Schramek A, Better O S, Brook J G, Alroy G G, Gellei B
Ann Surg. 1974 Sep;180(3):368-72. doi: 10.1097/00000658-197409000-00019.
Some observations on the natural history, surgical management and metabolic phenomena in B.C.S. are presented. In one patient side to side portocaval shunt brought dramatic improvement with asymptomatic followup of 5 years. The patient died of causes unrelated to B.C.S. At postmortem the shunt was patent and normal liver architecture was preserved. The second patient presented with a fulminant course culminating in acute hepatorenal syndrome. There was marked hypofibrinogenemia and extreme elevation of SGOT. Emergency portocaval shunt was followed by marked improvement in liver status. The patient died of respiratory complications on the 8th postoperative day. The third patient is one year on conservative treatment only and well. In this patient excessive renal tubular reabsorption of sodium at a distal site was documented. This may explain the generalised edema formation in patients with B.C.S. even without inferior vena cava obstruction. This is apparently the first reported instance of renal tubular function studied in B.C.S.
本文介绍了布加综合征（B.C.S.）的自然病史、手术治疗及代谢现象。一名患者接受了侧侧门腔分流术，术后5年无症状随访，病情显著改善。该患者死于与布加综合征无关的原因。尸检时分流管通畅，肝脏结构正常。第二名患者病情急骤，最终发展为急性肝肾综合征。患者出现明显的纤维蛋白原血症及谷草转氨酶（SGOT）极度升高。急诊行门腔分流术后，肝脏状况明显改善。该患者术后第8天死于呼吸并发症。第三名患者仅接受了一年的保守治疗，目前情况良好。该患者记录到远端肾小管对钠的重吸收过多。这可能解释了布加综合征患者即使没有下腔静脉阻塞也会出现全身性水肿的原因。这显然是首次报道对布加综合征患者肾小管功能进行研究的实例。
