British physician G. Budd (1808-1882) and Austrian immunologist H. Chiari (1851-1916) reported this condition separately (1846, 1899) as obstruction of the hepatic vein due to thrombophlebitis, but it is now understood to be obstruction of the inferior vena cava and hepatic vein just below the diaphragm. Causes include thrombophlebitis and congenital abnormalities. In acute cases, the patient begins with severe symptoms such as vomiting and abdominal pain, and dies within one month. In chronic cases, the main symptoms are the development of bypass blood circulation due to obstruction of the inferior vena cava, edema of the lower body, and portal hypertension due to obstruction of the hepatic vein. When the stenosis of the inferior vena cava is due to membranous tissue, a dehiscence surgery developed in Japan is effective, but when the stenosis is extensive, surgical treatment becomes difficult. Source: Encyclopaedia Britannica Concise Encyclopedia About Encyclopaedia Britannica Concise Encyclopedia Information |
イギリスの医師 G.バッド (1808~82) とオーストリアの免疫学者 H.キアリ (51~1916) が血栓性静脈炎による肝静脈の閉塞症として別々に (1846,99) 報告したものであるが,現在では横隔膜直下の下大静脈と肝静脈の閉塞症と理解されている。原因としては,血栓性静脈炎のほかに先天異常があげられる。急性の場合は嘔吐,腹痛などの激しい症状で始って1ヵ月以内に死亡する。慢性の場合のおもな症状には,下大静脈の閉塞のためバイパスの血行路の発達,下半身の浮腫,肝静脈の閉塞による門脈圧亢進がある。下大静脈の狭窄が膜性組織による場合は日本で開発された裂開手術が有効であるが,狭窄部が広範な場合,外科的治療は困難になる。
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