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급성 간부전과 용혈성 빈혈로 증상 발현을 보인 Wilson 씨병
권상옥(Sang Ok Kwon),이광훈(Kwang Hoon Lee),심영학(Young Hak Shim),박진현(Jin Hyun Park),장우익(Woo Ik Jang),전근제(Geun Jae Jeon),최승옥(Seoung Ok Choi) 대한소화기학회 1989 대한소화기학회지 Vol.21 No.1
The first manifestations of Wilsons disease are usually neuropsychiatric disorders and less fre- quently, hepatic disorders (cirrhosis or chronic hepatitis) or transient hemolytic anemia. The purpose of this work is to describe a less well known mode of onset of the disease characterized by the association of intravascular hemolysis and acute liver failure. The diagnosis of Wilson's disease was based in the presence of the Kayser-Fleischer ring and decreased serum ceruloplasmin, and acute intravascular hemolysis was based in the sudden develop- ment of anemia, the high value for the reticulocyte count, the absence of demonstrable bleeding, unconjugated hyperbilirubinemia. Necropsy revealed active liver cirrhosis, micronodular type with marked cholestasis. The mechanism of this syndrome is unknown. A hypothetical explanation might result from hepatic cell necrosis due to accumulation of copper, the consequences being acute liver failure and destruction of erythrocytes by the large amounts of copper released from the necrotic hepatic cells to the plasma. In conclusion: 1) The syndrome of acute intravascular hemolysis and acute liver failure is very suggestive of Wilsons disease. 2) This association can be the first clinical manifestation of the disease. 3) The treatment is generally unsuccessful, and the outcome is usually fatal.
1987년 영서 지방에 발생한 광대 버섯과(Amanita) 독버섯 중독 16예의 임상적 고찰
이광훈,이종원,민병철,최승옥,장우익,권상옥,박찬일,김양섭 대한내과학회 1990 대한내과학회지 Vol.38 No.1
The number of case of mushroom poisoning is increasing as a result of the increasing popularity of wild mushroom consumption. Reports of cases of mushroom poisoning and fatalities have been increases. Over-whelming majority of mushroom poisonings resulting in death are attributable to the genus Amanita. The annual recurrence of fatalities and the lack of awareness on the part of many physicians about Amanita poisonings emphasize the importance of our report. Clinical features and laboratory findings of 16 patients with suspicious mushroom poisoning treated on 1987 in Wonju christian Hospital are reviewed and the following results obtained. 1) The mushroom that ingested by expired patients has been identified as Amnita Virosa through consultation to department of mycology of Rural Development Administration. 2) The overall mortality rate of Amanita poisonings were 63%. 3) Incubation period after ingestion of mushroom was 10~24 hours (average 14.4±3.3 hours) and there were no significance between groups. 4) On peripheral blood examination the hemoglobin, hematocrit, and platelet count in group 2 were significantly low compare to group 1 (p<0.05). 5) When group 1 and group 2 were compared for results of SGOT, SGPT, creatinine, and prothrombin time, group 2 showed significantly high results than groue 1 (p<0.05).