Table 1.
Comparison of clinical features of pancreatic insufficient CF patients (excluding MZ twins) in the twin/sibling study group with euglycaemia, hyperglycaemia or diabetes
| Characteristic | Euglycaemia | Hyperglycaemia or possible diabetes | Diabetes |
|---|---|---|---|
| Number of participants | 509 | 387 | 102 |
| Mean age (years) | 12.5h | 15.9h | 23.6 |
| White | 476/509 (94%) | 353/387 (91%) | 96/102 (94%) |
| Female | 237/509 (47%) | 175/387 (45%) | 55/102 (54%) |
| CFTR genotype | |||
| ΔF508×2a | 273/507 (54%) | 218/386 (56%) | 59/100 (59%) |
| Severe CFTR×2b | 443/489 (91%) | 356/369 (95%) | 88/93 (95%) |
| Lung function percentilec | |||
| Cross-sectional | 0.73 (n=391)h | 0.67 (n=357)h | 0.55 (n=98) |
| Longitudinal | 0.63 (n=253)h | 0.58 (n=299)f | 0.51 (n=94) |
| Nutritional statusd | |||
| Cross-sectional | −0.08 (n=500)h | −0.49 (n=387)e | −0.76 (n = 100) |
| Longitudinal | −0.04 (n=492)h | −0.29 (n=382) | −0.47 (n=94) |
| Steroid treatment (≥30 days/year) | 90/509 (18%)h | 78/387 (20%)h | 46/102 (45%) |
| Steroid treatment (days/year) | 19 (n = 304)h | 31 (n=268)h | 80 (n = 59) |
| Positive test for Pseudomonas aeruginosa | 344/462 (74%)h | 310/366 (85%)g | 90/92 (98%) |
| Meconium ileus | 85/444 (19%)e | 76/342 (22%)f | 8/86 (9%) |
| Lung transplantation | 1/451 (0.2%)h | 4/361 (l%)g | 9/89 (10%) |
a
Homozygous for ΔF508
b
Both CFTR alleles with severe (e.g. frameshift or nonsense) mutations (list available upon request)
c
Cross-sectional and longitudinal measures of lung function were based on forced expiratory volume in 1 s as defined previously [19]
d
Nutritional status was assayed by body mass index standard deviation score (BMI SDS), with longitudinal averages as defined previously [23]. Cross-sectional BMI SDS was calculated by averaging over the year prior to enrolment
e
p<0.05,
f
p<0.01,
g
p<0.001,
h
p<0.000l vs diabetes