Fig 1.

Initial nNO in primary immunodeficiency, PCD and healthy controls were compared using a Kruskal-Wallis test with post-hoc pairwise Mann-Whitney U tests. Horizontal lines represent the group median. Dashed line represents the PCD diagnostic cut-off of 77 nL/min. ****p < 0.0001. Only two PCD subjects (both with pathogenic variants in RSPH1) had initial or repeat nNO values above the PCD diagnostic cutoff of 77 nL/min (solid black horizontal line). This is consistent with previous observations that pathogenic variants in RSPH1 can sometimes result in nNO measurements above the diagnostic cutoff [29]. Two PID subjects initially had nNO measurements below the diagnostic cutoff of 77nL/min. Both of these subjects eventually had nNO measurements that rose above 77 nL/min, though one subject with RAG1 immunodeficiency had repeat nNO values fall below this cutoff.