Powell-Jackson P R, Ede R J, Williams R
Gut. 1986 Sep;27(9):1101-5. doi: 10.1136/gut.27.9.1101.
Two cases of the Budd-Chiari syndrome are described in whom the diagnosis was finally confirmed at necropsy. The presentation was with encephalopathy, occurring within eight weeks of first symptoms and coming therefore within the definition of fulminant hepatic failure. In one, thought to have non-A, non-B hepatitis, encephalopathy progressed to grade 4 coma with death 12 days after presentation. In the other, mistakenly thought to have intra-abdominal malignancy, an exploratory laparotomy exacerbated the encephalopathy with death three weeks later. In neither case were non-invasive investigations, such as ultrasound and isotope scanning, carried out which might have facilitated an earlier diagnosis and consideration for orthotopic liver transplantation, probably the most appropriate form of therapy for these very severe cases.
本文描述了两例布加综合征病例，最终尸检确诊。临床表现为脑病，在首发症状出现后八周内发生，因此符合暴发性肝衰竭的定义。其中一例被认为患有非甲非乙型肝炎，脑病进展为4级昏迷，发病后12天死亡。另一例被误诊为腹内恶性肿瘤，剖腹探查加重了脑病，三周后死亡。两例均未进行超声和同位素扫描等非侵入性检查，而这些检查可能有助于早期诊断并考虑原位肝移植，这可能是这些重症病例最合适的治疗方式。
