Table 2.

Clinical studies investigating changes in VWF and ADAMTS13 parameters in acute liver injury/failure.

Cohort	VWF				ADAMTS13			VWF: ADAMTS13	Other	Ref
	Ag	Act	Ratio	HMW Multimers	Ag	Act	Ratio	Ratio
ALI/ALF, mixed etiology (50% APAP) n=50	↑	↑	↓	↓	↓	↓	↓	NA	Increased VWF-dependent platelet adhesion and aggregation. Low ADAMTS13:Act associated with poor outcome	[44]
ALF, mixed etiology (45% APAP) with (n=10) or without (n=6) AKI	↑ in both groups, higher in patients with AKI	NA								[62]
AH (n= 27) and ALF (n=11)	↑ higher in ALF than AH	NA	NA	4 out 11 ALF had UL-VWF. None in AH	NA	↓ Lower in ALF than AH	NA	NA	ADAMTS13:Act higher in survivors than non-survivors. VWF:Ag lower in survivors. ADAMTS13 inhibitor in 67% of ALF patients and in 22% of AH.	[61]
ALF (n=40), unknown etiology	NA	↑	NA						↑ VWF deposition in liver	[64]
ALI/ALF (n=676), mixed etiology (46% APAP)	↑ highest in non-APAP	NA				↓ lowest in non-APAP	NA		↑ VWF levels, ↓ ADAMTS13 levels in patients with more severe ALI/ALF (presence of encephalopathy or SIRS). ↑VWF and lower ADAMTS13 in non-transplant-free survivors.	[78]

ALI/ALF, acute liver injury/failure; NA, not assessed; AKI, acute kidney failure; SIRS, systemic inflammatory response syndrome; AH, acute hepatitis; APAP, acetaminophen.