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혈액투석 환자에서 유전성출혈모세혈관확장증에 의한 폐동정맥기형
박세영 ( Seyoung Bahk ),부성현 ( Seong Hyeon Bu ),김형덕 ( Hyung Duk Kim ),원유동 ( Yoodong Won ),이해규 ( Hae Giu Lee ),김영옥 ( Young Ok Kim ) 대한내과학회 2021 대한내과학회지 Vol.96 No.3
Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia. (Korean J Med 2021;96:247-251)