Table 2

Clinical features of the 318 paediatric patients with periodic fever according to different genotypes

Two mutationsOne mutationNegativep Value*
No of patients4469205
Age at onset (months; mean±SD)48±4130±3641±500.05
Disease duration at screening (years; mean±SD)9±6.85.1±6.23.6±3.4NS
Duration of fever episodes (days; mean±SD)3±1.65.0±6.96.0±7.10.02
Positive family history (%)3325120.01
Severe abdominal pain (%)823332<0.001
Mild abdominal pain (%)53520<0.001
Chest pain (%)4176<0.001
Pleurisy (%)2032<0.001
Arthritis (%)13912NS
Arthralgia (%)434347NS
Myalgia (%)2819390.009
Rash (%)72626<0.001
Conjunctivitis (%)71519NS
Exudative pharyngitis (%)2651520.005
Erythematous pharyngitis (%)306069<0.001
Enlarged cervical lymph nodes (%)225171<0.001
Painful cervical lymph nodes (%)1528350.002
Oral aphthosis (%)182543,9<0.001
Headache (%)523745NS
Splenomegaly (%)91214NS
Diarrhoea (%)3748270.005
Vomiting (%)334031NS
Erysipeloid rash (%)062.4NS
Periorbital oedema (%)099NS
  • * p Values were assessed by a χ2 test for heterogeneity.

  • Non-specific rash (urticarial, erythematous, maculopapular), in at least one occasion.

  • NS, not significant.