01 October 2021 : Original article
Outcomes After Living Donor Liver Transplantation in Pediatric Patients with Inherited Metabolic Diseases
Yukihiro Sanada1ABCDEF*, Yasunaru Sakuma1DF, Yasuharu Onishi1B, Noriki Okada1B, Naoya Yamada1B, Yuta Hirata1B, Go Miyahara1B, Takumi Katano1B, Toshio Horiuchi1B, Takahiko Omameuda1B, Alan Kawarai Lefor
1DF, Naohiro Sata1DF
DOI: 10.12659/AOT.932994
Ann Transplant 2021; 26:e932994
Table 4 Outcomes of LDLT for patients with MMA in our institution.
| Number | MMACase 1 | MMACase 2 | MMACase 3 |
|---|---|---|---|
| Recipient gestational age/BW/gender | 38 w/2860 g/Male | 36 w/2632 g/Female | 39 w/2576 g/Male |
| Onset/symptom | Day3/Suckling defect (pH 7.320, NH3 613) | Day22/Abnormal screening test (pH 7.180, NH3 232) | Day20/Abnormal screening test (pH 7.416, NH3 125) |
| Disease type | mut | mut | mut |
| Treatment | Protein-restricted diet (1.5 g/kg/day) levocarnitine, vitamins | Protein-restricted diet (2.0 g/kg/day) levocarnitine, vitamins | Protein-restricted diet (2.7 g/kg/day) levocarnitine, vitamins |
| Developmental disorder before LDLT | DQ 70 | – | DQ 78 |
| Recipient age at LDLT | 10 months old | 2 months old | 9 months old |
| Post-transplant complications | Portal vein stenosisCytomegalovirus infection | Hepatic vein stenosisCytomegalovirus infection | None |
| Post-transplant protein-restricted diet | Severe fussy eater | Protein-restricted diet (30 g/day) | Concomitant use of protein-restricted milk |
| Medications at present | Tac levocarnitine, vitamins (6.1POY) | Tac levocarnitine, vitamins (3.8POY) | Tac, mPSL, MMF levocarnitine, vitamins (3.7POY) |
| Growth disorder at present | BH: 113.5 cm (−2.3 SD)BW: 23.3 kg (−0.6S D) | BH: 98.0 cm (−0.9 SD)BW: 15.9 kg (0.0 SD) | BH: 96.2 cm (−2.2 SD)BW: 14.2 kg (−1.3 SD) |
| Developmental disorder at present | Special needs education | None | Attention-deficit hyperactivity disorder |
| Renal function at present | eGFR 77 ml/min/1.73 m | eGFR 110 ml/min/1.73 m | eGFR 96 ml/min/1.73 m |
| MMA – methylmalonic academia; BW – body weight; w – week of gestational age; LDLT – living donor liver transplantation; DQ – development quotient; Tac – tacrolimus; mPSL – methylprednisolone; MMF – mycophenolate mofetil; POY – post-operative year; BH – body height; SD – standard deviation; eGFR – estimated glomerular filtration rate. | |||