Table 1 Demographic data

CHF

CHF-c

1

CHF

Bx (x2)

11

F

ADPKD

Y

Cirrhosis with multiple liver nodules

39

42

104

2.4

Transjugular intrahepatic portosystemic shunt

144

Alive, stable

2

CHF

Bx (x2)

6

M

ADPKD

Y

Cirrhosis

155

98

136

0.5

Kidney transplant

144

Alive, stable

3

CHF

Bx

22

M

ARPKD, spina bifida

Y

Cirrhosis

48

57

706

9.2

Kidney transplant (x3) and liver transplant

93

Alive

4

CHF

Bx

55

F

Chronic renal failure of unknown cause

Y

Cirrhosis with enhancing lesion

37

84

130

0.3

Dialysis, splenorenal shunt

4

Deceased (sepsis, multiorgan failure)

5

CHF

Bx

1

M

Congenital hypoglycemia, hyperammonemia, seizure, developmental delay

N

Hepatosplenomegaly, mild central intrahepatic duct dilatation

49

45

388

0.5

N/A

14

Alive, stable

6

CHF

Bx

18

F

ARPKD, Caroli syndrome, alpha1antitrypsin deficiency (PiMS)

Y

Caroli's disease

23

20

44

1.3

N/A

21

Alive

7

CHF

Bx

11

M

ADPKD, hereditary pancreatitis (PRSS1 mutationN291)

Y

Cirrhosis

25

28

169

<0.2

N/A

17

Alive

8

CHF

Bx

19

M

Fragile X syndrome, cognitive impairment (autism)

Y

Multiple bridging band of fibrosis, innumerable (>20) macronodule

279

369

351

1.5

N/A

14

Alive

CHF-i

9

CHF and steatohepatitis

Bx

70

F

Diabetes, Anti-mitochondria 1:320, steatohepatitis

N

Cirrhosis

222

132

118

N/A

N/A

72

Alive, stable

10

CHF

Bx

65

F

Epigastric pain, weight loss

N

Cirrhosis

17

23

80

0.3

N/A

39

Alive, stable

11

CHF and hepatocellular carcinoma

Explant

56

M

Hepatitis C cirrhosis

Y

Cirrhosis and liver lesion

44

146

73

42.7

Liver transplant

14

Deceased ( metastatic carcinoma )

12

CHF and hepatocellular carcinoma

Resection

71

M

Alcohol use

Y

Liver lesion

35

28

70

0.9

N/A

1

Alive

13

CHF and cholangiocarcinoma

Resection

70

F

Crohn's disease, stage IV descending colon cancer

Y

Liver lesion

45

33

72

0.8

N/A

33

Alive

14

Paucity of intrahepatic bile duct

Bx (x4)

12

M

Nephronophthisis type 11 (Homozygous TMEM67 gene 1843T>C 2012)

Y

Cirrhosis

169

253

1409

1.3

Kidney transplant

127

Alive, pending Liver transplant

15

HPS/NRH

Bx + explant

16

M

Nephrolithiasis

Y

Cirrhosis

45

18

107

0.5

Liver transplant

192

Alive

16

HPS

Bx (x3)

22

F

Hepatosplenomegaly and liver failureat 6 month of age

Y

Cirrhosis

38

30

74

2

N/A

38

Alive

17

HPS/NRH

Bx

44

F

Unilateral renal agenesis, long-term dialysis dependence

Y

Possible cirrhosis

9

18

181

1

N/A

1

Alive

18

HPS

Bx

3

F

Alport syndrome (Monoallelic POLG1 mutation), failure to thrive

N

Nodular liver surface

28

45

173

0.2

N/A

45

Deceased (Lemierre syndrome)

19

HPS/NRH

Bx

62

M

Chronic kidney disease, cardiomyopathy and heart failure

N

Cirrhosis

9

19

53

0.7

N/A

15

Deceased (Heart failure)