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 1 Demographic data for recipients and graft information.
| Patient | Recipients with IMDs | Recipients with BA | p-value |
|---|---|---|---|
| Period | May 2001–December 2020 | ||
| Number | 44 | 221 | |
| Gender | Male: 22, Female: 22 | Male: 74, Female: 147 | 0.041 |
| Age (years old) | 3.0 (0.0–15.0) years old | 1.0 (0.0–16.0) years old | 0.076 |
| Weight | 14.2 (2.6–64.9) kg | 9.2 (4.8–58.5) kg | 0.079 |
| Original disease | OTCD: 19,Wilson’s disease: 5,Neonatal hemochromatosis: 5,Maple syrup urine disease: 4,MMA: 3,Progressive familial intrahepatic cholestasis type 1: 2,Citrullinemia: 2,Others: 4 | – | |
| ABO-compatibility | Identical/Compatible: 32,Incompatible: 12 | Identical/Compatible: 189,Incompatible: 32 | 0.046 |
| PELD/MELD score | 0 (0–29) | 11 (0–37) | |
| Type of graft | Left lateral segment: 20,Left lobe: 12,Segment 2 monosegment: 6,Left lobe + caudate lobe: 3,Reduced left lateral segment: 2,Segment 3 monosegment: 1 | Left lateral segment: 156,Left lobe: 41,Reduced left lateral segment: 13,Left lobe+caudate lobe: 7,Segment 2 monosegment: 3,Posterior segment: 1 | |
| GV/SLV | 65.7±19.0% | 73.7±21.0% | 0.012 |
| Operation time | 864 min±260 min | 870 min±280 min | 0.843 |
| Cold ischemic time | 111 min±59 min | 141 min±1 hr 51 min | 0.204 |
| Warm ischemic time | 50 min±19 min | 51 min±18 min | 0.451 |
| Bleeding volume | 79.3±117.7 ml/kg | 101.4±98.8 ml/kg | 0.002 |
| Transfusion volume | 133.6±202.8 ml/kg | 121.4±102.8 ml/kg | 0.070 |
| Observation period | 10.6±5.4 years | ||
| IMDs – inherited metabolic diseases; BA – biliary atresia; OTCD – ornithine transcarbamylase deficiency; MMA – methylmalonic academia; PELD – pediatric end-stage liver disease; MELD – model for end-stage liver disease; GV/SLV – graft volume/standard liver volume ratio. | |||