Chelation therapy in liver diseases of childhood: Current status and response

Table 3 Pediatric studies of chelation in liver diseases

Ref.	Disease	Drug	Follow up duration	Response	Adverse effects
Dhawan et al[60]	WD	DPA (n = 32)	Median:11.78 (1.45-34.2) yr	20/32 (62.5%)	Minor- 6.3%; Major- 21.9%
Wang et al[106]	WD	DPA/TA (n = 9)	Mean: 5.1 4.1 yr	All responded	Not mentioned
Das et al[50]	WD	DPA (n = 65), TA(n = 4)	Median: 3.6 (0.8-12) yr	DPA (42/65) 64.6%, TA (3/4) 75%	DPA 10.8%
Arnon et al[107]	WD	TA (n = 10)	Treatment duration: 18 mo. Follow up:12-60 mo	All responded	1/10 (10%) reported hepatotoxicity
Taylor et al[108]	WD	TA (n = 16)	6.4 (0.78-18.6) yr	14/16 (87.5%)	1 had allergic reaction
Santos Silva et al[59]	WDAll decompensated liver disease	DPA (n = 1)TA (n = 4)	18-60 mo	All responded one still had raised transaminase	3/4 (75%) on DPA developed cytopenia
Bavdekar et al[73]	ICC	DPA (n = 68)	3.5 (1-7) yr	29/68 (42.6%) alive after follow up	5 children had proteinuria
Tomar et al[75]	ICC	DPA (n = 60)	12 mo duration	13/17 (76.5%) of grade III survived	11.8% drug rash, 5.9% fever
Tanner et al[74]	ICC (15 children treated with DPA in both trials together)	DPA (n = 15)	6 yr	Trial I: 1/15 (6.7%) survived in 6 yr, Trial II: 5/10 (50%) survived in 6 yr	Not mentioned
Horselen et al[77]	Case report CACC (age 7 yr)	DPA	19 mo	Hepatic copper normalized	none
Maggiore et al[78]	Case report CACC (age 10 yr)	DPA	24 mo	No improvement	Not mentioned
Rodeck et al[109]	CACC (age 6 and 10 mo)	DPA	18 mo, other child deteriorated immediately following DPA initiation	One child improved and other developed acute liver failure requiring liver transplantation	None
Flynn et al[83]2002	NH	DFO (n = 5) with antioxidant	Follow up at 48 mo	2/5 (40%) survived without transplantation	Not mentioned
Rodrigues et al[84] 2005	NH	DFO with antioxidant (n = 9)	Follow up 3-9.8 yr	1/9 (11.1%) survived without transplantation	Not mentioned
Sigurrdson et al[85] 1998	NH	DFO with antioxidant (n = 8)	Not mentioned	None survived without transplantation	Not mentioned
Masera et al[110] 2013	HJV hemochromatosis Case report (7/F)	DFX	12 mo of treatment	Iron indices improved on 12 mo treatment	Not mentioned

DPA: D-Penicillamine; TA: Trientine; WD: Wilson’s disease; ICC: Indian childhood cirrhosis; NH: Neonatal Hemochromatosis; DFO: Deferoxamine; DFX: Deferasirox; CACC: Copper associated childhood cirrhosis.