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      • 가와사끼병에서 γ-글로블린 1회 대량요법과 분주요법의 치료 효과

        길홍량 충남대학교 의과대학 지역사회의학연구소 1993 충남의대잡지 Vol.20 No.2

        We studied 58 children , who were diagnosed as Kawasaki disease at department of ped. Chungnam university hospital from Jan,1990 to Nov. 1993. These cases were divided into two groups according to the mode of therapy administerated during acute phase : 18 cases in group A with a combination of intravenous gammaglobulin(2gm/kg single) and oral aspirin (100mg/kg/day) and 40 cases in group B treated with combination of intravenous gammaglobulin (400mg/kg/day 5 days)and oral aspirin. The clinical and echocardiographic findings were as follows 1) The age & sex distribution was comparable in both groups : the mean age was 30.0± 17.8 months for group A and 35.6± 28.7 months for group B. There were 13 boys and 5 girls in group A and 28 boys and 12 girls in group B. 2) Single high dose gammaglobulin was started at 6.1± 2.1 days in group A and at 6.1± 2.6 days in group B after onset of illness. 3) No significant difference was noted in the hemoglobin, WBC count, platelet count & ESR during course of disease. 4) The echocardiographic abnormalities at 2 month after onset were noted in 3 cases (16.6%) out of 18 cases in group A and in 10 cases (25.0%) out of 40 in group B. The author concluded that single high-dose intravenous gammaglobulin compared to splitted dose of that, is safe and effective in reducing the clinical findings including the fever and the prevalence of coronary abnormalities.

      • CATCH22의 분자유전학적 특성에 관한 연구

        길홍량,이영하,정용헌 충남대학교 의과대학 지역사회의학연구소 2000 충남의대잡지 Vol.27 No.2

        To investigate molecular genetic characteristics of CATCH 22 syndrome, we studied the correlation between phenotype and target gene screening and mode of inheritance using FISH (fluroscence in situ hybridization) and STRP(short tandem-repeated polymorphism). Hemizygosity for a region of 22q11.2 was found in 21 CATCH 22 patients. 2 of 19 family (11%) had familial CATCH 22 syndrome and affected 2 parents were mothers. No familial cases were found among CATCH 22 syndrome with absent thymus/ DiGeorge syndrome. The conotruncai cardiac anomalies such as Tetralogy of Fallot and vascular anomalies were common among CATCH 22 syndrome. Both the STRP maker screening(D22S941) and FISH(D22S75) were efficient and useful for the detection of chromosome 22q11 deletion and the evaluation of parent of origin but show some heterogeniety. Markers D22S75 and D22S941 appear to be consistently hemizygous in patients with deletions, so it was very useful in screening the patients with the conotrucal cardiac anomalies.

      • KCI등재

        소아 심근염과 심근증

        길홍량 대한소아청소년과학회 2007 Clinical and Experimental Pediatrics (CEP) Vol.50 No.11

        Myocarditis represent an important condition encountered by general pediatricians & general practitioners. Its presentation is varied, and therefore a high index of suspicion must be maintained when the possibility of myocarditis is raised. A progression from viral myocarditis to dilated cardiomyopathy has long been hypothesized. Treatment is initially aimed at achieving hemodynamic stability and is largely supportive. There is currently little evidence to support the immunomodulatory or specific antiviral therapies. Pediatric cardiomyopathies are a heterogeneous group of disorders with diverse genetic, infectious, mitochodrial and metabolic etiologies. The timing and severity of presentation vary according to cardiomyopathy type as well as genetic and ethnic factors. The behavior of specific cardiomyopathies can be predicted by morphological and functional attributes, as well as underlying patient characteristics.

      • 심도자술 및 심혈관조영술을 시행한 소아 심혈관질환의 임상적 고찰

        길홍량 충남대학교 의과대학 지역사회의학연구소 1995 충남의대잡지 Vol.22 No.2

        A clinical study was made on 195 patients with cardiovascular disease who were admitted to the department of pediatrics Chungnam National University Hospital & taken cardiac catherterization and cineangiography from March 1993 to December 1995. The results were as follows 1) The ratio of male to female was about 1 : 1 among 195cases, male 99 cases, female 96 cases. 2) The age distribution of the patients with cardiovascular disease was as follows ; 7.2% under 1 month of age, 35.4% under lyear of age, 80% under 5year of age, & 20% over 5year of age. 3) The distribution of cardiovascular disease in order of frequency as follows : VSD 42.6%, Tetrallogy of Fallot 11.3%, Patent ductus arteriosus 8.7%, Atrial septal defect 7.7%, Pulmonary stenosis 5.1%. 4) Kawasaki disease with aneurysm & transposition of grat arteries were appeared as male dominance. Patent ductus arteriosus was female preponderence. 5) The ratio of acyanotic to cyanotic congenital heart disease was 3.8 : 1. 6) Major complication during cardiac catheterization & cineangiography appeared in 19 cases (9.7%) : supraventricular tachycardia 9cases, bleeding requiring transfusion 5cases, & death 3 cases. The causes of death were cardiac perforation & arrythmia.

      • KCI등재
      • KCI등재후보

        CATCH 22 증후군의 22q11 미세결실 및 임상-유전적 상관성에 관한 연구

        길홍량,이영하,정용현 대한소아청소년과학회 2000 Clinical and Experimental Pediatrics (CEP) Vol.43 No.12

        목 적 : CATCH 22 환자와 그 가족들을 대상으로 이형증성 표현형의 분석을 통하여 인식 가능한 증후군의 기준을 제시하고, 높은 다형성을 보이는 STRP 표지자를 이용한 이종접합성의 소실을 가족단위로 검색함으로써 한국인 고유의 새로운 다형성 유전적 표지자 개발의 기초 자료로 활용함은 물론 임상-유전학적 상관관계의 확인을 통해 유전상담에 도움을 주고자 하였다.방 법 : 선천성 심질환과 구개열을 주소로 충남대학교병원에 내원한 환자 중 CATCH 22 환자와 그 가족들을 대상으로 임상 표현형 분석과 STRP 표지자를 이용한 이종접합성의 소실을 가족단위로 검색하였다. Purpose : Deletion of chromosome 22q11 is associated with DiGeorge syndrome, velocardiofacial syndrome, and conotruncal anomaly face syndrome. This study was performed to determine the criteria of clinical phenotype as recognizable syndrome and to research the loss of heterozygosity in CATCH 22 patients and their family. Methods : An evaluation of the clinical and genetic profiles of 30 persons of CATCH 22 syndrome or their family referred with a diagnosis of either congenital heart disease or cleft palate was undertaken. The deletions of 22q11 were analyzed using the fluorescences in situ hybridization(N25, Oncor) and short tandem-repeat polymorphic makers(STRP, D22S941). Results : The dysmorphic features of CATCH 22 showed considerable overlap and intrafamilial difference was common. The familial cases of CATCH 22 were transmitted maternally as autosomal dominant. The target gene study using the STRP maker(D22S941) in these series showed good clinico-genetic correlation but some heterogeneity. Conclusion : Although 22q11 deletion was large in size and high variable in polymorphic markers, extensive evaluation clinically as well as genetically will be necessary for subgrouping of CATCH 22 syndrome due to good clinicogenetic correlation. Furthermore, we also suggest the development of new polymorphic markers to research the unknown characteristics of polymorphic markers in Korean patients with CATCH 22 syndrome.

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