Table 4.
Overview of data from PA studies/case studies (abstracts only)
| References | n | Transplant typea | Median (range) transplant age (years) | Post-transplant metabolic control | Post-transplant neurological health | Complications | Median (range) duration of follow-up (years) | Deaths (cause and number) |
|---|---|---|---|---|---|---|---|---|
| Alvarez-Elias et al. [7] | 1 | LDL/DDK |
Liver transplant: 2 (n/s) Kidney transplant: 4.9 (n/s) |
Not reported | Not reported | Nephrotic syndrome (8 mo post-liver transplantation) with progression to ESRD necessitating renal transplant (n = 1) | 3 (n/a) | None reported |
| Celik et al. [55] | 64 | LT [segmental (n = 30), whole (n = 34)] | 2.3 (0.25–13) | Not reported | Not reported | Graft loss (n = 17) | ≤ 10 |
Graft loss (n = 12; incl. 3 after retransplantation) Stroke (n = 1) Multiple organ failure/sepsis (n = 2) Unknown causes (n = 1) |
| Celik et al. [56] | 1 | LT | Not specified | No further metabolic crises reported | Not reported | None | 0.6 (0.1–1.1) | None reported |
| Curnock et al. [57] | 14 |
Cadaveric graft LT (n = 13, including 4 auxiliary) Live-related donor LT (n = 1) |
2 (0.8–8.0) | No further metabolic decompensations reported | Developmental progress (n = 11) |
≥ 1 episode of acute cellular rejection (n = 5) Metabolic stroke (n = 2) Cardiomyopathy (n = 3) |
4 (2–22) |
Biliary peritonitis (n = 1) Acute or chronic rejection (n = 1) PTLD (n = 1) |
| Duckworth et al. [58] | 6 | LT | 3.5 ± 2.3b | Not specified | Not reported | Post-LT rejection (n = 2; one mild and one severe) | > 1 | None reported |
| Longo [59] | 3 | OLT | n/s (0.75–13) | No further metabolic crises | Patients had improvements of developmental milestones (no further data) | None specified | n/s (1.6–3.9) | None reported |
| Molera et al. [60] | 4 | LT [whole liver graft (n = 2), LDLT (n = 2)] | 5.2 (2.9–7.5)b | No further metabolic crises reported | Stable or improved neurological status at 1-year post LT (n = 4) | HAT (n = 2) | 2.1 (0.31–3.2) | None reported |
| Nassogne et al. [61] | 1 | LDLT | 12.5 | No further metabolic crises | Not specified | Late-onset cardiac failure (n = 1) | 4.5 (n/a) | None reported |
| Nguyen et al. [62] | 7 | OLT | Not specified | Not specified | Not specified | Not specified | > 3 (n/s) | Cause not specified (n = 1) |
| Ovchinsky et al. [63] | 1 | DDLT | 4 | No further metabolic crises | Not specified | None reported | 0.8 (n/a) | None reported |
| Quintero et al. [64] | 5 | LT [whole liver graft (n = 3), LDLT (n = 2)] | 5.2 (2.9–7.5)b | No further metabolic decompensations | Stable or improved neurological status (n = 5) | HAT (n = 3) | 2.1 (0.31–3.2) | None reported |
| Reddy et al. [65] | 4 | APOLT | n/s (0.75–31) | No further metabolic crises | Not specified |
HAT (n = 1) Recurrence of symptoms secondary to portal steal (n = 1) |
n/s (0–6.3) | None reported |
| Valamparampil et al. [66] | 5 | APOLT (all left auxiliary liver transplants) | 2.7 (n/s) | No metabolic episodes following transplantation | Progressive improvement in development (n = 5) | None reported | 1.8 (n/s) | None reported |
| Vara et al. [67] | 5 | LT [auxiliary LT (n = 1), orthotopic left lateral segment graft (n = 4)] | 1.5 (0.8–7) | No further metabolic decompensations reported |
Moderate learning difficulties (n = 2) Mild learning difficulties (n = 2) Developmental delay with a probable metabolic stroke (resulting in mild hemiplegia and focal seizures) (n = 1) |
Recurrent herpes simplex virus infection (n = 2) EBV-positive PTLD (n = 1) Probable metabolic stroke resulting in mild hemiplegia and focal seizures (n = 1) |
5.8 (1–14) | None reported |
| Vara et al. [68] | 13 | LT [left lateral segment grafts (n = 7), left lateral segment (n = 2), right lobe auxiliary grafts (n = 2), whole graft (n = 1), live related donor (n = 1)] | 2 (1–7) | No metabolic decompensations reported in 9 surviving patients | No evidence of cardiomyopathy in 9 surviving patients |
HAT requiring retransplantation (n = 1) Chronic cholangiopathy requiring retransplantation (n = 1) Pulmonary haemorrhage (n = 1) Biliary sepsis (n = 1) |
4 (0.6–20.5) |
Pulmonary haemorrhage (n = 1; 29 days post LT) Biliary sepsis (n = 1; 42 days post LT) Lymphoproliferative disorder (n = 1; 14 years post LT) |
| Walker et al. [69] | 5 | LT | 1.8 (0.75–7.0) | Not reported | Not reported | Impaired systolic function (n = 1) | 4.25 (0–10.5) | None reported |
APOLT auxiliary partial orthotopic liver transplantation, DDLT deceased-donor liver transplantation, EBV Epstein–Barr virus, ESRD end-stage renal disease, HAT hepatic artery stenosis, KT kidney transplantation, LDL/DDK living-donor liver/deceased-donor kidney, 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
bMean (range) transplant age shown