Table 1.
Ages, Sex, Ethnicity, Consanguinity, Clinical Presentation, and Treatments of DIRA Patients.
| Median Age (Days) | IQR (Days) | Outlier (Days) |
|---|---|---|
| 8 | 24.208 | 121.667, 121.667, 364 * |
| Sex | n = 25 | Percentages (%) |
| Male | 16 | 64 |
| Female | 9 | 36 |
| Ethnicity | n = 25 | Percentages (%) |
| Asian Indian | 1 | 5 |
| Brazilian | 3 | 12 |
| Puerto Rican | 4 | 16 |
| Turkish | 1 | 4 |
| Iranian-Persian | 1 | 4 |
| Lebanese | 3 | 12 |
| Portuguese and French | 1 | 4 |
| Canadian (Newfoundland) | 1 | 4 |
| Dutch | 6 | 24 |
| Not Known | 3 | 12 |
| Consanguinity | n = 25 | Percentages (%) |
| Non-consanguineous | 13 | 52 |
| Consanguineous | 9 | 36 |
| Not Known | 3 | 12 |
| Clinical Presentation | n = 25 | Percentages (%) |
| Pustular Rash | 21 | 84 |
| Widening of Ribs | 15 | 60 |
| Periosteal Reaction | 13 | 52 |
| Multifocal Osteolytic Lesions | 17 | 68 |
| Cervical Vertebral Fusion | 4 | 16 |
| Hepatosplenomegaly | 6 | 24 |
| Multifocal Osteomyelitis | 12 | 48 |
| Hepatomegaly | 1 | 4 |
| Treatments | n = 25 | Percentages (%) |
| Prednisone/Prednislone/Methylprednisolone | 16 | 64 |
| Canakinumab | 2 | 8 |
| IVIG | 2 | 8 |
| Soriatane (acitretin) | 2 | 8 |
| Etanercept | 2 | 8 |
| Bisphosphonates IV | 1 | 4 |
| Methotrexate | 2 | 8 |
| Azathioprine | 1 | 4 |
| Cyclosporine | 2 | 8 |
| Thalidomide | 1 | 4 |
| Interferon-γ | 1 | 4 |
| Oral/Intravenous Antibiotics | 17 | 68 |
| Antifungals | 2 | 8 |