Clinical features of the 318 paediatric patients with periodic fever according to different genotypes
| MEVF | ||||
|---|---|---|---|---|
| Two mutations | One mutation | Negative | p Value* | |
| No of patients | 44 | 69 | 205 | |
| Age at onset (months; mean±SD) | 48±41 | 30±36 | 41±50 | 0.05 |
| Disease duration at screening (years; mean±SD) | 9±6.8 | 5.1±6.2 | 3.6±3.4 | NS |
| Duration of fever episodes (days; mean±SD) | 3±1.6 | 5.0±6.9 | 6.0±7.1 | 0.02 |
| Positive family history (%) | 33 | 25 | 12 | 0.01 |
| Severe abdominal pain (%) | 82 | 33 | 32 | <0.001 |
| Mild abdominal pain (%) | 5 | 35 | 20 | <0.001 |
| Chest pain (%) | 41 | 7 | 6 | <0.001 |
| Pleurisy (%) | 20 | 3 | 2 | <0.001 |
| Arthritis (%) | 13 | 9 | 12 | NS |
| Arthralgia (%) | 43 | 43 | 47 | NS |
| Myalgia (%) | 28 | 19 | 39 | 0.009 |
| Rash† (%) | 7 | 26 | 26 | <0.001 |
| Conjunctivitis (%) | 7 | 15 | 19 | NS |
| Exudative pharyngitis (%) | 26 | 51 | 52 | 0.005 |
| Erythematous pharyngitis (%) | 30 | 60 | 69 | <0.001 |
| Enlarged cervical lymph nodes (%) | 22 | 51 | 71 | <0.001 |
| Painful cervical lymph nodes (%) | 15 | 28 | 35 | 0.002 |
| Oral aphthosis (%) | 18 | 25 | 43,9 | <0.001 |
| Headache (%) | 52 | 37 | 45 | NS |
| Splenomegaly (%) | 9 | 12 | 14 | NS |
| Diarrhoea (%) | 37 | 48 | 27 | 0.005 |
| Vomiting (%) | 33 | 40 | 31 | NS |
| Erysipeloid rash (%) | 0 | 6 | 2.4 | NS |
| Periorbital oedema (%) | 0 | 9 | 9 | NS |