Rakela J, Kurtz S B, McCarthy J T, Ludwig J, Ascher N L, Bloomer J R, Claus P L
Gastroenterology. 1986 Jun;90(6):2004-7. doi: 10.1016/0016-5085(86)90274-x.
A 22-yr-old woman presented with fulminant Wilson's disease. The diagnosis was suspected clinically and was later confirmed with chemical and pathologic studies. She presented with acute hepatic failure, hemolysis, and acute anuric renal failure. Postdilution hemofiltration and continuous arteriovenous hemofiltration with oral D-penicillamine allowed removal of a total of 95,700 micrograms of copper; 78,665 micrograms of the total were removed via postdilution hemofiltration alone. On the 57th day, the patient received successful liver and renal transplants. We found that the determination of serum copper was instrumental in the diagnosis of fulminant Wilson's disease, that postdilution hemofiltration allowed a rapid removal of copper in the presence of renal failure, and that, finally, orthotopic liver transplantation should be performed early in the clinical course of these patients. This patient is the longest survivor of this serious condition.
一名22岁女性患暴发性威尔逊病。临床怀疑诊断并随后经化学和病理研究确诊。她表现为急性肝衰竭、溶血和急性无尿性肾衰竭。采用后稀释血液滤过及持续动静脉血液滤过联合口服D-青霉胺，共清除铜95700微克；其中仅后稀释血液滤过就清除了78665微克。在第57天，患者接受了成功的肝和肾移植。我们发现血清铜测定有助于暴发性威尔逊病的诊断，后稀释血液滤过在肾衰竭情况下能快速清除铜，最后，原位肝移植应在这些患者临床病程早期进行。该患者是这种严重疾病最长的幸存者。
