new paradigm, institutional development becomes both a key objective of cooperation as well as a method of work; i.e. an overall approach geared at delivering aid in ways that build ownership, mobilize local capacity and produce institutional change, improving governance, policy reform and development. Mainstreaming institutional development is both a timely and complex exercise. In order to enhance the exercises of success, it seems useful to agree on a set of basic premises(or guiding principles) for effective implementation. Institutional reforms for good governance are key elements in the process of development for all developing countries. Development can not be achieved without improved governance and institutional reform including capacity development. The major role of implementing these tasks must be played by governments of developing countries in collaboration of international donor agencies. Success or failure of institutional development mainly depends on ownership, leadership, strong commitment and capacity development at home. The heart of the problem in developing countries is not the lack of funding or technical know-how, but a matter of governance and the resulting inability to make good use of existing institutions and capacities. Helping to overcome these fundamental institutional barriers to development is the key challenge for the international donor community. Needless to say that if donor agencies want to provide an effective contribution to this agenda, they will need to reform their own corporate culture and management system, procedures and capacities.

A Pathological Observation on Pulmonary Mycoplasmosis in the Mice

Kim Dal-Hyun(김달현) 한국실험동물학회 1988 한국실험동물학회 학술발표대회 논문집 Vol.1988 No.-

발전과 양호한 거버넌스(good governance)

김달현(Kim, Dal-Hyun) 국제개발협력학회 2009 국제개발협력연구 Vol.1 No.1

This paper is to explore several important issues in the process of aggregate development process. The concept of aggregate development covers the more comprehensive concept than those of economic and social development. In other words, development means the progress or process of all the sectoral society such as economy, society, politics, science, culture and technology. Ultimately, it is to make better world over time. In the recent, sustainable human development was appeared in line with the holistic view of development. Sustainable human development means the process of meeting the present human needs without endangering the opportunities of the future generation. Hence, it has the nature of political, economic, social, and environmental dimensions. While economic development is concerned with the material-oriented welfare, sustainable human development is concerned with human-oriented(or human-centered) welfare. The role of governance in connection with development is widely and increasingly recognized in international development cooperation arena. Governance means the aggregate structure relevant to the allocation and management of economic and social resources, and is to achieve the goalssuch as socio-economic development, social order and peace, political freedom and participation. The actors of governance are state, private sector and civil society. The characteristics of governance are participation, accountability, transparency, rule of law, responsiveness, consensus orientation, equity, and effectiveness and efficiency. When these elements are successfully established, we will be able to achieve good governance, through which sustainable development can be attained. Good governance can provide us with political, economic, social and environmental benefits. This paper also attempts to explore the relationship between governance and development through the analysis of comparative study by using World Bank s country-specific governance indicators (i.e. U.K, Korea, Botswana, China, India, Columbia, Uzbekistan and Zimbabwe). There has been no generally agreed view on the relationship between governance and economic development. However, there is some supporting evidence that there is a close linkage between governance and economic development from the cross-section analysis of many countries. For instance, the empirical evidence shows that Eastern European countries pursued market economy and democracy, so they achieved successful development. (i.e. the cases of Poland, Hungary and Czeco Republic in Eastern Europe. However, China adopted the socialist market economic development scheme under authoritarian government (gradual approach), so that China achieved rapid economic development without expanding political freedom of the people. On the other hand, Russia experienced social order and economic collapse by adoption of Big Bang approach, because she used open-door policy with excessive political freedom and without institution building. In contrast to China, India has experienced sustainable development under democratic regime. On governance and development, there are two theories; growth-enhancing governance and market-enhancing governance. In 1960s and 1970s, growth-enhancing governance was prevailed while marketenhancing governance was popular after the 1980s. There have been disagreed views on economic development of developing countries. In order to promote development, the reforms of both administrative /judiciary governance and economic governance (institution building and capacity development) are essentially required, and they eventually can contribute to enhancing political and socio-economic development, if successful. It is concluded that development can not be attained without reforms of governance.

1,2-Dimethylhydrazine Dihydrochloride 유발 랫드 대장암 모델에서 p53의 돌연변이 발생에 관한 연구

김달현(Dal-Hyun Kim),허권(Kwon Hur),서일복(Il-Bok Seo),김대용(Dae-Yong Kim) 한국실험동물학회 1999 Laboratory Animal Research Vol.15 No.1

CHO 세포에서 생산된 재조합 Erythropoietin ( EPO ) 의 약효

김현수(Hyun Su Kim),박관하(Kwan Ha Park),김달현(Dal Hyun Kim),이동억(Dong Eok Lee),김석준(Suk Joon Kim),하병집(Byung Jhip Ha),오명석(Myung Suk Oh) 한국응용약물학회 1994 Biomolecules & Therapeutics(구 응용약물학회지) Vol.2 No.4

In vivo activity of recombinant human erythropoietin (rh-EPO) has been examined using polycythemic model in mice and acute hemorrhage model in rats. The number of reticulocytes in blood stream was increased after a single injection of rh-EPO depending on the dosage of rh-EPO in polycythemy model. It seemed that optimal dose of rh-EPO for polycythemic mice was around 1-10 U/kg. Rh-EPO also showed the effectiveness for increase of reticulocyte numbers both in male and female rats after bleeding. The number of reticulocytes and the change of hemoglobin concentration in the blood stream of normal rats has been examined after injection of rh-EPO. The maximum value of reticulocyte was observed on the 6th day of the injection in these normal rats. In addition, the increase of reticulocyte and the concentration of hemoglobin were dependent on the dosage of rh-EPO. The increase of hemoglobin concentration was continued to the 9th day after injection. In this study, the efficacy of rh-EPO was confirmed in both mice and rats.

