Use of the molecular adsorbents recirculating system as a treatment for acute decompensated Wilson disease.
Acute decompensated Wilson disease presenting as fulminant liver failure is a life-threatening condition for which liver transplantation is the ultimate treatment. It is listed as a status 1 indication according to the United Network for Organ Sharing classification. A massive amount of copper released during the attack induces hemolytic anemia and acute renal failure. Conventional chelating therapy attempting to remove copper from the patient is not satisfactory because there is inadequate time for these drugs to take action and patients are usually oliguric. The Molecular Adsorbents Recirculating System (MARS) is a form of modified dialysis that removes putative albumin-bound toxins associated with liver failure. It is believed that extracorporeal albumin dialysate absorbs the circulating copper molecules that are trapped in the patient's circulation. We report 2 patients with acute decompensated Wilson disease treated with MARS. In the first case, the patient was started on MARS once conventional treatment failed. A significant amount of copper was removed from her circulatory system, and her condition stabilized afterwards. The treatment gained her extra time, and she was eventually bridged to liver transplantation. In the second case, the patient was started on MARS treatment early in the course of his illness, and his condition soon stabilized after the treatment. He was able to return to his home country for liver transplantation. In both cases, MARS was used as a means of preventing deterioration rather than salvaging devastation. In conclusion, MARS may confer benefits to patients with acute decompensated Wilson disease if it is started early in the course of illness.
Chiu
Alexander
A
Intensive Care Unit, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China.
Tsoi
Nai Shun
NS
Fan
Sheung Tat
ST
D000293:Adolescent; D000327:Adsorption; D003300:Copper / Q000302:isolation & purification; D005260:Female; D017583:Hemodiafiltration / Q000295:instrumentation; D006527:Hepatolenticular Degeneration / Q000628:therapy*; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D055815:Young Adult
D003300:Copper