Prekallikrein activation and high-molecular-weight kininogen consumption in hereditary angioedema.

@article{Schapira1983PrekallikreinAA,
  title={Prekallikrein activation and high-molecular-weight kininogen consumption in hereditary angioedema.},
  author={Marc Schapira and Lynn D Silver and Cheryl F. Scott and Alvin H Schmaier and L J Prograis and John G. Curd and Robert W. Colman},
  journal={The New England journal of medicine},
  year={1983},
  volume={308 18},
  pages={1050-3}
}
Patients with hereditary angioedema lack C-1 inhibitor, a plasma alpha 2-glycoprotein that inhibits both the proteolytic action of C1, the activated first component of the complement system, and the activity of components of the contact phase of coagulation: kallikrein, factor XIa, and factor XIIa. Such patients have been shown to have low levels of C4 and C2, the natural substrates for C-1, but the levels were not correlated with the presence of symptoms. We studied three patients with… CONTINUE READING