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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 3 Outcomes of LDLT using grafts from OTCD heterozygous carrier donors.

NumberOTCDCase 1OTCDCase 2OTCDCase 3OTCDCase 4OTCDCase 5
InstitutionsNagasaka H [] (2001)9 Mukhtar A [] (2013)10 Rahayatri TH [] (2016)11 Rahayatri TH [] (2016)11 Our patient [] (2012)12
Recipient age/gender4.8 y/Female42 y/Male5 y/Female5 y/Female3.3 y/Male
Recipient OTC activity of liver13%15%9.7%0%
Donor age/gender relationship/symptom35 y/FemaleMother/None36 y/FemaleSister/None34 y/FemaleMother/None38 y/FemaleMother/None32 y/FemaleMother/None
Donor allopurinol loading testOrotic acid(urine) (35.62μmol/L)Orotic acid(urine) (22.75 μmol/L)
Graft OTC activity of liver49%62%42.6%104.4%
GraftLLSGV: 252 gRLV: 79.8%RLGRWR: 0.9%RLV: 40%LLSLLSLLSGV/SLV: 54.0%RLV: 81.8%
Recipient post-transplant courseUneventfulPOD2: NH3 762POD5: deathPOD1: NH3 338CVVHD (4 days)Medication (phenylbutyrate)POD2: NH3 430CVVHD (3 days)No medicationUneventful
Donor post-transplant courseUneventfulPOD3: NH3 280POD12: dischargeUneventfulUneventfulUneventful
OTCD – ornithine transcarbamylase deficiency; y – years old; LLS – left lateral segment; RL – right lobe; GV – graft volume; GRWR – graft-to-recipient weight ratio; GV/SLV – graft volume/standard liver volume ratio; RLV – remnant liver volume; POD – post-operative day;0 CVVHD – continuous veno-venous hemodialysis.

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