Dr. Jonathan Bernstein now presenting on bradykinin receptors - B2R is constitutively present, expressed in many tissues.
Factor XIIa initiates a cascade leading to bradykinin formation. Kallikrein regulates the conversion of Factor XII to Factor XIIa.
There are individuals have been identified with a congenital deficiency of prekallikrein - no apparent clinical complications attributable to detect - aPTT prolonged on lab test but no associated clinical significance.
Current treatment strategies in hereditary angioedema - on demand acute treatment vs. prophylaxis.
In phase III trials for HAE - lanadelumab, oral kallikrein inhibitors (avoralstat and bcx7353) and a new subcutaneous C1 inhibitor.
Lanadelumab - shown in Phase III to have a significant reduction in the mean HAE attack rate and increased numbers of attack-free patients. Editor's note: lanadelumab is FDA-approved in the U.S. as of 2018.
As our understanding of HAE pathophysiology increases, the management will continue to evolve.
Dr. Ellis @DrAnneEllis