Fulminant Wilson's disease with haemolysis and renal failure: copper studies and assessment of dialysis regimens.

Hamlyn, A N; Gollan, John L.; Douglas, A.G.; Sherlock, Sheila

doi:10.1136/bmj.2.6088.660

Cited by 64 publications

(15 citation statements)

References 9 publications

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“…Those methods have included hemofiltration or dialysis, albumin dialysis, and the Molecular Adsorbents Recirculating System (MARS). Hamlyn et al in 1997 studied peritoneal dialysis and hemodialysis in fulminant Wilson disease and found in one case that significant amounts of copper were removed with peritoneal dialysis, but there was no significant clinical improvement [11]. Postdilutional hemofiltration is another method that has been to shown to allow rapid removal of copper in the presence of renal failure until OLT could be performed [12].…”

Section: Discussionmentioning

confidence: 97%

Therapeutic plasmapheresis as a bridge to liver transplantation in fulminant Wilson disease

Jhang

Schilsky

Lefkowitch

et al. 2007

J of Clinical Apheresis

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Wilson disease is an autosomal recessive disorder of copper metabolism that leads to the accumulation of copper mainly in the liver, cornea, brain, and kidney. Rarely, Wilson disease can present as fulminant hepatic failure with direct antiglobulin test-negative hemolytic anemia and renal failure. In the absence of liver transplantation, this disease is uniformly fatal because medical therapy is ineffective. This report describes the successful use of plasmapheresis for a patient with fulminant Wilson disease as a bridge to transplantation. Five daily therapeutic plasmapheresis procedures using fresh frozen plasma as a replacement fluid were performed over 6 days. Serum copper, urinary copper excretion, and hemolysis were significantly reduced and renal function improved. The patient's clinical status improved and she remained clinically stable until a liver transplant was possible. Plasmapheresis can be a successful medical treatment in fulminant Wilson disease and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.

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Section: Discussionmentioning

confidence: 97%

Therapeutic plasmapheresis as a bridge to liver transplantation in fulminant Wilson disease

Jhang

Schilsky

Lefkowitch

et al. 2007

J of Clinical Apheresis

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“…[14] Red blood cell destruction results in a direct hemolytic anemia, which may progress to acute renal failure if the patient is left untreated. [15,16] Some patients with FWD presented with hemolytic anemia and renal insufficiency. The two groups experienced severe hemolysis, which suggested that their prognosis was irrelevant to hemolysis.…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and management of fulminant Wilson’s disease: a single center’s experience

et al. 2015

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For patients with FWD, the episode of severe hepatic encephalopathy or/and spontaneous bacterial peritonitis indicates worse prognosis, and PTA is a recommendable predictor. An emergent liver transplantation should be considered for patients whose PTA is below 20%, or for those with severe HE or/and SBP. The comprehensive therapy of corticosteroid, copper-chelating agent and TPE is effective for patients without SBP and whose PTA is higher than 20%.

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“…The acute increase in the copper load is believed to be a pathogenic factor, inducing severe Coomb's negative hemolytic anemia 9 and renal failure. 10 We hypothesized that reduction of the copper load by MARS may confer benefit in the management of acute decompensated Wilson disease. This hypothesis is supported by other investigators who have used extracorporeal treatments to remove excessive copper in acute decompensated Wilson disease.…”

Section: Discussionmentioning

confidence: 99%

Use of the molecular adsorbents recirculating system as a treatment for acute decompensated wilson disease

2008

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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.

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Fulminant Wilson's disease with haemolysis and renal failure: copper studies and assessment of dialysis regimens.

Cited by 64 publications

References 9 publications

Therapeutic plasmapheresis as a bridge to liver transplantation in fulminant Wilson disease

Therapeutic plasmapheresis as a bridge to liver transplantation in fulminant Wilson disease

Diagnosis and management of fulminant Wilson’s disease: a single center’s experience

Use of the molecular adsorbents recirculating system as a treatment for acute decompensated wilson disease

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