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

Li, Liang; Zhang, Xiaole; Xu, Wen; Guo, Qun; Zhou, Jianhua

doi:10.1159/000493909

Cited by 10 publications

(4 citation statements)

References 27 publications

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“…Although plasmapheresis as a bridge therapy to liver transplant has been widely described in literature, only few case reports have reported survival with only plasmapheresis without requiring liver transplant. [8][9][10][11][12]…”

Section: Discussionmentioning

confidence: 99%

Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis

Mirza

Ravi

Malhotra

et al. 2021

Journal of Child Science

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Wilson disease (WD) is a disorder of copper metabolism resulting in accumulation of copper in vital organs of the human body, predominantly in the liver and the brain. Acute liver failure in WD has a bad prognosis, especially with a score ≥11 in the revised WD prognostic index; emergency liver transplantation is considered the only life-saving option in this scenario. Here, we reported a girl patient with WD-induced liver failure and poor prognostic score who was rescued by plasmapheresis. She also manifested severe Coombs negative hemolytic anemia and acute kidney injury. This case report highlights the utility of an adjunctive modality besides liver transplantation for the management of fulminant liver failure caused by WD.

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Section: Discussionmentioning

confidence: 99%

Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis

Mirza

Ravi

Malhotra

et al. 2021

Journal of Child Science

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“…The available literature reports that CPFA can effectively remove bilirubin, tryptophan, phenols[ 78 , 79 ], bile acids[ 79 ], and cytokines[ 80 ]. It has been used for liver indications such as ALF and ACLF[ 81 , 82 ], after LT[ 83 ], in hypoxic hepatitis[ 84 ], and combined with PE therapy for ALF due to Wilson’s disease[ 85 ]. Future studies are required to elucidate its role as an LRT.…”

Section: Compination Modalitiesmentioning

confidence: 99%

Liver replacement therapy with extracorporeal blood purification techniques current knowledge and future directions

Papamichalis¹,

Oikonomou²,

Valsamaki³

et al. 2023

World J Clin Cases

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Clinically, it is highly challenging to promote recovery in patients with acute liver failure (ALF) and acute-on-chronic liver failure (ACLF). Despite recent advances in understanding the underlying mechanisms of ALF and ACLF, standard medical therapy remains the primary therapeutic approach. Liver transplantation (LT) is considered the last option, and in several cases, it is the only intervention that can be lifesaving. Unfortunately, this intervention is limited by organ donation shortage or exclusion criteria such that not all patients in need can receive a transplant. Another option is to restore impaired liver function with artificial extracorporeal blood purification systems. The first such systems were developed at the end of the 20 th century, providing solutions as bridging therapy, either for liver recovery or LT. They enhance the elimination of metabolites and substances that accumulate due to compromised liver function. In addition, they aid in clearance of molecules released during acute liver decompensation, which can initiate an excessive inflammatory response in these patients causing hepatic encephalopathy, multiple-organ failure, and other complications of liver failure. As compared to renal replacement therapies, we have been unsuccessful in using artificial extracorporeal blood purification systems to completely replace liver function despite the outstanding technological evolution of these systems. Extracting middle to high-molecular-weight and hydrophobic/protein-bound molecules remains extremely challenging. The majority of the currently available systems include a combination of methods that cleanse different ranges and types of molecules and toxins. Furthermore, conventional methods such as plasma exchange are being re-evaluated, and novel adsorption filters are increasingly being used for liver indications. These strategies are very promising for the treatment of liver failure. Nevertheless, the best method, system, or device has not been developed yet, and its probability of getting developed in the near future is also low. Furthermore, little is known about the effects of liver support systems on the overall and transplant-free survival of these patients, and further investigation using randomized controlled trials and meta-analyses is needed. This review presents the most popular extracorporeal blood purification techniques for liver replacement therapy. It focuses on general principles of their function, and on evidence regarding their effectiveness in detoxification and in supporting patients with ALF and ACLF. In addition, we have outlined the basic advantages and disadvantages of each system.

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“…Fresh frozen plasma is readily available in most hospitals. Adverse events, such as hypocalcemia are easily treated (3,10,11).…”

Section: What Is Newmentioning

confidence: 99%

Fulminant Wilson Disease in Children

Proost¹,

Cassiman²,

Levtchenko³

et al. 2020

J. pediatr. gastroenterol. nutr.

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Objectives -Since 2005 a New Wilson Index (NWI) ≥ 11 is used as a predictor of death without transplantation in fulminant Wilson's disease (WD). Plasma exchange is advocated as a new treatment modality.Methods -We present a patient with fulminant WD treated with plasma exchange. All published cases applying plasma exchange for fulminant WD were reviewed systematically.Results -A 14-year-old girl presented with hemolysis and fulminant liver failure. She had no encephalopathy; NWI was 14. As a bridge to transplantation plasma exchange was started immediately. Complete remission was achieved with plasma exchange and later chelation therapy with D-penicillamine. She is now at 3-year transplant free survival. Literature review identified 37 patients presenting with fulminant WD and NWI ≥ 11 who were treated with plasma exchange. Seventeen of these patients (i.e. 46%) recovered without transplantation.Conclusions -Multiple case reports and case series demonstrate transplant free survival after plasma exchange and subsequent chelation therapy, despite a NWI ≥ 11. Plasma exchange affects the clinical course and is a therapeutic option in children and young adults presenting with fulminant WD.

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

Cited by 10 publications

References 27 publications

Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis

Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis

Liver replacement therapy with extracorporeal blood purification techniques current knowledge and future directions

Fulminant Wilson Disease in Children

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