Clinical, lung function and nutritional characteristics of children and adults in the South Africa Cystic Fibrosis Registry captured in 2018#, stratified by age
| 0–6 years n=80 | 6–17 years =162 | ≥ 18 years n=171 | Total | |
| Subjects n | 80 | 162 | 171 | 413 |
| Female sex n (%) | 40 (50.0) | 87 (53.7) | 94 (55.0) | 221 (53.5) |
| Age years | n=80 | n=162 | n=171 | n=413 |
| Median (IQR) | 3.4 (2.1–5.0) | 11.3(8.8–14.6) | 26.9 (21.6–34.3) | 14.7 (7.4–24.4) |
| Ancestry** n (%) | n=80 | n=162 | n=171 | n=413 |
| Caucasian | 41 (51.3) | 102 (63.0) | 146 (85.4) | 289 (70.0) |
| Mixed | 19 (23.7) | 38 (23.4) | 24 (14.0) | 81 (19.6) |
| Black African | 20 (25.0) | 19 (11.7) | 1 (0.6) | 40 (9.7) |
| Indian | 0 (0) | 3 (1.9) | 0 (0) | 3 (0.7) |
| Pancreatic insufficient n (%) | n=80 | n=162 | n=171 | n=413 |
| Insufficient | 75 (93.8) | 144 (88.9) | 146 (85.4) | 365 (88.4) |
| Socioeconomic factors | ||||
| Household cigarette smoke**n (%) exposure/smoker** n (%) | n=80 | n=162 | n=171 | n=413 |
| Yes | 13 (16.3) | 39 (24.1) | 8 (4.7) | 60 (14.5) |
| Receiving social welfare grant n (%) | n=80 | n=162 | n=171 | n=413 |
| Yes | 15 (18.8) | 31 (19.1) | 19 (11.1) | 65 (15.7) |
| Private health insurance** n (%) | n=80 | n=162 | n=171 | n=413 |
| Yes | 40 (50.0) | 86 (53.1) | 116 (67.8) | 242 (58.6) |
| Microbiology n (%) | n=80 | n=162 | n=171 | n=413 |
| Age in years of 1st Pseudomonas aeruginosa, median (IQR) | 1 (0.0–2.0) | 4 (1.0–8.0) | 5 (1.0–17.0) | 3 (1.0–9.0) |
| Ever had P. aeruginosa** n (%) | 27 (33.8) | 58 (35.8) | 102 (59.6) | 187 (45.3) |
| Chronic P. aeruginosa** n (%) | ||||
| Yes | 8 (10.0) | 28 (17.3) | 80 (46.8) | 116 (28.1) |
| Unknown¶ | 39 (48.8) | 53 (32.7) | 56 (32.7) | 148 (35.8) |
| Chronic MSSA** n (%) | ||||
| Yes | 6 (7.5) | 40 (24.7) | 26 (15.2) | 72 (17.4) |
| Unknown¶ | 39 (48.8) | 53 (32.7) | 58 (33.9) | 150 (36.3) |
| Ever had MRSA n (%) | 2 (2.5) | 15 (9.3) | 9 (5.3) | 26 (6.3) |
| Chronic MRSA* n (%) | ||||
| Yes | 0 (0) | 7 (4.3) | 7 (4.1) | 14 (3.4) |
| Unknown¶ | 38 (47.5) | 52 (32.1) | 59 (34.5) | 149 (36.1) |
| Chronic Burkholderia cepacia* n (%) | ||||
| Yes | 0 (0) | 5 (3.1) | 8 (4.7) | 13 (3.1) |
| Unknown¶ | 38 (47.5) | 52 (32.1) | 61 (35.7) | 151 (36.6) |
| Chronic Aspergillus spp.* n (%) | ||||
| Yes | 0 (0) | 15 (9.3) | 14 (8.2) | 29 (7.0) |
| Unknown¶ | 37 (46.3) | 52 (32.1) | 58 (33.9) | 147 (35.6) |
| Chronic Haemophilus influenzae* n (%) | ||||
| Yes | 1 (1.3) | 4 (2.5) | 1 (0.6) | 6 (1.5) |
| Unknown¶ | 39 (48.8) | 54 (33.3) | 60 (35.1) | 153 (37.0) |
| Another fungus/mould* n (%) | 12 (15.0) | 26 (16.0) | 45 (26.3) | 83 (20.1) |
| Any NTM isolate n (%) | 0 (0) | 2 (1.2) | 3 (1.8) | 5 (1.2) |
| Pulmonary therapies n (%) (>3 months continuous) | n=80 | n=162 | n=171 | n=413 |
| Inhaled hypertonic saline | 45 (56.3) | 85 (52.5) | 79 (46.2) | 209 (50.6) |
| Recombinant DNase** | 9 (11.3) | 40 (24.7) | 71 (41.5) | 120 (29.1) |
