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증례 : 순환기 ; 제1형 신경섬유종증 환자에서 나타난 폐고혈압 1예
나수진 ( Soo Jin Na ),이혜연 ( Hye Yeon Lee ),김현선 ( Hyun Seon Kim ),성현진 ( Hyeon Jin Seong ),전부석 ( Bu Seok Jeon ),전희경 ( Hui Kyung Jeon ) 대한내과학회 2013 대한내과학회지 Vol.85 No.5
신경섬유종증과 관련된 폐고혈압은 심각한 기능적, 혈역 학적 장애를 초래할 수 있다. 따라서 신경섬유종증 환자에서 호흡곤란, 기절, 피로 등 폐고혈압의 증상이나 징후가 나타 난다면 임상의들은 가능한 조기에 심초음파 등의 검사를 하여 폐고혈압을 발견하고 치료를 시작할 수 있도록 해야 한다. 또한 신경섬유종증과 관련된 폐고혈압의 병태생리적 이해를 위해 체계적이고 규모 있는 연구가 필요하며 이를 바탕으로 생존율 향상에 도움이 되는 추가적인 치료법에 대한 논의가 이루어져야 할 것이다. Neurofibromatosis type I is a genetic disease caused by mutations in the neurofibromin 1 (NF1) gene. Although it is characterized by a number of distinct clinical features, including cafe au lait macules, freckling in the axillary or inguinal regions, neurofibromas, and Lisch nodules (iris harmartomas), it can affect all physiological systems in the body [1]. Neurofibromatosis-related pulmonary hypertension has also been reported, and some patients showed a poor prognosis despite having received proper medical treatment [2-4]. We herein describe a case of pulmonary hypertension in a patient with neurofibromatosis type I who had no identified risk factors of pulmonary hypertension. To our knowledge, this is the first such report in Korea. (Korean J Med 2013;85:521-525)
장경윤,박경선,최유아,김지희,전부석,허성호 이화여자대학교 의과학연구소 2012 EMJ (Ewha medical journal) Vol.35 No.1
Arterial remodeling is commonly observed in human atherosclerosis. It is a heterogeneous response ranging from positive remodeling to negative remodeling. Negative remodeling is a condition in which the vessel area decreases in size, often as a result of a structural change in the coronary vessel wall. But its contribution to myocardial ischemia in a de novo lesion has not been clearly shown. A 51-year-old female with exertional angina was admitted to our hospital. Coronary angiography was performed, revealing a severe stenosis at the middle part of the right coronary artery (RCA). Although we predilated ballooning at the middle RCA, the degree of stenosis did not improve. Thus intravascular ultrasound (IVUS) was performed. The lesion was not nearly showed plaque burden and severe negative remodeling. Though the cross-sectional narrowing percentage was significant, we decided to medical treatment for fearing coronary perforation by stenting. This case report intends to emphasize that severe coronary stenosis should be performed IVUS before the stenting. We describe a rare case with severe negative remodeling at the middle part of the RCA without atheroma plaque.
횡문근융해증과 경한 신손상을 동반한 급성 A형 간염 1예
조규민,김창욱,성현진,허준,전부석,이종환,심은희,이석종,이창돈 영남대학교 의과대학 2012 Yeungnam University Journal of Medicine Vol.29 No.1
A 48-year-old male visited the emergency room of the authors’ hospital due to nausea, vomiting, and myalgia for four days. Acute hepatitis A was identified from the serologic marker of the hepatitis A virus. Mild elevation of the serum creatinine and creatinine phosphokinase(CPK) suggested rhabomyolysis, which was confirmed with the serum aldolase, myoglobin, and urine myoglobin. With supportive care, both the liver and renal func- tions were recovered gradually and fully. This case shows that rhabdomyolysis can be one of the mechanisms of renal complication in cases of acute symptomatic hepatitis A.