Table 5.
Overview of data from MMA 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) |
|---|---|---|---|---|---|---|---|---|
| Almeida et al. [70] | 1 | LT | 10 (n/a) | Not reported | Post-LT VIQ, PIQ and FSIQ scores displayed a positive change (VIQ = 91; PIQ = 84; FSIQ = 84) | None reported | 1 (n/a) | None reported |
| Barshop et al. [71] | 1 | DLT | 28 (n/a) | No metabolic decompensations | Not specified | None reported | 0.75 (n/a) | None reported |
| Boyer et al. [72] | 4 | DDKT | 5.7 (5–10) | Number per year dramatically decreased (no data specified) | Neurological complications stabilised (no further deterioration) | Hepatocarcinoma (n = 1) | n/s (0.5–3.0) | Hepatocarcinoma (n = 1) |
| Corno et al. [47] | 1 | LT | 9 (n/a) | Fatal metabolic crisis (n = 1) | Not reported | Fatal metabolic crisis (n = 1) | Not reported | Fatal metabolic crisis (n = 1) |
| Fukuda et al. [73] | 10 | LDLT | n/s (0.6–7) |
Severe metabolic acidosis (n = 2) No further metabolic decompensations in surviving patients (n = 9) |
No significant improvement in patient IQ |
Viral infection (n = 6) Severe metabolic acidosis (n = 2) |
3.5 (n/s) | Severe metabolic acidosis following rejection and sepsis (n = 2) |
| Jiang and Sun [74] | 7 |
LDLT (n = 3) DDLT (n = 4) |
Not specified | No further metabolic crises | Not specified | None reported | Not specified | None reported |
| Matsumoto et al. [75] | 1 | LDKT | 26 (n/a) | No further metabolic crises | Not specified | None reported | 0.5 (n/a) | None reported |
| Nakajima et al. [76] | 1 | LDLT | 5.3 (n/a) | Marked reduction of metabolic decompensation (no additional data) | Leigh’s encephalopathy (n = 1) | Leigh’s encephalopathy (n = 1) | > 1.5 (n/a) | None reported |
| Shenoy et al. [77] | 5 | KT | 10.8 (5.8–17.8) | No episodes of metabolic decompensation reported in the immediate perioperative period | Neurological deterioration (n = 1) |
Severe haemorrhagic pancreatitis (n = 1) Bacterial endocarditis (n = 1) Progressive deterioration in graft function (n = 1) Pancreatitis (n = 1) Neurological deterioration (n = 1) |
≤ 6 |
Severe haemorrhagic pancreatitis leading to death (n = 1) Bacterial endocarditis, progressive deterioration in graft function, pancreatitis, and neurological deterioration (n = 1) |
| Sissaoui et al. [78] | 13 |
KT (n = 7) Combined LT/KT (n = 5) LT (n = 1) |
KT: 9.7 (5–17) Combined LT/KT: 14 (6–19) LT: n/s (n/a) |
Not specified |
LT/KT: Axonal neuropathy (n = 1) Myoclonus (n = 1) |
KT: renal failure recurrence (n = 4) Combined LT/KT: graft rejection (n = 1) Biliary problems (n = 1) Axonal neuropathy (n = 1) Myoclonus (n = 1) LT: none reported |
KT: 5 (n/s) Combined LT/KT: 1.5 (n/s) LT: 0.5 (n/a) |
None reported |
| Yamamoto et al. [79] | 1 | KT | 26 (n/a) | No further episodes of metabolic decompensation reported | No further neurological deterioration (at 10 months post-transplant) | None reported | 0.8 (n/a) | None reported |
| Yoshino et al. [80] | 2 | LT | 6.3 (5.2–7.3) | Not specified |
Episodes of quick torsional movements of the head (n = 1) Tonic seizure (n = 1) |
Episodes of quick torsional movements of the head (n = 1) Tonic seizure (n = 1) Weakness of the right extremities and flexion of the right upper extremity (n = 1) |
2.3 (2–2.6) | None reported |
ALT auxiliary liver transplantation, CMV cytomegalovirus, KT kidney transplantation, LDLT living-donor liver transplantation, LT liver transplantation, LT/KT liver/kidney transplantation, OLT orthotopic liver transplantation, n/a not available, n/s not specified
aVarious types of liver transplantation used