5
Table 1 Number and Type of Hereditary Angioedema Attacks in 209 Patients . . . . . . . . . . . . . . . . . . . . . . . . . 19
Table 2 Number and Type of Attacks in 26 Pediatric Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Table 3 Type of Angioedema Attacks in 1,168 Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Table 4 Per-episode and Per-patient Analysis of Cutaneous Swelling Sites . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
Table 5 Symptoms Associated With 33,671 Abdominal Attacks in 153 Patients . . . . . . . . . . . . . . . . . . . . . . . 21
Table 6 Factors That Precipitated Attacks of Angioedema in Patients With Hereditary Angioedema . . . . . . . . 22
Table 7 Differential Diagnosis of Hereditary, Allergic, and ACE-inhibitor-related Angioedema . . . . . . . . . . . . . 27
Table 8 Pharmacokinetics of Berinert
®
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
Table 9 Times to Onset of Symptom Relief and Complete Resolution by Body Location in I.M.P.A.C.T.1 . . . . . 37
Table 10 Duration of 11,150 Episodes of Cutaneous Swelling in 47 HAE Patients Left Untreated
or Treated With Berinert
®
C1-INH Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
Table 11 Summary of Adverse Events in Clinical Trials . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44
Table 12 Summary of Adverse Events Occurring in at Least 2 Patients by Preferred Term. . . . . . . . . . . . . . . . . . 44
Table 13 Overview of the Safety Profile Between 1985 and 2011 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
Table 14 Contents in Each 500 IU Vial of Berinert
®
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Table 15 C1-INH Inhibits Numerous Target Proteases at its Serpin Domain . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
Table 16 C1-INH Binds Reversibly With Target Substrates at its Nonserpin Domain . . . . . . . . . . . . . . . . . . . . . . 57
Table 17 Biochemical Properties of Different C1-INH Concentrates . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Table 18 Virus Reduction Capacity of the Manufacturing Process of Berinert
®
. . . . . . . . . . . . . . . . . . . . . . . . . . 62
Table 19 Mean Prion Reduction Capacity of the Manufacturing Process of Berinert
®
. . . . . . . . . . . . . . . . . . . . . 63
List of Tables
Contents
