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이봉우,서중석 大韓法醫學會 1999 대한법의학회지 Vol.23 No.1
Congenital pulmonary lymphangiectasis is a rare cause of severe respiratory distress in the newborn period and most of these patients die, usually within the first 24 hours of life. The light microscopic characteristics of the lungs are a network of partly tubular, partly cystically enlarged lymph vessels with-in large areas of connective tissue and they have thin walls lined by endothelium. Also congenital pulmonary lymphangiectasis is closely associated with cardiovascular malformation, and most frequently occurs in the clinical setting of congenital heart disease with or without pulmonary venous obstruction(total anomalous venous return, atresia of large pulmonary veins). The authors describe three typical cases of congenital pulmonary lymphangiectasis.
양경무,권일훈,조갑래,서중석 大韓法醫學會 1999 대한법의학회지 Vol.23 No.1
Adult polycystic kidney disease is a genetic disease characterized by bilateral multiple renal cysts and is transmitted as an autosomal dominant traits. The disease usually manifests in the fourth decade of life. The affected patients usually die with end stage renal failure, cardiovascular assaults and infection etc. Cardiovascular assaults include ruptured berry aneurysm, spontaneous intracranial hemorrhage, acute myocardial infarction, dissecting aortic aneurysm and hypertensive heart failure. Fatal intracranial hemorrhage and hypertensive heart failure occur in 15% and 2% of patients, respectively. Genetically-dater-mined structural weakness in the arterial wall have been suggested as a possible factor in the genesis of berry aneurysm. We have experienced three autopsy cases with cardiovascular causes of sudden death. Two cases have intracranial hemorrahage (1 berry aneurysm and 1 intracerebral hemorrhage) and the remaining one is dead with hypertensive cardiomyopathy. All of them are aged over 40 years and associated with polycystic liver disease. Histologically, myriad cysts of enlarged bilateral kidney are lined by single layer of cuboidal cells with focal hyperplastic epithelial configuration. Diffusely scattered liver cysts are also lined by flat to cuboidal epithelium and often associated with portal fibrosis.