Table 2.C1 inhibitor deficiency Patient Nr.1 Patient Nr.2 Patient Nr.3 Patient Nr.4 Patient Nr.5 Sex Male Male Female Male Male Age 43 18 11 26 10 (years) Famiiy 1 Nr. 3 Recurrent edema Family Edema of upper of the upper Frequent Frequent airways(mother Glomerullonephritis history airways respiratory respiratory died),oncle,son (grand mother, infection infection,sinusitis father Frequent Frequent Edema of Clinical respiratory Frequent Recurrent expressi infections, respiratory respiratory infections, respiraotry cheeksafter on abdominal pain, infections stomatologic infections, abdominal pain treatment abdominal pain edema C34 C3 normal C3 normal C3 normal C3 normal Laborat C4 not detecable C4 not detecable C4 not detecable C4 not detecable C4 not detecable ory C1 INH not C1 INH not C1 INH not C1 INH not C1 INH not detecable detecable detecable detecable detecable CH1000 CH1000 CH1000 CH1000 CH1000 CIK normal CIK normal CIK normal CIK normal CIK normal Danazol Therapy Danazol,C1 C1 inhibitor C1 inhibitor C1 inhibitor inhibitor C1 inhibitorPatient Nr. 1 Patient Nr. 2 Patient Nr. 3 Patient Nr. 4 Patient Nr. 5 Sex Male Male Female Male Male Age (years) 43 18 11 26 10 Family Nr. 1 1 1 2 3 Family history Edema of upper airways (mother died), oncle, son Recurrent edema of the upper airways (grand mother, father Frequent respiratory infection Glomerullonephritis Frequent respiratory infection, sinusitis Clinical expressi on Frequent respiratory infections, abdominal pain, edema Frequent respiratory infections, abdominal pain Frequent respiraotry infections Edema of cheeksafter stomatologic treatment Recurrent respiratory infections, abdominal pain Laborat ory C3 C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA  IgG,IgM normal C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA, IgG  IgM normal C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA  IgG,IgM normal C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA  IgG,IgM normal C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA  IgG,IgM normal Therapy Danazol C1 inhibitor C1 inhibitor C1 inhibitor Danazol, C1 inhibitor C1 inhibitor Table 2. C1 inhibitor deficiency