Unadjusted and adjusted associations with severe lung disease in children≥6 years and adults in the South Africa Cystic Fibrosis Registry, 2018
| Severe lung disease of FEV1z ≤3.0 (n=292) | |||||
| Univariable analysis# | Multivariable analysis (n=190) | ||||
| n | Unadjusted OR (95% CI) | p-value | Adjusted OR (95% CI) | p-value | |
| Neonatal bowel obstruction (ref: no) | 275 | 0.58 (0.28–1.19) | 0.126 | NS | |
| Age diagnosis (units: 10 years) | 285 | 1.26 (0.90–1.76) | 0.180 | NS | |
| Current age (units: 10 years) | 292 | 1.99 (1.55–2.54) | <0.001* | 2.23 (1.50–3.31) | <0.001 |
| p.Phe508del (ref: neither record) | |||||
| Homozygous | 292 | 0.77 (0.37–1.57) | 0.069 | NS | |
| Heterozygous | 292 | 1.45 (0.70–3.01) | |||
| Caucasian (ref: other ancestry) | 292 | 1.50 (0.83–2.72) | 0.171 | NS | |
| Ever had P. aeruginosa (ref: no) | 279 | 4.36 (1.65–11.49) | 0.001* | 1.66 (0.34–8.12) | 0.529 |
| Time since first P. aeruginosa isolate (per 10-year interval)¶ | 133 | 2.12 (1.38–3.25) | <0.001* | 1.25 (0.85–1.84) | 0.259 |
| Chronic P. aeruginosa (ref: no) | 201 | 3.81 (2.08–6.95) | <0.001* | 1.98 (0.90–4.34) | 0.088 |
| Chronic MSSA (ref: no) | 198 | 0.57 (0.30–1.10) | 0.089 | NS | |
| Ever had MRSA (ref: no) | 261 | 2.46 (0.98–6.18) | 0.055 | NS | |
| Chronic MRSA (ref: no) | 198 | 8.88 (1.89–41.73) | 0.001* | 16.75 (1.74–161.50) | 0.015 |
| Other fungus or mould (ref: no) | 292 | 2.19 (1.22–3.93) | <0.009* | 1.31 (0.58–2.93) | 0.518 |
| ABPA (ref: no) | 261 | 0.29 (0.06–1.30) | 0.064 | NS | |
| Household cigarette smoke exposure/smoker (ref: no) | 292 | 0.62 (0.30–1.28) | 0.181 | NS | |
| CF-related diabetes (ref: no) | 286 | 3.79 (2.10–6.84) | <0.001* | 1.03 (0.46–2.33) | 0.939 |
| CF-liver disease with cirrhosis (ref: no) | 282 | 2.87 (1.15–7.14) | 0.073 | NS | |
| Undernutrition+ (ref: no) | 292 | 3.16 (1.72–5.83) | <0.001* | 5.20 (2.23–12.13) | <0.001 |
FEV1z: forced expiratory volume in 1 s z-score; MSSA: methicillin-sensitive S. aureus; MRSA: methicillin-resistant S. aureus; ABPA: allergic bronchopulmonary aspergillosis; CF: cystic fibrosis; WHZ: weight-for-height z-score; BMIZ: body mass index z-score. Severe lung disease: FEV1z ≤3 in children ≥6 years. #All variables with unadjusted p-values <0.2 in the univariable analyses are tabulated. Refer to supplementary table S3 for full set of univariable results. ¶Included as an interaction term, to apply only to those who have ever had P. aeruginosa. +Undernutrition includes: WHZ ≤1.0, <2 years of age; BMIZ ≤1.0, 2–17 years; or BMI <18.5 kg·m−2, ≥18 years. *Unadjusted p-values are shown; indicated variables had adjusted p-values <0.2 using Holm's method and were thus included in the multivariable regression model.