Groups | Case # | Final histologic diagnosis | Specimena | Age | Sex | Pertinent disease or congenital disorders | Portal hyertension (Y/N) | Liver imaging | ALT | AST | ALP | TB | Treatment | Follow-up (months) | Outcome | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
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 | ||
Mimics | 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) |