| Sex | Age at onset/diagnosis | Genetic testing | Initial clinical manifestations | Age at LT, years | Indication for LT | Donor | Heterozygous donor | Graft type | Graft weight, g | GRWR, % | Operation time, minutes | Blood loss, ml/kg | CIT, minutes | ICU Stay, days | Hospital stays, days | Posttransplant complications |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | F | 3Â days/5Â months | c.839dupT, c.996_998del in PCCA | Poor feeding, episodic vomiting, lethargy | 1.7 | Frequent MDs | Mother | Yes (c.996_998del) | Left lateral | 210 | 1.84 | 500 | 17.5 | 145 | 4 | 33 | CMV Viremia, EBV Viremia |
2 | F | 6 months/7 months | c.30_39del10, c.331C > T in PCCB | Poor feeding, lethargy | 2.1 | Frequent MDs | Father | Yes (c.30_39del10) | Left lateral | 299 | 2.60 | 390 | 9.6 | 100 | 4 | 32 | CMV Viremia, EBV Viremia, Bile leakage |
3 | M | 6 months/7 months | c.733G > A in PCCB* | Poor feeding, lethargy, muscle hypotonia | 5.8 | Frequent MDs | Mother | No | Left lateral | 252 | 1.68 | 525 | 4.7 | 85 | 5 | 30 | EBV Viremia |
4 | M | 3 months/4 months | c.1207C > T, c.319G > A in PCCB | Poor feeding, episodic vomiting, lethargy | 1.3 | Frequent MDs | Father | Yes (c.319G > A) | Reduced-size left lateral | 144 | 1.92 | 385 | 40.0 | 110 | 6 | 32 | EBV Viremia |
5 | F | 10 days/8 months | c.2002G > A (homozygous) in PCCA | Poor feeding, episodic vomiting | 2.7 | Frequent MDs and dilated cardiomyopathy | Mother | Yes (c.2002G > A) | Left lateral | 206 | 1.87 | 290 | 13.6 | 104 | 4 | 12 | CMV Viremia |
6 | M | 6 months/8 months | c.331C > T, c.493C > T in PCCB | Neurodevelopmental delay | 4.5 | Preventative treatment | Father | Yes (c.331C > T) | Left lateral | 236 | 1.31 | 443 | 5.6 | 144 | 2 | 13 | CMV Viremia |
7 | M | 21 days/1.5 months | c.1283C > T, c.839delT in PCCB | Poor feeding, episodic vomiting, lethargy, seizure | 1.1 | Frequent MDs | Father | Yes (c.839delT) | Left lateral | 205 | 1.99 | 336 | 8.7 | 138 | 13 | 17 | CMV Viremia |