| Inhaled antibiotics** | 31 (38.8) | 71 (43.8) | 120 (70.2) | 222 (53.8) |
| Low-dose azithromycin** | 47 (58.8) | 127 (78.4) | 153 (89.5) | 327 (79.2) |
| Complications/comorbidity n (%) | n=80 | n=162 | n=171 | n=413 |
| ABPA | 0 (0) | 8 (4.9) | 10 (5.8) | 18 (4.4) |
| CF-related diabetes | 0 (0) | 9 (5.6) | 53 (31.0) | 62 (15.0) |
| CF-related liver disease* | ||||
| With cirrhosis+ | 2 (2.5) | 11 (6.8) | 10 (5.8) | 23 (5.6) |
| Without cirrhosis | 5 (6.3) | 29 (17.9) | 29 (17.0) | 63 (15.3) |
| Pneumothorax | 0 (0) | 1 (0.6) | 1 (0.6) | 2 (0.5) |
| Haemoptysis major (>250 mL) | 1 (1.3) | 5 (3.1) | 1 (0.6) | 7 (1.7) |
| Occurrence of malignancy | 0 (0) | 0 (0) | 2 (1.2) | 2 (0.5) |
| Nutritional status | ||||
| WHZ (current age 0–2 years) | n=16 | — | — | n=16 |
| Median (IQR) | −0.6 (−1.4–1.2) | — | — | −0.6 (−1.4–1.2) |
| WHZ ≤1 | 7 (43.8) | — | — | 7 (43.8) |
| BMIZ (current age 2–17 years) | n=60 | n=161 | — | n=221 |
| Median (IQR) | 0.4 (−0.4–1.1) | −0.5 (−1.1–0.4) | — | −0.3 (−1.0–0.6) |
| BMIZ ≤1 | 6 (10.0) | 50 (31.1) | — | 56 (25.3) |
| BMI kg·m−2 (age ≥18 years) | n=161 | n=161 | ||
| Median (IQR) | 21.2 (19.2– 23.8) | 21.2 (19.2–23.8) | ||
| Undernutrition§,* n (%) | n=76 | n=161 | n=161 | n=398 |
| Yes | 13 (17.1) | 50 (31.1) | 28 (17.4) | 91 (22.9) |
| Lung function | — | n= 140 | n= 152 | n= 292 |
| FEV1 % pred** n (%) | ||||
| >70 | — | 107 (76.4) | 62 (40.8) | 169 (57.9) |
| 40–70 | — | 30 (21.4) | 67 (44.1) | 97 (33.2) |
| <40 | — | 3 (2.1) | 23 (15.1) | 26 (8.9) |
| FEV1 % pred** median (IQR) | — | 87.2 (71.3–102.0) | 64.6 (50.1–83.1) | 77.4 (58.1–91.8) |
| FEV1z n (%) | — | |||
| ≤ −1.0; ≥2.0 | — | 30 (21.4) | 25 (16.4) | 55 (18.8) |
| ≤ −2.0; ≥3.0 | — | 15 (10.7) | 28 (18.4) | 43 (14.7) |
| ≤ −3.0 | — | 26 (18.6) | 70 (46.1) | 96 (32.9) |
| FEV1z ** median (IQR) | — | −1.0 (−2.4–0.1) | −2.8 (−4.0– −1.4) | −1.9 (−3.4–0.7) |
BMIZ: body mass index z-score; WHZ: weight-for-height z-score; NTM: non-tuberculous mycobacterium; FEV1z: forced expiratory volume in 1 s z-score; FEV1 % pred: forced expiratory volume in 1 s per cent predicted; ABPA: allergic bronchopulmonary aspergillosis; CF: cystic fibrosis; MSSA: methicillin-sensitive S. aureus; MRSA: methicillin-resistant S. aureus. #Excludes 20 not seen in 2018, 2 with previous liver transplants and 12 with previous lung transplants. ¶Unknown: Chronic pulmonary infection status was classified as unknown if less than four respiratory samples were submitted for culture during the year or the infection status of each pathogen could not be established from past medical records. +Includes liver cirrhosis with portal hypertension. §Undernutrition definition: World Health Organization (WHO) nutritional reference equation WHZ ≤1 sd in children <2 years of age; BMI z-score (BMI kg·m−2) ≤1.0 children aged 2–17 years; and BMI <18.5 kg·m−2 in adults ≥18 years of age. *Indicates significance of differences in characteristic by age group, p<0.05; and **p<0.001.