TABLE 3

Ratings of clinical feature contribution to supporting a “clinical presentation of CF” (n=54 responses)

Not individually supportiveSomewhat supportiveStrongly supportiveTotal (weighted)
Clinical features
 Pancreatic insufficiency0 (0)13 (24.07)41 (75.93)#142.5
 Infertility/CBAVD0 (0)13 (24.07)41 (75.93)#142.5
 Bronchiectasis – diffuse1 (1.85)22 (40.74)31 (57.41)#126
 Radiographic pancreatic fibrosis1 (1.85)33 (61.11)20 (37.04)109.5
 Daily sputum production6 (11.11)38 (70.37)10 (18.52)87
 Aquagenic wrinkling16 (29.63)24 (44.44)14 (25.93)78
 Frequent need for antibiotics for chest10 (18.52)36 (66.67)8 (14.81)78
 Vitamin A/E deficiency10 (18.52)38 (70.37)6 (11.11)75
 Nasal polyposis14 (25.93)32 (59.26)8 (14.81)72
 ABPA diagnosis13 (24.07)38 (70.37)3 (5.56)66
 Bronchiectasis – asymmetrical19 (35.19)27 (50)8 (14.81)64.5
 Radiographic rhinosinusitis15 (27.78)36 (66.67)3 (5.56)63
 Liver disease/steatosis/cirrhosis29 (53.7)4 (7.41)55.5
 Obstructive spirometry20 (37.04)32 (59.26)2 (3.7)54
 Osteoporosis/osteopenia32 (59.26)20 (37.04)2 (3.7)36
 Constipation37 (68.52)14 (25.93)3 (5.56)30
 Vitamin D deficiency37 (68.52)15 (27.78)2 (3.7)28.5
Airway microbiology
Burkholderia cepacia complex2 (3.7)14 (25.93)38 (70.37)#135
Pseudomonas aeruginosa23 (42.59)30 (55.56)#124.5
Stenotrophomonas maltophilia7 (12.96)31 (57.41)16 (29.63)94.5
Mycobacterium abscessus sp.7 (12.96)32 (59.26)15 (27.78)93
Achromobacter species9 (16.67)30 (55.56)15 (27.78)90
 MRSA12 (22.22)33 (61.11)9 (16.67)76.5
 MSSA12 (22.22)36 (66.67)6 (11.11)72
Mycobacterium avium complex12 (22.22)37 (68.52)5 (9.26)70.5
Aspergillus fumigatus sp.22 (40.74)31 (57.41)1 (1.85)49.5
Streptococcus pneumoniae48 (88.89)6 (11.11)0 (0)9

Data are presented as n (%), unless otherwise stated. Total score calculated on a basis of 0, 1.5 and 3 points allocated for each count of “Not supportive”, “Somewhat supportive” and “Strongly supportive”, respectively. CF: cystic fibrosis; CBAVD: congenital bilateral absence of the vas deferens; ABPA: allergic bronchopulmonary aspergillosis; MRSA: methicillin-resistant Staphylococcus aureus; MSSA: methicillin-sensitive S. aureus. #: responses with ≥50% selection as “Strongly supportive” of need for follow-up at a specialist CF centre.