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Table 1 Demographics, preoperative characteristics, and operative findings of seven recipients with propionic acidemia who underwent LDLT

From: Therapeutic potential of living donor liver transplantation from heterozygous carrier donors in children with propionic acidemia

 

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

3 days/5 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

  1. CIT cold ischemia time, CMV cytomegalovirus, d day, EBV Epstein-Barr virus, ICU intensive care unit, GRWR graft-to-recipient weight ratio, LDLT living donor liver transplantation, MDs metabolic decompensations
  2. *There was suspected heterozygous deletion mutation in exon 9 of the PCCB gene