TABLE 4.

Mortality in conditions associated with congenital hepatic fibrosis

		CHF subtype
	All conditions	Isolated CHF	ARPKD	CD/CS	Type V CC
	Reported: 411	Reported: 41	Reported: 249	Reported: 117	Reported: 4
	Presence: 130	Present: 13	Present: 80	Present: 36	Present*: 1
Age at death, y: sample reported (median, range)	31 (14, 0–71.5)	8 (29, 12–61)	18 (5.7, 0–43)	6 (17, 0.9–71.5)
Development time from	36 (1.8, 0–45)	8 (2, 0–45)	23 (0.5, 0–32.8)	6 (1.8, 0.7–28)
CHF subtype diagnosis, y: sample reported (median, range)

Time followed after diagnosis (y): 164 reports (5, 0–38). Cause of death: 78 reports—sepsis (37%), cholangiocarcinoma (15%), gastrointestinal bleeding (6%), portopulmonary hypertension (5%), liver failure (4%), other (33%). Other causes of death included respiratory failure and pulmonary hypoplasia (6), cerebral ischemia (5), unspecified malignancy (3), graft failure (2), renal failure (2), encephelopathy (1), uremia (1), encephelopathy (1), multiple organ failure (1), central line complication (1), posttransplant lymphoproliferative disorder (1), motor vehicle crash (1), fulminant hepatitis B (1). ARPKD = autosomal recessive polycystic kidney disease; CC = choledochal cyst; CHF = congenital hepatic fibrosis; CS = Caroli syndrome.

^*No age of death reported.