Bridging Use of Plasma Exchange and Continuous Hemodiafiltration before Living Donor Liver Transplantation in Fulminant Wilson's Disease

Nagata, Yoshiko; Uto, Hirofumi; Hasuike, Satoru; Ido, Akio; Hayashi, Katsuhiro; Eto, Toshiharu; Hamakawa, Toshiro; Tanaka, Kōichi; Tsubouchi, Hirohito

doi:10.2169/internalmedicine.42.967

Cited by 41 publications

(25 citation statements)

References 15 publications

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“…Although plasmapheresis is accepted as a Category I indication in the American Society for Apheresis (ASFA) Special Issue 2013 for WD, this recommendation was based on low-quality evidence (Grade 1C) [16]. So far, there are only a few case reports describing the effect of plasmapheresis in children with Wilsonian crisis [15,[18][19][20][21][22]. Theoretically, in critical WD patients, plasmapheresis is an effective treatment for the following reasons.…”

Section: Discussionmentioning

confidence: 99%

Plasmapheresis Combined with Continuous Plasma Filtration Adsorption Rescues Severe Acute Liver Failure in Wilson’s Disease before Liver Transplantation

Zhang

et al. 2018

Blood Purif

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Wilsonian crisis (hemolytic crisis and acute liver failure [ALF] in Wilson’s disease) is fatal and almost all patients ultimately need a timely liver transplantation to save their lives. How to safely pass the critical period to liver transplantation is still a big challenge to doctors especially to pediatricians facing more difficult situations in blood purification. Here, we report about a 7-year-old child (weight 21 kg) presenting with severe hemolysis and impending ALF that made a rapid recovery with prompt initiation of plasmapheresis combined with continuous plasma filtration adsorption (CPFA) and chelation therapy. Rapidly efficient removal of copper, bilirubin, and albumin-binding toxins by hybrid blood purification alleviated hemolysis and liver injury and successfully bridged the patient over the critical period to late liver transplantation. Moreover, a review of the literature was performed examining the use of plasmapheresis, molecular adsorbent recirculation system, single-pass albumin dialysis, and continuous veno-venous hemodiafiltration in Wilson disease. The experience of our case points to that plasmapheresis combined with CPFA treatment can improve clinical symptoms and bridge children over through Wilsonian crisis to late live transplantation, and CPFA treatment is feasible and safe in children weighing more than 20 kg.

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Section: Discussionmentioning

confidence: 99%

Plasmapheresis Combined with Continuous Plasma Filtration Adsorption Rescues Severe Acute Liver Failure in Wilson’s Disease before Liver Transplantation

Zhang

et al. 2018

Blood Purif

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“…Whereas MARS or the equivalent technology requires that specialized devices be available, the widespread availability of plasmapheresis at most transplant and non-transplant centers alike would permit the rapid initiation of this treatment almost anywhere. Nagata et al have shown that the combination of hemodialysis and plasma exchange may be a good choice to reduce copper load in the setting of renal failure [23].…”

Section: Discussionmentioning

confidence: 99%

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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“…The specific prognostic index revised Wilson prognostic index represents a valid tool for assessing these critically ill patients [121,122] . As bridging therapies to liver transplantation, or as an alternative altogether in regions were liver transplantation is not possible, strategies such as rapid plasma exchange [124,125] via any method such as plasmapheresis [126] , hemofiltration [127,128] , albumin dyalisis [129] , or exchange transfusion, may be successfully used to lower circulating copper levels, renal protection from coppermediated tubular damage, and reduce hemolysis [130] . Albeit some degree of improvement has been reported, the need for liver transplantation has not been obviated in numerous cases, although successful treatment without transplantation has been reported [131] .…”

Section: Fulminant Hepatic Failurementioning

confidence: 99%

Wilson’s disease: A review of what we have learned

Rodríguez–Castro

Hevia‐Urrutia

Sturniolo

2015

WJH

140

123

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Wilson's disease (WD), which results from the defective ATP7B protein product, is characterized by impaired copper metabolism and its clinical consequences vary from an asymptomatic state to fulminant hepatic failure, chronic liver disease with or without cirrhosis, neurological, and psychiatric manifestations. A high grade of suspicion is warranted to not miss cases of WD, especially less florid cases with only mild elevation of transaminases, or isolated neuropsychiatric involvement. Screening in first and second relatives of index cases is mandatory, and treatment must commence upon establishment of diagnosis. Treatment strategies include chelators such as D-penicillamine and trientine, while zinc salts act as inductors of methallothioneins, which favor a negative copper balance and a reduction of free plasmatic copper. As an orphan disease, research is lacking in this field, especially regarding therapeutic strategies which are associated with better patient compliance and which could eventually also reverse established injury.

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Bridging Use of Plasma Exchange and Continuous Hemodiafiltration before Living Donor Liver Transplantation in Fulminant Wilson's Disease

Cited by 41 publications

References 15 publications

Plasmapheresis Combined with Continuous Plasma Filtration Adsorption Rescues Severe Acute Liver Failure in Wilson’s Disease before Liver Transplantation

Plasmapheresis Combined with Continuous Plasma Filtration Adsorption Rescues Severe Acute Liver Failure in Wilson’s Disease before Liver Transplantation

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

Wilson’s disease: A review of what we have learned

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