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. 2020 Apr 8;37(5):1866–1896. doi: 10.1007/s12325-020-01305-1

Table 2.

Overview of data from published papers of PA studies/case studies

References n Transplant typea Median (range) transplant age (years) Post-transplant metabolic control Post-transplant neurological health Complications Median (range) duration of follow-up Deaths (cause and number)
Arrizza et al. [8] 1 OLT 22 (n/a) No decompensations Reversal of severe cardiomyopathy (n = 1) None reported 10 (n/a) None reported
Barshes et al. [9] 2 OLT 1.7 (1.3–2.0) No decompensations

Improvement in bilateral ganglia lesions (n = 1)

Improved cognition (n = 1)

HAT requiring retransplant (n = 1) 1.4 (0.3–2.4) None reported
Charbit-Henrion et al. [10] 12 LT 3.2 (1.6–6.8) No decompensations Reversal of cardiomyopathy (n = 3)

Repeat transplantation required (n = 4): biliary cirrhosis secondary to HAT (n = 2), primary non-function of the graft (n = 1), hepatic dysfunction after HAT (n = 1)

Moderate renal failure (n = 1)

PTLD (n = 2)

Bipolar disorder (n = 1)

Kidney transplant (n = 1)

Chronic hepatitis (n = 1)

Biliary cirrhosis (n = 2)

Biliary stricture (n = 1 after second transplant)

ARDS (n = 4)

Acute encephalopathy (n = 1)

Biliary sepsis (n = 1)

17 (0–21)

Multi-organ failure (n = 4)

Hepatic failure (n = 3)

Critelli et al. [11] 3 LDLT 8.7 (1.2–11.8) No decompensations Not reported

HAT (n = 1)

CMV infection (n = 1)

Colonic perforation (n = 1)

EBV viremia (n = 2)

2.1 (1.6–2.1) None reported
Kasahara et al. [12] 3 LDLT 2 (0.6–2.2) No decompensations Normal mental development (n = 3)

CMV infection (n = 2)

Intestinal perforation which necessitated relaparotomy (n = 1)

3.3 (n/s) None reported
Kasahara et al. [13] 9 LDLT 2.2 (0.4–12.0) Recurrent metabolic decompensation (n = 9) No episodes of cardiac insufficiency reported Septic complications (n = 4) Not reported Sepsis (n = 4)
Kayler et al. [14] 1 LT 3 (n/a) Not specified Not specified Not specified 0.25 (n/a) Cause not specified (n = 1)
Lam et al. [15] 1 KT 42 (n/a) n/s n/s n/s 3 (n/a) None reported
Moguilevitch and Delphin [16] 1 LT 1 (n/a) Not reported Not reported Increase in liver function tests (n = 1) n/s None reported
Morioka et al. [17] 3 LDLT 2 (1–5) No decompensations Not reported None reported 2.5 (1.8–4.9) None reported
Nagao et al. [18] 1 LDLT 0.6 (n/a) No decompensations Improved neurologic function (n = 1)

Intestinal perforation (n = 1)

CMV infection (n = 1)

18 (n/a) None reported
Quintero et al. [19] 6 LDLT 5.2 (1.3–7.5) No decompensations Stabilisation/ improvement of baseline neurological impairment (n = 6)

HAT (n = 2)

Arterial vasospasm without thrombus during surgery (n = 2)

Delayed biliary anastomosis (n = 2)

1.5 (0.5–4.0) None reported
Rammohan et al. [20] 6

APOLT (n = 5)

OLT (n = 1)

n/s n/s Stabilisation of cardiomyopathy (n = 1)

HAT (n = 1; APOLT)

Graft dysfunction leading to severe metabolic decompensation (n = 1; OLT)

4.2 (n/s) Severe metabolic decompensation (n = 1)
Rela et al. [21] 1 ALT 2 (n/a) Metabolic decompensation (n = 1) Acceptable neurological development (n = 1) None reported 10 (n/a) None reported
Romano et al. [22] 2 OLT N/a No decompensations Reversal of cardiomyopathy (n = 2) Cardiac arrest (recovered) (n = 1) 14.5 (7.0–22.0) None reported
Schlenzig et al. [23] 2 OLT 8.0 (7.0–9.0) No decompensations Neurological sequelae involving basal ganglia (n = 1)

Acute rejection (n = 1)

Chronic rejection (n = 1)

CMV infection (n = 2)

Incomplete HAT (n = 1)

Persistent insulin-dependent diabetes mellitus (n = 1)

1.3 (n/s) Incomplete HAT (n = 1)
Shanmugam et al. [24] 5 APOLT 2.75 (0.7–4.6) No decompensations Progressive improvement in developmental scores (n = 5)

HAT (n = 1)

High ammonia without encephalopathy (n = 1)

Acute cellular rejection (n = 4)

2.7 (1.6–4.2) None reported
Vara et al. [25] 5 LT 1.5 (0.8–7.0) No decompensations Neurological decompensation (n = 1)

HAT requiring retransplantation (n = 1)

Metabolic stroke (n = 1)

7.3 (2.2–15.0) None reported
Yorifuji et al. [26] 3 LDLT 2 (1.2–5.1) Reduced metabolic decompensation

Improvement in neurological health (n = 1)

Data n/a for other patients

Acute metabolic decompensation (n = 1) 1.4 (0.7–3.8) None reported

ALT auxiliary liver transplantation, APOLT auxiliary partial orthotopic liver transplantation, ARDS acute respiratory distress syndrome, CMV cytomegalovirus, EBV Epstein–Barr virus, HAT hepatic artery thrombosis, KT kidney transplantation, LDLT living-donor liver transplantation, LT liver transplantation, OLT orthotopic liver transplantation, PTLD post-transplant lymphoproliferative disease, n/a not available, n/s not specified

aVarious types of liver transplantation used