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01 October 2021: Original Paper

Outcomes After Living Donor Liver Transplantation in Pediatric Patients with Inherited Metabolic Diseases

Yukihiro Sanada ABCDEF* , Yasunaru Sakuma DF , Yasuharu Onishi B , Noriki Okada B , Naoya Yamada B , Yuta Hirata B , Go Miyahara B , Takumi Katano B , Toshio Horiuchi B , Takahiko Omameuda B , Alan Kawarai Lefor DF , Naohiro Sata DF

DOI: 10.12659/AOT.932994

Ann Transplant 2021; 26:e932994

Table 4 Outcomes of LDLT for patients with MMA in our institution.

NumberMMACase 1MMACase 2MMACase 3
Recipient gestational age/BW/gender38 w/2860 g/Male36 w/2632 g/Female39 w/2576 g/Male
Onset/symptomDay3/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 typemutmutmut
TreatmentProtein-restricted diet (1.5 g/kg/day) levocarnitine, vitaminsProtein-restricted diet (2.0 g/kg/day) levocarnitine, vitaminsProtein-restricted diet (2.7 g/kg/day) levocarnitine, vitamins
Developmental disorder before LDLTDQ 70DQ 78
Recipient age at LDLT10 months old2 months old9 months old
Post-transplant complicationsPortal vein stenosisCytomegalovirus infectionHepatic vein stenosisCytomegalovirus infectionNone
Post-transplant protein-restricted dietSevere fussy eaterProtein-restricted diet (30 g/day)Concomitant use of protein-restricted milk
Medications at presentTac levocarnitine, vitamins (6.1POY)Tac levocarnitine, vitamins (3.8POY)Tac, mPSL, MMF levocarnitine, vitamins (3.7POY)
Growth disorder at presentBH: 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 presentSpecial needs educationNoneAttention-deficit hyperactivity disorder
Renal function at presenteGFR 77 ml/min/1.73 meGFR 110 ml/min/1.73 meGFR 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.

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Annals of Transplantation eISSN: 2329-0358
Annals of Transplantation eISSN: 2329-0358