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Table 3 Pattern of inflammatory parameters in MWS patients carrying the NLRP3 E311K mutation

From: NLRP3 E311K mutation in a large family with Muckle-Wells syndrome - description of a heterogeneous phenotype and response to treatment

  Number of individuals (%) with elevations and associated levels
Classical inflammatory markers (normal)  
Elevated ESR (< 22 mm/h)  
   Number (%) 7/13 (54%)
   Mean mm/h (stdv) 31 (20-55 mm/h)
Elevated CRP (< 0.5 mg/dl)  
   Number (%) 13/13 (100%)
   Mean (stdv) 2.25 (0.66-5.6)
Elevated Ferritin (< 30 μg/dl)  
   Number (%) 3/13 (23%)
   range 33-48 μg/dl
Elevated Fibrinogen (< 170 mg/dl)  
   Number (%) 5/13 (38%)
   range 439-540 mg/l
Hematological abnormalities  
   Patients with hemoglobin abnormalities (%) 0/13 (0%)
   Patients with leukocytosis (%) 6/13 (46%)
   Mean leucocyte count/μl (range) 10,507 (7,170-21,200)
   Patients with neutrophilia (%) 5/13 (38%)
   Mean neutrophil count/μl (range) 7,695 (3434-18211)
   Patients with thrombocytosis (%) 0/13 (0%)
Biomarkers and cytokines  
Serum amyloid A (SAA) (< 10 mg/l)  
   Number (%) 10/13 (77%)
   mean (range) 40.7 (10-254)
Interleukin-1 (IL-1) (< 5 pg/ml)  
   Number(%) 0/8 (0%)
   mean (range) 0.52 (0.2-1.48)
Interleukin-6 (IL-6) (< 5 pg/ml)  
   Number (%) 5 (38%)
   mean (range) 6.37 (5.9-15.7)
Tumor necrosis factor α (TNF-α) (< 8 pg/ml)  
   Number (%) 7 (54%)
   mean (range) 9.37 (8,2-16.5)
TNF receptor I (TNF-RI) (< 1,966 pg/ml)  
   Number (%) 2 (15%)
   mean (range) 1,970-> 5,000
TNF receptor II (TNF-RII) (< 3,170 pg/ml)  
   Number (%) 2 (15%)
   mean (range) 3,210-> 5,000
Proteinuria  
Elevated total urine protein 10/13 (77%)
Elevated alpha 1-microglobulin [μg/ml] 5/13 (28%)
range 13.2-262
Elevated alpha 2-microglobulin (mg/l) 2/13 (15%)
range 0.3-> 1.2
  1. CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; IL-1, Interleukin-1; SAA, Serum amyloid A; TNF-α, Tumor necrosis factor α; TNF-RI, TNF receptor I;