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Systemic Sclerosis Sine Scleroderma
김채기 ( Chae Gi Kim ),류헌모 ( Hun Mo Rhoo ),권중구 ( Joong Goo Kwon ),이창형 ( Chang Hyeong Lee ),송용호 ( Yong Ho Song ),최정윤 ( Jung Yoon Choe ) 대한류마티스학회 2000 대한류마티스학회지 Vol.7 No.3
Systemic sclerosis (SSc) is a generalized connective tissue disorder of unknown etiology. Clinically, there is a broad spectrum of disease ranging from widespread severe skin thickening to skin thickening limited to the distal extremities. In rare cases of systemic sclerosis, no cutaneous change only with internal organ involvement has been reported, which is called `systemic sclerosis sine scleroderma (ssSSc)`. We describe a patient with Raynaud`s phenomenon, who showed intestinal pseudoobstruction as a presenting symptom but did not show any skin change. She had also an esophageal motility disorder, but other organ involvement was not evident. Antinuclear antibody was positive. Her obstruction symptoms were improved by decompression by nasogastric tube and pharmaceutical treatment with erythromycin and octreotide.
박병규 ( Byeong Kyu Park ),조윤영 ( Yoon Young Cho ),류헌모 ( Hun Mo Rhoo ),조형호 ( Hyeong Ho Jo ),손민영 ( Min Young Son ) 대한내과학회 2016 대한내과학회지 Vol.91 No.2
본 증례는 후복막섬유증이 의심되어 내원한 환자에서, 골수외 형질세포종이 진단된 사례이다. 골수외 형질세포종이 두경부가 아닌 후복막강에 발생했다는 점에 의의가 있고 표준 치료인 방사선 치료가 아닌 전신 항암화학요법으로 초기치료를 했다는 점에서 의의가 있다. 후복막강종양 또는 후복막섬유증이 의심되는 환자에서 골수외 형질세포종의 가능성을 고려하여 검사가 진행되어야 한다. Extramedullary plasmacytoma (EMP) is a plasma cell tumor located outside of the bone marrow. It most often occurs in the upper respiratory tract (85%), as well as the head and neck, and very rarely occurs in the retroperitoneum. Here we report the case of a 57-year-old woman with retroperitoneal EMP. (Korean J Med 2016;91:216-223)