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Retrospective evaluation of the value of additive therapeutic plasma exchange (PEX) compared to standard medical therapy (SMT) in Amanita toxin-associated acute liver failure in children and adolescents within the last 10 years at a international group of liver transplant centers.
Amanita toxin-associated acute liver failure is a life-threatening condition that can often lead to the need for an emergency liver transplantation. The disease may also be fatal, particularly in patients who are not eligible for a liver transplant due to advanced age or corresponding comorbidities.
Therapeutic plasma exchange treatment has been shown to significantly improve patient survival in other cases of acute liver failure and has since become standard treatment for acute liver failure in many, but not all, liver transplant centers. However, no patients with Amanita toxin-associated acute liver failure were included in these cohorts.
The hypothesis of the planned study is that an additive therapeutic plasma exchange treatment (PEX) can improve liver transplantation-free survival in these patients compared to standard medical therapy (SMT) alone. Since the therapy procedure in different transplant centers in differs with regard to the use of therapeutic plasma exchange, we are therefore planning a multicenter retrospective comparison of PEX with SMT with regard to transplant-free survival and other clinical endpoints. For this very small cohort of patients with acute liver failure, which also varies in frequency depending on the season and weather conditions, there will certainly never be a sufficiently powered randomized and controlled study. This analysis may change the standard procedure for patients with Amanita toxin-associated acute liver failure.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Therapeutic Plasma Exchange (PEX) | Patients receiving in addition to Standard Medical Therapy (SMT) at least one treatment with Therapeutic Plasma Exchange (PEX) |
| |
| Standard Medical Therapy (SMT) | Patients receiving only Standard Medical Therapy (SMT) of Amanita Toxin associated acute liver failure, including intensive care support (invasive ventilation, vasopressors, renal replacement therapy), silibinin and n-acetylcystein. Included in this group are also patients receiving albumine dialysis or other extracorporeal liver assist therapies excluding therapeutic plasma exchange. |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Therapeutic Plasma Exchange (PEX) | Device | Therapeutic plasma exchange with treatment sessions >=1 replacing varying fractions of patient´s whole plasma with healthy donor plasma |
| Measure | Description | Time Frame |
|---|---|---|
| Liver Transplant free Survival | Survival and free of liver transplantation until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) | until day 28 from initial diagnosis of acute liver failure |
| Measure | Description | Time Frame |
|---|---|---|
| Overall Survival | Survival until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with International normalized ratio (INR) > 1.5) | until day 28 from initial diagnosis of acute liver failure |
| Liver transplantation |
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Inclusion Criteria:
Exclusion Criteria:
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All patients with Amanita toxin related acute liver failure, both adult and pediatric
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| Name | Affiliation | Role |
|---|---|---|
| Klaus Stahl, MD | Hannover Medical School, Hannover Germany | Principal Investigator |
| Richard Taubert, MD | Hannover Medical School, Hannover Germany | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Hospital Aachen (RWTH) | Aachen | Germany | ||||
| Hannover Medical School |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 31348553 | Result | Stahl K, Hadem J, Schneider A, Manns MP, Wiesner O, Schmidt BMW, Hoeper MM, Busch M, David S. Therapeutic plasma exchange in acute liver failure. J Clin Apher. 2019 Oct;34(5):589-597. doi: 10.1002/jca.21737. Epub 2019 Jul 26. | |
| 37178148 | Result | Stahl K, Bode C, David S. Bridging patients with acute-on-chronic liver failure for transplantation: plasma exchange to stabilize multiorgan failure? Intensive Care Med. 2023 Jul;49(7):890-891. doi: 10.1007/s00134-023-07092-x. Epub 2023 May 13. No abstract available. |
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Plan to share on reasonable request
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| ID | Term |
|---|---|
| D017114 | Liver Failure, Acute |
| D017093 | Liver Failure |
| ID | Term |
|---|---|
| D048550 | Hepatic Insufficiency |
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
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| ID | Term |
|---|---|
| D010951 | Plasma Exchange |
| ID | Term |
|---|---|
| D001803 | Blood Transfusion |
| D001691 | Biological Therapy |
| D013812 | Therapeutics |
| D010956 | Plasmapheresis |
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Liver transplantation until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) |
| until day 28 from initial diagnosis of acute liver failure |
| High urgency (HU) listing for liver transplantation | Initiated high urgency listing for liver transplantation until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) | until day 28 from initial diagnosis of acute liver failure |
| Acute kidney injury (AKI) and max. grade of AKI (I-III) | Acute kidney injury and max. grade of AKI (I-III) until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) | until day 28 from initial diagnosis of acute liver failure |
| Renal Replacement Therapy (RRT) | Initiated renal replacement therapy until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) | until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) |
| Vasopressor therapy | Initiated vasopressor therapy until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) | until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) |
| Invasive ventilation | Initiated invasive ventilation until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) | until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) |
| Maximum grade of hepatic encephalopathy (HE) | Maximum grade of hepatic encephalopathy (HE) until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) | until day 28 from initial diagnosis of acute liver failure (encephalopathy of any grade and coagulopathy with INR > 1.5) |
| Hanover |
| Germany |
| ASST Ospedale Papa Giovanni XXIII | Bergamo | Italy |
| INCMNSZ | Mexico City | Mexico |
| Curry Cabral Hospital | Lisbon | Portugal |
| Hospital Clínic de Barcelona | Barcelona | Spain |
| 32583446 | Result | Stahl K, Busch M, Fuge J, Schneider A, Manns MP, Seeliger B, Schmidt JJ, Wiesner O, Schmidt BMW, Taubert R, Vondran FWR, Hoeper MM, David S. Therapeutic plasma exchange in acute on chronic liver failure. J Clin Apher. 2020 Aug;35(4):316-327. doi: 10.1002/jca.21799. Epub 2020 Jun 24. |
| D001781 |
| Blood Component Removal |
| D016060 | Sorption Detoxification |
| D005112 | Extracorporeal Circulation |
| D013514 | Surgical Procedures, Operative |