Hamlyn A N, Gollan J L, Douglas A P, Sherlock S
Br Med J. 1977 Sep 10;2(6088):660-3. doi: 10.1136/bmj.2.6088.660.
Two girls, aged 12 and 17 years, presented with hepatocellular dysfunction and severe haemolysis due to Wilson's disease (hepatolenticular degeneration). This was accompanied by acute renal failure. In the absence of renal function sufficient for the urinary excretion of penicillamine, studies were performed to assess the potential of peritoneal dialysis, ascites removal by ultrafiltration-reinfusion, and haemodialysis as alternative excretory pathways for copper. The greatest amount of copper, as judged by rising bath concentrations, seemed to be eliminated with haemodialysis. But this was accompanied by a progressive increase in serum copper concentrations with rapid clinical and biochemical deterioration leading to death within 48 hours. A small amount of copper was lost with ascites removal. Significant amounts of copper were removed during peritoneal dialysis (36 mumol/day (2287 microgram/day)), although a clinical response was not evident before haemodialysis was introduced. The administration of penicillamine orally, intravenously, or intraperitoneally produced no measurable increase in copper excretion into the peritoneal dialysate. Hence peritoneal dialysis alone appears to offer the greatest potential benefit with regard to both eliminating copper and altering the course of this fulminant form of Wilson's disease.
两名分别为12岁和17岁的女孩，因威尔逊病（肝豆状核变性）出现肝细胞功能障碍和严重溶血，并伴有急性肾衰竭。由于肾功能不足以使青霉胺经尿液排出，因此开展了相关研究，以评估腹膜透析、超滤-回输腹水以及血液透析作为铜的替代排泄途径的潜力。根据透析液中铜浓度升高判断，血液透析似乎能清除最多的铜。但这伴随着血清铜浓度的逐渐升高以及临床和生化指标的迅速恶化，导致患者在48小时内死亡。超滤-回输腹水可排出少量铜。腹膜透析期间可清除大量铜（36微摩尔/天（2287微克/天）），不过在开始血液透析之前，未观察到明显的临床反应。口服、静脉注射或腹腔内注射青霉胺均未使进入腹膜透析液的铜排泄量出现可测量的增加。因此，就清除铜和改变这种暴发性威尔逊病的病程而言，单独进行腹膜透析似乎具有最大的潜在益处。
