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증례 : 순환기 ; 성인에서 악성고혈압을 동반한 누난증후군
엄문용 ( Moon-yong Eom ),김원장 ( Won Jang Kim ),김경희 ( Kyung Hee Kim ),김유나 ( Yu Na Kim ),최웅 ( Woong Choi ),정영리 ( Young Lee Jung ),조현아 ( Hyun Ah Cho ) 대한내과학회 2015 대한내과학회지 Vol.89 No.4
본 저자들은 호흡곤란과 악성고혈압으로 내원한 27세 남자에 대해 이차성고혈압을 의심하에 평가하던 중 전형적인 이학적 소견 및 PS 및 HCMP 소견을 통해 NS로 최종 진단된 환자에서 신동맥협착 소견이 동반된 증례를 문헌고찰과 함께 보고한다. 한편 환자에게 동반된 소견 중 폐협착에 대해서는 경피적 풍선 폐동맥판막성형술을 통한 시술로 해결하였으며 신동맥협착 소견에 대해서는 약물치료를 계획하였다. Noonan syndrome is an autosomal dominant disorder characterized by dysmorphic facial features, congenital heart defects and short stature. To date, renal artery stenosis has not been associated with Noonan syndrome. We report the case of a 27-year old male who presented with malignant hypertension associated with renal artery stenosis, dysmorphic facial features, pectus excavatum, pulmonary stenosis and hypertrophic cardiomyopathy who was diagnosed with Noonan syndrome. (Korean J Med 2015;89:433-438)
김태훈,오영수,엄문용,정영리,조현아,최웅,심원흠 대한심장학회 2014 Korean Circulation Journal Vol.44 No.6
A 71-year-old woman who had suffered from pulmonary thromboembolism with deep vein thrombosis for 12 years presented the hospitalwith a huge thoracic aortic aneurysm. During thoracic endovascular therapy, she had a sudden coronary artery occlusion without havingorganized stenosis or plaque rupture even under the dual antiplatelet treatment and heparinization. She turned out to be having a proteinS deficiency. A procedure related thrombotic adverse event in patient with protein S deficiency is very rare, so we report a case with literaturereview.
정영리 ( Young-lee Jung ),최락경 ( Rak-kyeong Choi ),엄문용 ( Moon-yong Eom ),조현아 ( Hyun-a Cho ),최웅 ( Woong Choi ),김동진 ( Dong-jin Kim ),함의근 ( Ui-geun Ham ) 대한내과학회 2016 대한내과학회지 Vol.91 No.2
본 증례에서는 드문 전이성 병변인 범발성 평활근종증과 혈관성 평활근종증이 동시에 발현된 환자에게 다학제 협진을 통한 접근으로 한 단계로 완전한 수술적 절제를 시행하고 재발 없이 치료를 받은 환자의 증례를 보고하였으며 이러한동일한 근육에서 유래된 두 전이성 병변의 연관성에 대하여는 아직 밝혀진 바가 없으므로 이에 대한 연관성에 대한 연구가 필요할 것으로 생각되어 간단한 문헌고찰과 함께 보고하는 바이다. Leiomyomatosis refers to benign smooth muscle cell tumors that often arise from unusual growth patterns and include benign metastasizing leiomyoma, disseminated peritoneal leiomyomatosis, and intravenous leiomyomatosis. Intravenous leiomyomatosis is the extension of a vascular tumor into the venous channels, whereas disseminated peritoneal leiomyomatosis is characterized by multiple leiomyomas growing along the submesothelial tissues of the abdominopelvic peritoneum. It is extremely rare for intravenous leiomyomatosis and disseminated peritoneal leiomyomatosis to occur simultaneously. A 42-year-old female presented with disseminated peritoneal leiomyomatosis and intravenous leiomyomatosis extended through the inferior vena cava into the right side of the heart. The patient underwent one-stage surgery under simultaneous sternotomy and laparotomy, and radical excision of the tumor was achieved using cardiopulmonary bypass. Here we describe a case in which complete removal of a leiomyomatosis with an unusual growth pattern was successfully performed using one-stage surgery. (Korean J Med 2016;91:185-190)