Results of the paediatric questionnaire relating to PAH and LAM
| Wrong responses | |
| What are the main symptoms/manifestations that could be suggestive of PAH? | |
| Wrong option: vision abnormalities | 62% |
| All other options: grading=0 | 10 (1–62)% |
| What are the main findings that could be suggestive of PAH? | |
| Wrong option: loose shoulders | 49% |
| All other options: grading=0 | 4 (0–49)% |
| What type of pattern of lung/respiratory function abnormalities would be suggestive of PAH? | |
| Wrong option: sleep apnoea/hypopnea | 84% |
| All other options: grading=0 | 26 (1–84)% |
| What are the main symptoms/signs/manifestations that could be suggestive of LAM? | |
| Wrong option: erythema nodosum | 64% |
| All other options: grading=0 | 9 (1–64)% |
| What type of pattern of lung function test would be suggestive of LAM? | |
| Wrong option: respiratory acidosis | 81% |
| All other options: grading=0 | 11 (3–81)% |
| What type of finding at imaging studies would be suggestive of LAM? | |
| Wrong option: cerebrovascular abnormalities on brain CT | 60% |
| All other options: grading=0 | 12 (3–61)% |
Data are presented as median (min–max), unless otherwise stated. The percentage of wrong responses was calculated on valid responses (i.e. after missing and “I don’t know” responses were excluded) and is the sum of two contributions. Wrong option: responses were considered wrong when a grade >0 was attributed to the wrong option in the questionnaire. All other options: responses were considered wrong when a grade = 0 was attributed to the other options in the questionnaire. The summary figure is reported in terms of median (minimum–maximum) of the percentages of wrong responses to each option. CT: computed tomography.