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