Recombinant human C1-inhibitor in the treatment of acute angioedema attacks.
BACKGROUND
Patients with hereditary C1-inhibitor deficiency have recurrent attacks of angioedema, preferably treated with C1-inhibitor concentrate. A recombinant human C1-inhibitor (rHuC1INH) was developed, derived from milk from transgenic rabbits. This study was undertaken to investigate the effects of rHuC1INH in the treatment of acute angioedema attacks in patients with a hereditary C1-inhibitor deficiency.


METHODS
Patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH (at a dose of 100 U/kg) within 8 hours after onset of an acute attack. Time to initiation of relief and time to minimal symptoms were reported by both the patient and the treating physician.


RESULTS
Thirteen severe angioedema attacks in 9 patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH. There was rapid improvement of clinical conditions for all attacks, with approximately 80 percent of treated patients experiencing symptom relief within 2 hours and minimal symptoms within 12 hours. There were no drug-related adverse events or immunogenic reactions to C1-inhibitor or rabbit proteins.


CONCLUSIONS
The transgenically produced rHuC1INH was successfully used in the treatment of acute angioedema attacks in patients with hereditary C1-inhibitor deficiency.
Choi
Goda
G
Department of Internal Medicine, Academic Medical Center, Free University Medical Center, Amsterdam, The Netherlands.
Soeters
Maarten R
MR
Farkas
Henriette
H
Varga
Lilian
L
Obtulowicz
Krystyna
K
Bilo
Barbara
B
Porebski
Greg
G
Hack
C Erik
CE
Verdonk
René
R
Nuijens
Jan
J
Levi
Marcel
M
D000208:Acute Disease; D000328:Adult; D000799:Angioedema / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D000818:Animals; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage* / Q000009:adverse effects / Q000235:genetics; D005260:Female; D030342:Genetic Diseases, Inborn / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D011817:Rabbits; D011859:Radiography; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000009:adverse effects / Q000235:genetics; D013997:Time Factors; D016896:Treatment Outcome
D050718:Complement C1 Inhibitor Protein; D011994:Recombinant Proteins