Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations

Table 1 Clinical characteristics and outcome of non-cirrhotic portal fibrosis in pediatric studies

Parameters		Prasad et al[90] (n = 45)	Sood et al[91] (n = 19)	Poddar et al[109] (n = 11)	Franchi-Abella et al[110] (n = 48)
Mean or median (range) age at presentation		14.5 (6-18) yr	13.8 (5.9-17.6) yr	11 (5-14) yr	8.75 (1 mo-16 yr)
At presentation
	Variceal bleed	49%	15.70%	54.60%	18.80%
	Lump upper abdomen	47%	84.20%	45.40%	43.80%
	Ascites	20%	-	18%	-
	Spleen size (mean) cm	10.5	12 (4.75–17.25)	8	-
	Variceal recurrence	39%	-	18%	-
Poor outcome
	Decompensation	4%	0	0	12.50%
	Hepatopulmonary syndrome	2%	5%	-	4.20%
	Follow-up duration (mean)	48 (3-120) mo	18 (2-51) mo	57.5 (12-78) mo	15 (1-26) yr
	Survival without transplant	93%	100%	100%	88%

Table 2 Clinical characteristics and outcome of congenital hepatic fibrosis in pediatric studies

Parameters		Rawat et al[106] (n = 40)	Poddar et al[105] (n = 15)	Parkash et al[111] (n = 25)	Luoto et al[112] (n = 27)
Mean or median age		1.3 yr	8 yr (10 mo-14 yr)	8.5 ± 2.7 yr	2.7 (0-13) yr
Associations
	Caroli’s syndrome	52.50%	9%	8%	11%
	Renal	92.50%	81.80%	24%	100%
	CHF	47.50%	54.50%	92%	37%
Presentations
	Variceal bleeding	27%	54.50%	60%	15%
	Cholangitis	25%	9%	0%	7.40%
	Recurrent cholangitis	7.50%	9%	0%	3.70%
	Decompensation	5%	18.20%	0%	19%
	Endotherapy	27%	100%	60%	30%
	Shunt surgery	0%	9%	20%	3.70%
Transplant
	Renal transplant	0%	-	-	41%
	Liver transplant	5%	-	-	3.70%
	Combined liver kidney transplant	45%	-	-	37%
Survival
	Overall survival	90%	100% [41 (1-80) mo]	100%	70% [10.6 (0.6-40) yr]
	Survival post-transplant (follow-up duration)	80% [5 (1.2-9)] yr	-	-	73.30%
	Survival non-transplant (follow-up duration)	100% [15 (4.5-19)] yr	-	-	100%

CHF: Congenital hepatic fibrosis.