Background
What are extracorporeal liver support systems?
What is plasma exchange?
Setting | Access device | Removed from patient | Replacement fluid | Duration | Anticoagulation |
---|---|---|---|---|---|
Paediatric intensive care unit (PICU) | Double-lumen, central venous catheter. Rarely, large peripheral veins are used | Commonly 1–1.5 × plasma volume, whereby plasma volume = ((70–80 mL × (weight (kg))) × (1 – haematocrit)). High volume is often defined as exchange of 10–15% of ideal body weight, or > 1.5–2 × plasma volume | 4.5% or 5% human albumin solution (HAS), or fresh frozen plasma (FFP), or a combination (higher fractions of FFP are given in ALF) | 1.0 plasma volume in 1–2 h; 2.0 plasma volume in 4 h. Duration may vary depending on the patient, device, and complications | Unfractionated heparin (10–20 units per kg per hour) or prostacyclin (4–8 ng per kg per minute). In a bleeding child with ALF, the circuit can be run without any anticoagulation |
Complications
Mechanism of action in ALF
High-volume plasma exchange
Practicalities and controversies
Timing of TPE
Indications for TPE in paediatrics
Evidence for plasma exchange in paediatrics
Citation | Time-frame | Country | Study design | Population | Details of TPE modality | Mortality and liver survival | Other key outcomes |
---|---|---|---|---|---|---|---|
Chien et al. 2019 [44] | 2003–2016 | Taiwan | Retrospective observational cohort study | n = 23 (total ALF), n = 18 (received TPE) | Exchange volume of 2–4 times estimated plasma volume; daily for three days then variable according to clinical condition | 11/23 (48%) had native liver survival 9/23 (39%) died without transplant 1/23 (4%) died post-transplant 2/23 (9%) survived post-transplant | The NLS group had fewer sessions of TPE than the non-NLS group (3 vs. 9, p < 0.01) |
Demirkol et al. 2010 [42] | 2005–2009 | Turkey | Retrospective observational case series | n = 14 (ALF) | Not described in detail (abstract). Fourteen patients underwent a total of 37 TPE sessions | 4/14 (29%) had native liver survival 5/14 died (36%) 5/14 (36%) had liver transplants | Biochemical variables improved when comparing pre-TPE and post-TPE values; no patients experienced serious adverse events |
Jørgensen et al. 2021 [27] | 2012–2019 | Denmark | Retrospective observational cohort study | n = 16 (ALF) | Fluid volume corresponding to 10% of body weight was exchanged with FFP; sessions for 3 consecutive days followed by re-assessment | 8/16 (50%) had native liver survival 5/16 (31%) died without transplant 1/16 (6%) died post-transplant 2/16 (13%) survived post-transplant | There were no bleeding-related complications, and no electrolyte or acid–base disturbances other than three children who developed alkalosis. Bilirubin, ALT and INR significantly declined with HVPE treatment |
Pawaria et al. 2021 [26] | 2014–2019 | India | Prospective nonrandomised interventional study | n = 37 (ALF due to Wilson’s) in total; n = 19 received HVPE, n = 18 received standard treatment | Exchange in one session of > 1.5 × plasma volume; exchange for three consecutive days (maximum 3–6 sessions) | 4/19 (21%) of HVPE group and 5/18 (28%) from standard treatment group were transplanted 9/19 (47%) of HVPE group and 3/18 (17%) of standard treatment group had transplant-free survival | 47.3% of the HVPE group had transplant free survival compared to 16.7% of the standard medical treatment group (OR 2.84, 95% CI 0.91–8.8, p = 0.049) |
Pham et al. 2016 [41] | 2000–2014 | USA | Retrospective observational case series | n = 10 (ALF due to Wilson’s) | Plasma exchange of 1–1.25 plasma volume for 42 of 43 exchanges; each patient had a median of 3.5 procedures (range 1–9) | 1/10 (10%) had native liver survival 9/10 (90%) survived post-transplant (follow-up of 6 months) | Of 43 TPE procedures, 70% required calcium supplementation, and 10% reported adverse events |
Singer et al. 2001 [43] | 1987–2000 | USA | Retrospective observational case series | n = 49 (ALF or ACLF) | Plasma volume of 2.2 + / − 0.6 removed, replaced with 74 + / − 11% FFP; daily until recovered, died or transplanted | 3/49 (6%) had native liver survival 14/49 (28%) died without transplant 15/49 (31%) died post-transplant 17/49 (35%) survived post-transplant | No change in neurological examination results. Significant improvement in coagulation, total bilirubin, and transaminases post-TPE. No major complications |
Hybrid/combination approaches | |||||||
Akcan Arikan et al. 2018 [45] | NS (24 months) | USA | Retrospective observational cohort study | n = 15 (ALF or ACLF) | Continuous veno-venous haemodiafiltration, centrifugal plasma exchange with FFP at 1.3–1.5 × plasma volume, and MARS | 2/15 (13%) had native liver survival 4/15 (27%) died without transplant 9/15 (60%) survived post-transplant | 13/15 (87%) of patients had improved hepatic encephalopathy grade, including all survivors |
Ide et al. 2015 [46] | 2006–2011 | Japan | Retrospective observational cohort study | n = 17 (ALF) | Continuous veno-venous haemodiafiltration, plasma exchange and liver transplantation. Plasma exchange used 100 mL/kg of FFP once daily until coagulopathy recovered | 15/17 (88%) survived post-transplant 2/17 (12%) died post-transplant | 11/15 survivors (73%) had no neurological morbidities |
Rodriguez et al. 2017 [31] | NS (30 months) | USA | Retrospective observational cohort study | n = 51 (ALF or ACLF, total); n = 20 received plasma exchange | Continuous veno-venous haemodiafiltration; 20 patients also received 5.8 + / − 3.8 plasma exchange sessions, with FFP placement. Plasma exchange was centrifugal and 1–1.5 × plasma volume | Of whole cohort, 29/51 (57%) died in hospital 26/51 (51%) received liver transplants | Patients receiving plasma exchange were more likely than non-recipients to have citrate accumulation than non-plasma exchange patients (p = 0.004) |
Schaefer et al. 2011 [47] | 2002–2010 | Germany | Retrospective observational cohort study | n = 10 (ALF or ACLF) | MARS (standard for n = 7, Mini for n = 3). This was alternated with combined plasma exchange and haemodialysis (for n = 8). Plasma exchange was 1.5 × plasma volume | 5/10 (50%) died 3/10 (30%) others were successfully transplanted 2/10 (20%) had native liver survival | Patients showed significantly greater reductions in bilirubin, ammonia and INR on PE/HD than on MARS/mini-MARS (p < 0.05) |
Tufan Pekkucuksen et al. 2020 [48] | 2013–2016 | USA | Retrospective observational cohort study | n = 63 (n = 20, 32% TPE for ALF or ACLF) | Tandem continuous veno-venous haemodiafiltration and plasma exchange. For plasma exchange; mean exchange volume 1.34 + / − 0.21. FFP used for liver failure patients | 25/63 (39.7%) died, transplant outcome not stated | Non-survivors had significantly greater time to initiation of TPE from PICU admission, than survivors (p = 0.029) |