Reference values for ICM
| Diagnosis | Reference | N | Rt,basal (Ω cm2) | ΔIsc,amiloride (μA/cm2) | ΔIsc,carbachol (μA/cm2) | ΔIsc,cAMP/forskolin (μA/cm2) | ΔIsc,histamine (μA/cm2) |
| PI-CF | 17 | 11 | 26.7±3* | −7.0±2* | −12.5 (n=6)/2.6 (n=2) | −3.2±2* (n=2) | |
| 18 | 42 | −7.1±2* | |||||
| 30 | 49 | −6.7±9 (−47/9) | |||||
| 31 | 51 | −8.7±11 (−17/5) | −5.3±10 (−15/5) | −5.0±10 (−14/6) | |||
| 20 | 55 | 27 | 3.9±3 (n=40) | ||||
| 22 | 32 | −2.5 (−16/8) | −2 (−22/5) | ||||
| Present study | 34 | 22.5±4 | −6.6±7 (−33/0) | −0.7±7 (−18/9) | 1.9±2 (−1/6) | −2.0±5 (−19/5) | |
| PS-CF | 18 | 9 | 2.6±3* | ||||
| 30 | 11 | 0.7±8 (−12/15) | |||||
| Present study | 7 | 26.2±7 | −5.5±6 (−17/0) | 4.2±4 (0/10) | 9.8±6 (0/19) | 5.6±5 (0/12) | |
| CBAVD | 32 | 21 | CF, no residual (n=4) CF, low residual (n=1) CF, high residual (n=6) Inconclusive (n=1) Normal (n=9) | ||||
| Pancreatitis | 33 | 7 | 34.8±9 (25/50) | ||||
| Heterozygotes | 30 | 22 | 38.7±26 (11/115) | ||||
| Healthy controls | 17 | 11 | 30.0±5* | −7.1±3* (n=10) | 26.7±4* | 12.6±4* (n=5) | |
| 18 | 47 | 35.1±3* | |||||
| 31 | 50 | −8.7±11 (−18/5) | 38.5±23 (13/66) | 33.0±26 (11/60) | |||
| 20 | 61 | 43.3±18 | 39.3±19 | ||||
| 34 | 22 | 23.9±9 | −5.2±9 | 29.9±14 | 17.4±15 | 29.8±22 | |
| Present study | 28 | 24.9±7 | −5.4±8 (−38/0) | 36.7±18 (13/97) | 16.6±14 (5/54) | 29.3±18 (5/85) |
All experiments from the references have been performed with the same ICM set-ups, registration mode and protocol by the CF centres in Rotterdam and Hannover, as described in the Methods section and in detail in the online supplement.
Mean±SD (range: lower/upper limit) are given unless otherwise indicated.
↵* SEM.
CBAVD, congenital bilateral absence of the vas deferens; CF, cystic fibrosis; ICM, intestinal current measurement; Isc, short-circuit current; PI, pancreatic-insufficient; PS, pancreatic-sufficient; Rt, transepithelial resistance.