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자가면역 췌장염의 동반증상 없이 발생한 IgG4 연관 경화성 담관염
천송욱 ( Song Wook Chun ),최자성 ( Ja Sung Choi ),강버들 ( Beo Deul Kang ),김유진 ( Yu Jin Kim ),한기준 ( Ki Jun Han ),조현근 ( Hyeon Geun Cho ),오화은 ( Hwa Eun Oh1 ),조재희 ( Jae Hee Cho ) 대한소화기학회 2013 대한소화기학회지 Vol.62 No.1
IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy. (Korean J Gastroenterol 2013; 62:69-74)