Table 3.
Overview of data from published papers of MMA 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) |
|---|---|---|---|---|---|---|---|---|
| Brassier et al. [27] | 4 | KT | 7.9 (5–10.2) | No decompensations | Neurological stability (n = 2) |
Hepatoblastoma followed by neurological complications (n = 1) Acute rejection responsive to prednisone (n = 1) Movement disorder (n = 1) |
2.8 (1.8–4.6) | Hepatoblastoma followed by neurological complications (n = 1) |
| Chen et al. [28] | 4 | LT | 1.4 (0.7–2.1) | 0.08 per year | Continued development (n = 4) | None reported | 3.5 (0.2–7.7) | None reported |
| Clothier et al. [29] | 1 | KT | 12 (n/a) | No decompensations | Not reported | Mild focal interstitial fibrosis (n = 1) | 6 (n/a) | None reported |
| Critelli et al. [11] | 6 |
OLT (n = 1) Combined LT/KT (n = 5) |
8.4 (1.9 –21.6) | No decompensations | Not reported |
Near-complete stenosis of the right hepatic vein at its junction with the inferior vena cava (n = 1) HAT (n = 1) Renal rejection (n = 1) Biliary anastomotic stricture (n = 1) EBV viremia (n = 1) Mild tubulointerstitial injury (n = 1) |
3.4 (1–11.6) | None reported |
| Duclaux-Loras et al. [30] | 1 | Combined LT/KT | 10.4 (n/a) | No decompensations | Not reported | Renal arterial thrombosis (n = 1) | 10 (n/a) | None reported |
| Hirotsu et al. [31] | 1 | LDLT | 1.8 (n/a) | No decompensations | Not reported | None reported | 0.2 (n/a) | None reported |
| Kasahara et al. [13] | 20 | LDLT | 2.2 (0.4–12) | Recurrent metabolic decompensation (n = 20) | Not reported |
Progressive renal insufficiency (n = 4) New onset of seizures (n = 3) |
Not specified | None reported |
| Kayler et al. [14] | 2 |
LT (n = 1) Combined LT/KT (n = 1) |
14.5 (13–16) | Not specified | Not specified |
Liver retransplantation (n = 1) EBV viremia (n = 1) Potential PTLD (n = 1) |
2.5 (1.1–3.9) | None reported |
| Khanna et al. [32] | 1 | Cadaveric LT | 28 (n/a) | No decompensations | Not reported | None reported | > 1 (n/a) | None reported |
| Lubrano et al. [33, 34] | 1 | KT | 17 (n/a) | No decompensations | Not reported | Chronic allograft nephropathy (n = 1) | 10 (n/a) | None reported |
| McGuire et al. [35] | 1 | LT/KT | 5 (n/a) | One metabolic decompensation at 10 months post transplantation | Neurological deterioration (hemiplegia, truncal ataxia and speech dyspraxia) (n = 1) | Cerebellar stroke (n = 1) | ≥ 10 months (n/a) | None reported |
| Morioka et al. [17] | 2 | LDLT | 6.5 (1–12) | Metabolic stroke (n = 1) | Not reported |
Metabolic stroke (n = 1) Aspergillosis (n = 1) |
0.1 (0.04–0.17) |
Metabolic stroke (n = 1) Aspergillosis (n = 1) |
| Morioka et al. [36] | 7 | LDLT | 4.3 (0.6–7.5) | Metabolic acidosis (n = 2) | Cognitive deficit improved (n = 7) |
Sepsis (n = 1) Graft dysfunction (n = 1) CMV viremia (n = 2) EBV viremia (n = 1) |
0.9 (0.3–1.75) | Sepsis (n = 1) |
| Nagarajan et al. [37] | 2 | LT/KT | 15.5 (10–21) | No decompensations |
Mental status changes (n = 1) Tremors (n = 1) |
Mild reversible acute rejection of the liver (n = 1) CMV gastritis (n = 1) Mental status changes (n = 1) Tremors (n = 1) Mild glucose intolerance (n = 1) |
3.3 (1.5–5) | None reported |
| Niemi et al. [38] | 14 |
LT (n = 6) Combined LT/KT (n = 8) |
7.4 (0.8–20.7) | No decompensations | All patients maintained or improved neurological health |
HAT requiring liver retransplantation (n = 1) Bleeding requiring re-exploration (n = 2) Drainage of subphrenic abscess (n = 1), Seizure (n = 1) Diabetes mellitus (n = 1) |
3.25 (0.25–14)b | None reported |
| Nyhan et al. [39] | 1 | OLT | 22 (n/a) | No decompensations | Neurologic manifestations (n = 1) |
Progression of pre-surgery renal failure (n = 1) Neurologic manifestations (n = 1) |
2 (n/a) | None reported |
| Sakamoto et al. [40] | 13 | LDLT | 9 (0.7–7) | Several metabolic episodes (n = 3) | Normal growth reported |
Severe adhesive intestinal obstruction (n = 1) Strangulation ileus (n = 1) Bile duct stenosis (n = 2) Acute renal failure (n = 1) Convulsion (n = 2) Portal vein stenosis (n = 1) Sepsis (n = 1) Cholangitis (n = 2) |
8.1 (4–16) | None reported |
| Spada et al. [41] | 2 |
Whole LT (n = 1) Split LT (n = 1) |
1.9 (0.75–3) | No decompensations | Adequate neurological development (n = 2) | None reported | 7 (2–12) | None reported |
| Stevenson et al. [42] | 3 | Combined LT/KT | 10.8 (n/s) | No decompensations | Not reported | Decreased renal function (n = 1) | > 1 (n/s) | None reported |
CMV cytomegalovirus, EBV Epstein-Barr virus, HAT hepatic artery thrombosis, KT kidney transplantation, LDLT living-donor liver transplantation, LT liver transplantation, LT/KT liver/kidney 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