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Table 1 Levels of cytokines in children with the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) and children with pneumonia (Pn)

From: Elevated levels of CXCL10 in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome (PFAPA) during and between febrile episodes; an indication of a persistent activation of the innate immune system

  Pn-nf p-value PFAPA-nf p-value PFAPA-f p-value Pn-f
  Pn-nf   PFAPA-f   PFAPA-f  
  vs   vs   vs  
  PFAPA-nf   PFAPA-nf   Pn-f  
  (n=10)   (n=21) (n=13) (n=14)   (n=14)
IL-1β nd 0.004 2.5 ns 2.6 0.002 nd
(LoD: 0.8)    (0.1, 5.4)   (0.9, 5.1)   
CXCL10 731.9 0.000 1762.5 .003 4787.2 0.002 1512.7
(LoD: 6.5) (582.7, 1038.0)   (1305.6, 3401.0)   (2801.1, 7743.5)   (685.5, 3179.4)
IL-6 15.4 ns 11.2 .001 49.6 0.035 21.7
(LoD: 1.1) (9.5, 23.7   (4.8, 19.0)   (21.1, 103.8)   (18.3, 29.7)
CCL4 70.5 ns 52.8 .019 63.0 ns 59.4
(LoD: 1.1) (52.9, 83.6)   (30.3, 77.2)   (47.5, 93.4)   (44.7, 75.0)
IL-17 41.6 ns 17.4 ns 7.4 0.002 64.5
(LoD: 0.2) (25.4, 135.7)   (0.7, 54.6)   (0.7, 29.5)   (26.6, 81.2)
CCL5 6878.3 0.017 3197.5 ns 3007.8 0.016 4856.7
(LoD: 1.2) (4066.2, 7154.3)   (2200.8, 4694.1)   (1864.5, 4019.3)   (3028.0, 6916.8)
IL-1ra 234.0 ns 129.3 ns 121.5 0.002 249.3
(LoD: 1.4) (116.4, 368.1)   (69.5, 196.8)   (95.7, 170.8)   (176.7, 328.9)
IL-2 17.6 0.010 nd ns nd 0.000 17.6
(LoD: 1.1) (7.3, 29.9)       (9.2, 21.7)
IL-9 24.4 ns 4.0 ns 6.0 0.011 29.7
(LoD: 0.7) (11.6, 77.5)   (0.6, 30.8)   (0.5, 17.7)   (15.3, 41.3)
CCL11 65.0 ns 20.8 ns nd 0.007 59.3
(LoD: 14.6) (10.4, 133.1)   (0.3, 71.6)     (14.7, 115.1)
FGF-2 35.9 ns 24.0 ns 26.4 0.044 44.7
(LoD: 6.8) (24.5, 63.2)   (4.8, 47.7)   (14.1, 40.6)   (27.4, 55.7)
G-CSF 74.0 ns 92.0 ns 126.9 0.016 94.0
(LoD: 1.1) (54.8, 103.4)   (61.6, 111.9)   (95.9, 169.0)   (55.6, 106.9)
CCL2 38.0 ns 36.1 ns 38.0 0.039 27.6
(LoD: 6.8) (34.5, 41.9)   (26.2, 42.1)   (28.4, 54.5)   (19.2, 32.9)
CCL3 15.5 ns 3.0 ns 4.2 0.004 16.9
(LoD: 2.4) (8.2, 21.5)   (0.2, 14.8)   (0.4, 7.0)   (13.6, 18.9)
PDGF-BB 1440.5 0.025 532.4 ns 406.0 0.004 1284.0
(LoD: 1.0) (529.7, 1942.0)   (301.5, 910.1)   (263.2, 1093.6)   (1056.0, 2813.3)
CXCL8 (IL-8) 19.1 ns 20.1 ns 19.0 ns 24.3
(LoD: 0.5) (12.7, 32.3)   (14.0, 29.9)   (15.6, 23.4)   (18.5, 29.1)
INF-γ 198.9 ns 279.4 ns 323.5 ns 226.6
(LoD: 19.3) (125.3, 336.9)   (208.3, 418.9)   (235.3, 437.4)   (166.7, 306.3)
IL-4 5.7 ns 7.0 ns 6.8 ns 6.8
(LoD: 0.5) (3.1, 8.2)   (4.9, 9.8)   (4.9, 9.4)   (4.4, 8.2)
IL-5 6.3 ns 5.7 ns 5.8 ns 12.0
(LoD: 0.8) (1.8, 21.2)   (3.1, 9.4)   (4.5, 9.3)   (1.8, 18.3)
IL-7 19.9 ns 25.9 ns 25.4 ns 19.5
(LoD: 0.5) (11.5, 26.3)   (19.1, 31.1)   (19.4, 29.6)   (12.5, 26.3)
IL-10 13.1 ns 6.5 ns 8.1 ns 8.3
(LoD: 0.9) (1.6, 24.9)   (3.2, 13.7)   (4.3, 11.1)   (1.2, 16.8)
IL-12p70 46.1 ns 25.8 ns 24.5 ns 40.4
(LoD: 0.5) (18.5, 89.5)   (12.2, 40.8)   (18.1, 33.4)   (14.2, 72.2)
IL-13 16.9 ns 10.1 ns 10.4 ns 13.3
(LoD: 2.1) (8.3, 36.1)   (6.4, 17.5)   (7.6, 14.0)   (7.2, 21.9)
IL-15 nd ns nd ns nd ns nd
(LoD: 4.2)        
GM-CSF nd ns nd ns nd ns nd
(LoD: 4.5)        
TNF-α 66.2 ns 67.1 ns 68.1 ns 82.5
(LoD: 3.0) (40.6, 126.8)   (42.9, 101.6)   (56.3, 113.1)   (55.9, 116.2)
VEGF 43.2 ns 22.3 ns 30.6 ns 38.3
(LoD: 0.5) (24.4, 70.0)   (9.2, 47.1)   (9.1, 43.4)   (21.7, 87.3)
  1. Values are given as pg/ml and presented as median (quartiles).
  2. Pn-nf: Children with pneumonia after complete remission.
  3. PFAPA-nf: Children with PFAPA between febrile episodes.
  4. PFAPA-f: Children with PFAPA during febrile episodes.
  5. Pn-f: Children with pneumonia shortly after admission.
  6. LoD: Limit of detection as stated by the manufacturer.
  7. ns: not significant.
  8. nd: not detectable.
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Pediatric Rheumatology

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