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증례 : 혈관염으로 인한 혈담낭염이 발생한 현미경적 다발혈관염 1예
한지숙 ( Ji Suk Han ),이성연 ( Sung Yeon Lee ),김형직 ( Hyung Jik Kim ),송영림 ( Young Rim Song ),김좌경 ( Jwa Kyung Kim ),홍은영 ( Eun Yeong Hong ),민수기 ( Soo Ki Min ) 대한내과학회 2012 대한내과학회지 Vol.83 No.1
PAN에 의한 담낭 혈관염과 혈담낭염에 대한 보고도 있으나, 현미경적 다발혈관염에 의한 담낭염 및 담낭내 출혈은 저자들이 처음으로 증례 보고하는 바이다. Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis, primarily associated with rapidly progressive glomerulonephritis and alveolar hemorrhage. Approximately 50% of MPA cases are associated with gastrointestinal involvement, but rarely do cases involve the gall bladder. We report an unusual case of MPA complicated by hemocholecystitis. A 62-year-old woman was admitted to our hospital with rapidly progressive renal dysfunction and pneumonia unresponsive to antibiotics, A chest CT scan showed bilateral diffuse alveolar consolidation, and perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) staining was positive. During the course of hospitalization, the patient complained of severe abdominal pain, and an abdominal CT scan revealed acalculous cholecystitis with hemorrhage. Cholecystectomy was performed, and a gall bladder biopsy revealed fibrinoid necrosis of small arteries without granuloma. Cholecystitis should be considered in patients with unexplained upper abdominal pain and MPA.
폐동정맥 기형을 동반한 유전성 출혈경향 말초혈관 확장증 1 예
김삼수,백완기,이준희,김인한,고광곤,조철호,김여주,문태훈,김준미,박찬섭,민수기,조상균,조수신,정원모,주영채 대한내과학회 1996 대한내과학회지 Vol.50 No.4
Hereditay hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is a familial disease that is characterized by delicate, ectatic vessels in the skin, mucous membrane and viscera. These patients have generalized vascular derangement. Pulmonary arteriovenous fistular is a rare congenital condition originated from malformation of capillary development. Direct communications between branches of the pulmonary artery and pulmonary vein cause right-to-left shunts, in which mixed venous blood cannot be oxygenated as it passes throuth the pulmonary circulation, Congenital arteriovenous malformation are associated with Osler-Weber-Rendu disease in 36-57% of cases. Recently, we experienced a rare case of hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformation.