Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome—Case Series and Literature Review

Table 2

Difference analysis in clinical features between children and adults in 63 Chinese case reports.

Clinical features	Children (); (%)	Adults (); (%)	value
Abnormal liver function	5 (17%)	9 (27%)	0.312
Liver transplant	4 (13%)	4 (12%)	1.00
Partial hepatectomy	5 (17%)	6 (18%)	0.874
Hepatomegaly	17 (57%)	7 (21%)	0.004
Splenomegaly	21 (70%)	22 (67%)	0.777
Splenectomy	3 (10%)	8 (24%)	0.137
EGVB	15 (50%)	19 (58%)	0.547
Ascites	5 (17%)	10 (30%)	0.204
Polycystic kidneys	8 (27%)	16 (48%)	0.075
Medullary sponge kidneys	4 (13%)	3 (9%)	0.700
Bile duct abnormalities	13 (43%)	19 (58%)	0.259
Portal vein malformations	3 (10%)	3 (9%)	1.000
Other systems	4 (13%)^†	7 (21%)^††	0.411

EGVB = esophageal and gastric variceal bleeding. ^†Four cases including pulmonary interstitial fibrosis, uterine and kidney malformation, patent ductus arteriosus, and arachnoid cyst. ^††Seven cases including arachnoid cyst and cerebral dysplasia, cerebral aneurysm, polycystic ovary syndrome, breast dysplasia, duodenal diverticulum tumor, macular degeneration, and IgG4-related autoimmune pancreatitis.