Angioedema and C1 inhibitor deficiency.
Deficiency of C1 inhibitor resulting in episodes of angioedema causes significant morbidity and mortality in affected patients, yet often goes undiagnosed for years. As biochemical understanding of the disorder has improved, competing theories for the pathophysiologic mechanism of angioedema have emerged. Further, existing therapeutic interventions have been refined by experience and newer modalities currently available in Europe may soon become available in the United States.
Orfan
N A
NA
Mary Imogene Bassett Hospital, Division of Allergy-Immunology, Cooperstown, New York.
Kolski
G B
GB
D000799:Angioedema / Q000209:etiology*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D005260:Female; D006801:Humans; D008297:Male
D003174:Complement C1 Inactivator Proteins