• Tuberculosis and Respiratory Diseases
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• 제50권3호
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• pp.378-384
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• 2001

Systemic arterialization of lung without sequestration은 폐격리증 없이 정상적인 폐실질과 기관지를 가지고 기관지계와 정상적으로 소통이 되어 있는 폐분절이 대동맥으로부터 분지한 기형 체동맥에 의해 동맥혈을 공급받는 보기 드문 폐혈관 기형으로 저자들은 증상이 없이 폐종괴로 오인되어 내원하여 흉부 전산화 단층촬영 및 혈관 조영술을 통하여 확진된 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제58권7호
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• pp.256-262
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• 2015

Purpose: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. Methods: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

• Journal of Chest Surgery
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• 제39권1호
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• pp.12-17
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• 2006

배경: 초 저체온 하 순환 정지는 총 폐정맥 환류 이상을 수술하는데 있어 중요한 보조 수단이다. 그러나 초 저체온 하 순환 정지 하에 심장 수술을 하는 것은 순환 정지 후 신경계 이상의 위험을 동반하고 있다. 이에 완전 순환 정지 없이 총 폐정맥 환류 이상을 수술하여 그 결과를 평가하고자 한다. 대상 및 방법: 2000년 4월에서 2004년 10월까지 10명의 환자의 의무 기록을 후향적으로 분석하였다. 결과: 해부학적 연결 이상의 위치는 심장 상부형이 7예,심장형이 1예, 심장 하부형이 2예였다. 평균 심폐기 가동 시간과 대동맥 차단 시간은 각각 116.8 $\pm$40.7분, 69.5$\pm$24.1분이었다. 수술로 인한 사망 예는 없었고, 합병증은 술 후 폐정맥 협착이 1예, 폐렴, 기흉, 창상 감염, 횡격막 마비가 각각 1예씩 있었다. 평균 16.6개월의 추적 관찰 기간동안 페동맥 협착이 없었던 모든 환아는 NYHA class I으로 지내고 있다. 결론: 총 폐정맥 환류 이상을 완전 순환 정지 없이 수술하여 아주 만족할 만한 결과를 얻을 수 있었다.

• 대한본초학회지
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• 제27권6호
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• pp.107-114
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• 2012

Objectives : In this study, we investigated the anti-diabetic and anti-inflammatory effects of water extract from leaves of Ligustrum japonicum (WLJ) in db/db mouse. Methods : The db/db mice were treated orally with WLJ (300 mg/kg/day) for 10 weeks to examine the long-term effects on hyperglycemia and glomerular tissue as well as biochemical and functional abnormalities in the kidney. Results : WLJ treatment markedly reduced plasma levels of glucose, triglyceride, creatinine, and systolic blood pressure in diabetic db/db mouse. Treatment of WLJ significantly increased plasma level of high density lipoprotein (HDL)-cholesterol. We also found that overexpressions of vascular cellular adhesion molecule (VCAM)-1 and endothelin (ET)-1 were observed in aortic tissue of db/db mouse, whereas, WLJ suppressed both expression of VCAM-1 and ET-1 in aorta. In renal tissue, overexpressions of ICAM-1 and TGF-${\beta}1$ were found in untreated db/db mouse, however, significantly decreased those levels by WLJ treatment. The insulin immunoreactivity of the pancreatic islets remarkably increased in WLJ treated db/db mouse compared with untreated db/db mouse. Taken together, WLJ treatment ameliorated hyperglycemia and hyperlipidemia via improvement of insulin secretion and lipid metabolism, respectively. Furthermore, WLJ treatment also ameliorated hypertension via inhibition of inflammatory process in vascular and renal tissues. Conclusions : Ligustrum japonicum has an anti-diabetic and anti-inflammatory effects in db/db mouse. Thus, these results suggested a beneficial effect of Ligustrum japonicum in treatment with diabetes and diabetic vasculopathy.