Proteolytic inactivation of plasma C1- inhibitor in XXXX .

@entity1 @entity32644 @entity295 @entity19459 @entity3459 @entity214
Activation of both the complement system and the contact system of @entity3459 is implicated in the pathophysiology of @entity295 . Because @entity19459 ( @entity19459 ) regulates the activation of both cascade systems, we studied the characteristics of plasma @entity19459 in 48 @entity1 with severe @entity295 on admission to the Intensive Care Unit at the Free University of Amsterdam. The ratio between the level of functional and antigenic @entity19459 (functional index) was significantly reduced in the @entity1 with @entity295 compared with healthy volunteers (P = 0.004). The assessment of modified (cleaved), inactive @entity19459 ( @entity32644 -Inh), and complexed forms of @entity19459 (nonfunctional @entity19459 species) revealed that the reduced functional index was mainly due to the presence of @entity32644 -Inh. On @entity214 -PAGE, @entity32644 -Inh species migrated with a lower apparent molecular weight (Mr 98,000, 91,000, and 86,000) than native @entity19459 (Mr 110,000). Elevated @entity32644 -Inh levels (greater than or equal to 0.13 microM) were found in 81% of all @entity1 , sometimes up to 1.6 microM. Levels of @entity32644 -Inh on admission appeared to be of prognostic value: @entity32644 -Inh was higher in 27 @entity1 who died than in 21 @entity1 who survived (P = 0.003). The mortality in 15 @entity1 with @entity32644 -Inh levels up to 0.2 microM was 27%, but in 12 @entity1 with plasma @entity32644 -Inh exceeding 0.44 microM, the mortality was 83%. The overall mortality in the @entity1 with @entity295 was 56%. We propose that the cleavage of @entity19459 in @entity1 with @entity295 reflects processes that play a major role in the development of fatal complications during @entity295 .
