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. 2014 Jul 28;4(3):247–256. doi: 10.1016/j.jceh.2014.07.005

Figure 1.

Figure 1

Proposed role of ADAMTS 13-ULvWF imbalance in pathogenesis of non-cirrhotic intrahepatic portal hypertension (NCIPH) and porto-pulmonary hypertension. A: Normal. Normal ADAMTS 13 production by the hepatic stellate cell leads to rapid cleavage of vWF from its p-selectin anchor; B: An increased pro-inflammatory drive of splanchnic origin leads to increased vWF production in venous endothelium which in the face of low ADAMTS 13 production results in ULvWF and platelet microthrombi; C: In the presence of low ADAMTS 13 production and increased splanchnic pro-inflammatory drive, porto-systemic shunting leads to build up of ULvWF and platelet rich thrombi in the pulmonary capillaries (Adapted from Ref 34). ADAMTS 13 :A disintegrin and metalloprotease with thrombospondin type 1 motif, member 13.