Table 2.
Biomarker levels in acute intermittent porphyria cases and controls
| Controls (n = 50) | a AIP cases (n = 50) | § Fold increase | $ Healthy controls | |
|---|---|---|---|---|
| U b ‐PBG c (μmol/mmol creatinine) | 0·4 (0·3–0·5) | 2·6 (0·9–8·5)*** | 6·5 | |
| U b ‐total porphyrins (nmol/mmol creatinine) | 6·7 (4·4–10·6) | 25·7 (8·5–83)*** | 3·8 | |
| S d ‐ALT (IU/l) e | 24·5 (18·8–34) | 31·0 (23–45) # | 1·3 | |
| P f ‐IL‐1β g (pg/ml) | 0·9 (0·65–1·2) | 1·6 (0·9–3·3)*** | 1·8 | 0·9 (0·4–2·0) |
| P f ‐ IL‐1RA g (pg/ml) | 28·0 (13–50) | 64·0 (23–197)*** | 2·3 | 21 (0–117) |
| P f ‐IL‐2 g (pg/ml) | 1·0 (0·0–2·0) | 6·0 (1·0–16)*** | 6·0 | 0·03 (0·0–7·9) |
| P f ‐IL‐4 g (pg/ml) | 1·0 (0·2–1·0) | 2·0 (1·0–4·0)*** | 2·0 | 1·0 (0·0–2·0) |
| P f ‐IL‐5 g (pg/ml) | 1·1 (0·7–1·4) | 1·7 (1·3–4·0)*** | 1·6 | 1·0 (0·5–3·9) |
| P f ‐IL‐6 g (pg/ml) | 2·0 (0·6–3·0) | 5·0 (2·0–9·3)*** | 2·5 | 2·0 (0·02–6·9) |
| P f ‐IL‐7 g (pg/ml) | 3·0 (1·4–5·0) | 7·0 (5·0–14)*** | 2·3 | 3·0 (0·0–11) |
| P f ‐CXCL8 g (pg/ml) | 5·0 (3·0–7·0) | 8·0 (6·0–14)*** | 1·6 | 4·5 (0·8–19) |
| P f ‐IL‐9 g (pg/ml) | 5·5 (4·0–8·3) | 14·0 (8·8–22)*** | 2·6 | 5·5 (2·0–32) |
| P f ‐IL‐10 g (pg/ml) | 0·14 (0·0–0·53) | 1·4 (0·2–6·0)*** | 10·1 | 0·1 (0·0–9·5) |
| P f ‐IL‐12(p70) g (pg/ml) | 2·0 (0·2–5·3) | 13·5 (6·8–25)*** | 6·8 | 1·8 (0·0–32) |
| P f ‐IL‐13 g (pg/ml) | 2·2 (1·7–4·0) | 5·0 (2·2–8·3)*** | 2·3 | 2·1 (0·9–14) |
| P f ‐IL‐15 g (pg/ml) | 0·0 (0–0·12) | 1·25 (0·0–5·0)*** | j ND | 0·0 (0·0–2·0) |
| P f ‐IL‐17 g (pg/ml) | 0·65 (0·0–9·3) | 16·0 (3·5–55)*** | 24·6 | 0·5 (0·0–54) |
| P f ‐CCL2 h (pg/ml) | 11·0 (8·0–14·0) | 16·5 (13·0–22)*** | 1·5 | 10·0 (3·0–28) |
| P f ‐CCL3 h (pg/ml) | 1·0 (0·15–3·0) | 3·0 (2·0–6·0)*** | 3·0 | 1·0 (0·0–11) |
| P f ‐CCL4 h (pg/ml) | 35·0 (29–46) | 57·5 (45–69)*** | 1·6 | 34·0 (21–157) |
| P f ‐CCL5 h (ng/ml) | 0·22 (0·11–0·52) | 1·13 (0·17–10·7)*** | 5·1 | 0·21 (0·02–4·0) |
| P f ‐CCL11 h (pg/ml) | 43·0 (20–67) | 79·0 (59–105)*** | 1·8 | 35·5 (0·5–153) |
| P f ‐FGF basic (pg/ml) | 12·0 (4·0–23·5) | 31·0 (10–70)*** | 2·6 | 12·0 (0·0–71) |
| P f ‐G‐CSF (pg/ml) | 7·0 (1·5–15·0) | 29·5 (4·8–68)*** | 4·2 | 7·0 (0·0–60) |
| P f ‐GM‐CSF (pg/ml) | 4·0 (0·14–6·3) | 14·0 (5·0–31)*** | 3·5 | 3·0 (0·0–15) |
| P f ‐IFN‐γ (pg/ml) | 13·5 (0–53) | 104 (13·5–264)*** | 7·7 | 13·5 (0·0–117) |
| P f ‐CXCL10 (pg/ml) | 601 (402–914) | 963 (711–1162)** | 1·6 | 597 (205–2238) |
| P f ‐TNF (pg/ml) | 8·0 (4·6–21) | 33 (12–84)*** | 4·1 | 8·0 (0·02–54) |
| P f ‐VEGF (pg/ml) | 2·0 (0·42–5·0) | 16·5 (6·8–29)*** | 8·3 | 1·8 (0·0–17) |
| P f ‐PDGF‐BB (pg/ml) | 2·0 (0·27–11·3) | 19·0 (3·5 −155)*** | 9·5 | 1·5 (0–117) |
| P f ‐C3bc (CAU i /ml) | 5·7 (4·8–7·6) | 7·8 (6·4–9·1)* | 1·4 | |
| P f ‐C3bc/C3 ratio (CAU i /ml/g/l) | 5·4 (4·5–7·2) | 7·0 (5·7–8·0)* | 1·3 | |
| S‐Immunoglobulin G (g/l) | 10·0 (8·5–11) | 10·9 (10–12) # | 1·1 | |
| B‐HbA1c (%) | 5·3 (5·0–5·6) | 5·5 (5·3–5·7)* | 1·1 | |
| S d ‐Triglyceride | 1·0 (0·7–1·4) | 1·2 (1·0–1·9) # | 1·2 | |
| S d ‐Total cholesterol | 5·1 (4·6–5·7) | 5·7 (5·1–6·4)* | 1·1 |
The data represent the median values and interquartile range (IQR). The data were analysed using the Wilcoxon matched‐pairs signed‐rank test on AIP cases (n = 50) versus matched controls. The P‐values are exact, two‐tailed. # P < 0·05; *P <0·01; **P < 0·001; ***P < 0·0001 on AIP cases (n = 50) versus matched controls. All P‐values < 0·001 when comparing the cytokines in the AIP cases with the 42 healthy controls. §Fold increase, AIP cases median value (n = 50)/controls median values (n = 50), $cytokine values, median and 2·5–97·5 percentiles in the 42 healthy controls. aAIP = acute intermittent porphyria; bU = urine; cPBG = porphobilinogen; dS = serum; eIL = interleukin; fP = ethylenediamine tetraacetic acid (EDTA) plasma; gIU/l = international units per litre; hCCL = chemokine ligand; iCAU/ml = complement arbitrary units per millilitre; jn.d. = not determined; kB = EDTA blood.