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Use of the molecular adsorbents recirculating system as a treatment for acute decompensated wilson d |
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Use of the molecular adsorbents recirculating system as a treatment for acute decompensated wilson disease.
Liver Transpl. 2008 Sep 29;14(10):1512-1516Chiu A, Tsoi NS, Fan ST.
Intensive Care Unit, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China.
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. Liver Transpl
14:1512-1516, 2008. (c) 2008 AASLD.
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