TABLE 8.
Phenotypic features in EDA-ID patientsa
| Phenotypic feature | No. of patients affected/total no. (%) |
|---|---|
| Ectodermal dysplasia | 24/24 (100) |
| Osteopetrosis | 6/24 (25) |
| Lymphedema | 3/15 (20) |
| Bronchiectasis | 2/2 (100) |
| Mycobacterial infections | 8/24 (33) |
| Severe bacterial infections | 23/24 (96) |
| Severe viral infections | 1/14 (7) |
| Severe fungal infections | 2/22 (9) |
| Hypogammaglobulinemia | 7/9 (78) |
| Elevated IgM Levels | 4/7 (57) |
| Elevated IgA Levels | 5/9 (56) |
| Poor antibody responses to polysaccharide antigens | 6/7 (86) |
| Increased B-cell counts | 2/5 (40) |
| Decreased | 13/24 (54) |
a
Compiled from references 1, 18, 19, 79, 136, 175, 210, and 300.