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 DFDOI: 10.12659/AOT.932994
Ann Transplant 2021; 26:e932994
Table 3 Outcomes of LDLT using grafts from OTCD heterozygous carrier donors.
Number | OTCDCase 1 | OTCDCase 2 | OTCDCase 3 | OTCDCase 4 | OTCDCase 5 |
---|---|---|---|---|---|
Institutions | Nagasaka H [] (2001)9 | Mukhtar A [] (2013)10 | Rahayatri TH [] (2016)11 | Rahayatri TH [] (2016)11 | Our patient [] (2012)12 |
Recipient age/gender | 4.8 y/Female | 42 y/Male | 5 y/Female | 5 y/Female | 3.3 y/Male |
Recipient OTC activity of liver | 13% | – | 15% | 9.7% | 0% |
Donor age/gender relationship/symptom | 35 y/FemaleMother/None | 36 y/FemaleSister/None | 34 y/FemaleMother/None | 38 y/FemaleMother/None | 32 y/FemaleMother/None |
Donor allopurinol loading test | Orotic acid(urine) (35.62μmol/L) | – | – | – | Orotic acid(urine) (22.75 μmol/L) |
Graft OTC activity of liver | 49% | – | 62% | 42.6% | 104.4% |
Graft | LLSGV: 252 gRLV: 79.8% | RLGRWR: 0.9%RLV: 40% | LLS | LLS | LLSGV/SLV: 54.0%RLV: 81.8% |
Recipient post-transplant course | Uneventful | POD2: NH3 762POD5: death | POD1: NH3 338CVVHD (4 days)Medication (phenylbutyrate) | POD2: NH3 430CVVHD (3 days)No medication | Uneventful |
Donor post-transplant course | Uneventful | POD3: NH3 280POD12: discharge | Uneventful | Uneventful | Uneventful |
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. |