새로운 유전자 재조합 기술에 의하여 생산된 Erythropoietin 의 일반약리작용

김현수(Hyun Su Kim),박관하(Kwan Ha Park),김달현(Dal Hyun Kim),이동억(Dong Eok Lee),김영훈(Young Hoon Kim),정성목(Seong Mok Jeong),임동문(Dong Moon Lim),조효진(Hyo Jin Cho),정재경(Jae Kyung Jung) 한국응용약물학회 1994 Biomolecules & Therapeutics(구 응용약물학회지) Vol.2 No.4

The general pharmacological properties of EPO were investigated in various animals administering intravenously and in vitro system. The results were as follows. 1. Central nervous system: EPO at doses of 70, 700, 7000 U/kg showed no effect in mice on general behavior, on strychnine- an ntetrazol-induced convulsion and on acetic acid-induced writhing syndrome. The hexobarbital-induced sle g time in mice was slightly reduced by EPO at a dose of 7000 U/kg but did not change at doses of 70, 700 U/kg. The body temperature in rats was slightly decreased by EPO at doses of 700, 7,000 U/kg but the change was in normal physiological range. 2. Respiratory and cardiovascular system: EPO showed no effect on respiratory rate, blood pressure, heart rate, femoral blood flow, and electrocardiogram in anesthetized dogs at doses of 70, 700, 7000 U/kg. 3. Smooth muscle: EPO at concentrations of 70, 700 U/ml had no effect on the contractile response of isolated guinea pig ileum to histamine and acetylcholine. 4. Water and electrolytes excretion: EPO at dose above 700 U/kg increased urine volume in rats but did not affect the concentrations of Na^+, K^+, Cl^- in urine. 5. Gastrointestinal system: EPO(70, 700, 7000 U/kg) had no effect on the intestinal charcoal meal propulsion in mice. 6. Blood coagulation system: The administration of EPO(70, 700, 7000 U/kg) had no effect on the plasma prothrombin time(PT) and activated partial thromboplastin time(APTT) in mice. Platelet aggregation induced by ADP and collagen was not influenced by EPO(70 U/ml, 700 U/ml). The overall results obtained indicated that EPO exerts almost no serious pharmacological effect even at a 100-fold clinical dose(7000 U/kg).

정상 ICR mouse 및 SD rat 에서 CJ-50001 (rG-CSF) 의 단회투여후 말초호중구수의 변동 및 용량상관성

김제학(Je Hak Kim),김현수(Hyun Su Kim),김달현(Dal Hyun Kim),임동문(Dong Moon Lim),조효진(Hyo Jin Cho),이현수(Hyun Soo Lee) 한국응용약물학회 1997 Biomolecules & Therapeutics(구 응용약물학회지) Vol.5 No.4

CJ-50001 is a recombinant granulocyte-colony stimulating factor (rG-CSF) developed by Cheil Jedang R&D Center. The effects of CJ-50001 on the increase of peripheral neutrophil count following intravenous and subcutaneous single administration at a dose of 20 ㎍/kg in normal ICR mice and SD rats, respectively, were compared with those of Grasin, a control drug. Both CJ-50001 and Grasin significantly increased the peripheral neutrophil number in four treatment groups and the maximum number of neutrophil was achieved at 12 to 18 h in rats and mice, respectively. The dose dependency test was studied for CJ-50001 only in normal mice by intravenous or subcutaneous administration. When administered i.v or s.c at the various doses in normal mice, CJ-50001 significantly increased the neutrophil number over the dose of 160 ng/kg, compared with the vehicle control group. From these results, it was concluded that CJ-50001 showed efficacy similar to Grasin in the peripheral neutrophil count increase.

마우스에서 항암제 유발 호중구감소에 대한 CJ-50001 의 회복촉진효과

김제학(Je Hak Kim),김현수(Hyun Su Kim),백남진(Nam Jin Baek),김달현(Dal Hyun Kim),최재묵(Jae Mook Choi),강재구(Jae Ku Kang),김기완(Ki Wan Kim) 한국응용약물학회 1997 Biomolecules & Therapeutics(구 응용약물학회지) Vol.5 No.4

Neutropenia is a major dose-limiting side effect of cancer chemotherapy. The therapeutic effects of CJ-50001 were examined on neutropenia caused by anticancer agents. Neutropenia was induced by cyclophosphomide (130 mg/kg), doxorubicin (4.5 mg/kg), and vincristine (1 mg/kg) in normal ICR mice and by cyclophosphamide (200 mg/kg) in CT26 adenocarcinoma bearing BALB/C mice. After the subcutaneous injection of anticancer agents, we administered subcutaneously recombinant human granulocyte-colonystimulating factor (100 ㎍/kg/day) to mice in order to stimulate neutrophil production. In normal and tumor-bearing mice, neutrophil production efficacy of CJ-50001 (rG-CSF) was similar to that of Grasin. These results suggest that CJ-50001 could be effective in its clinical use for neutropenia treatment.