A) Phospholipid classes of the control groups (healthy (n = 11), left column of pair; bronchitis (n = 10), right column of pair).

<p>Lipid class composition is expressed as % of the analyzed displayed lipid classes (left graph). B) Phosphatidylcholine species composition is expressed as % phosphatidylcholine (right graph). The species of the other phospholipid classes are displayed in the <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0117985#pone.0117985.s002" target="_blank">S2 Fig</a>. Data are means. Species present at an abundance of < 0.5% were not displayed.</p

Display of results at the disease category level.

<p>A) Phosphatidylcholine, the major surfactant phospholipid class and B) its species dipalmitoylphosphatidylcholine (PC 32:0) are given as individual results of all patients included in the study according the disease category they belong to. The statistical comparisons were done by ANOVA and Dunn’s post hoc test; all significant results are displayed in each figure. To take multiple comparisons into consideration, an overall cut-off level of P<0.0182 was calculated. For A) 1-way ANOVA gave a P = 0.0020, for B) P < 0.0038; the significant results of Dunn’s post hoc tests are indicated as following: ____ = P < 0.05, _ _ _ = P < 0.01, and …. = P < 0.001. The species of the other phosphatidylcholines, the other phospholipid classes and their species composition are displayed in <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0117985#pone.0117985.s003" target="_blank">S3 Fig</a>. Horizontal bar indicates median.</p

Display of results at the individual diagnosis level for the disease category “ILD-related to the alveolar surfactant region”.

<p>Lipid class composition (graph A) on left side) and phosphatidylcholine species composition (graph B) on right side) are indicated as means for each diagnosis. The number of subjects is detailed in <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0117985#pone.0117985.t001" target="_blank">Table 1</a>. The numbers above the columns indicate the mean phospholipid concentrations (μmol/l). No statistical comparisons were done; the graphical display should allow a rapid identification of deviations from the controls. The other species are provided in <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0117985#pone.0117985.s004" target="_blank">S4 Fig</a>. Only lipid species present at an abundance of > 0.5% are displayed.</p

Calculation and expression of the results of the lipid analysis.

<p>All analyzed lipids are the sum of all analyzed phospholipid classes, cholesteryl ester and free cholesterol (all expressed as nmol/ml). Phospholipid classes are expressed as % of all analyzed phospholipids and the species as percentage of that phospholipid class. In the body of the manuscript mainly the results of all lipid classes and the composition of one of its classes, i.e. phosphatidylcholine, are indicated. Composition of the other classes are presented in the supplemental materials. For overview see also panel A in <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0117985#pone.0117985.s001" target="_blank">S1 Fig</a>.</p

Display of results at the individual diagnosis level “ABCA3 transporter deficiency, two mutations/Controls-healthy” (panel A) and B)) and “Controls-bronchitis/Controls-healthy” (panel C) and panel D)).

<p>A) and C) Phosphatidylcholine and B) and D) Phosphatidylglycerol species of the children with ABCA3 transporter deficiency due to two disease causing mutations (n = 5) or controls-bronchitis (n = 10) in relation to that of the control children (n = 11). Mean values of each group were taken for calculation of the ratio. A ratio below 1 indicates lower values in the children with ABCA3 transporter deficiency, i.e. lack of transport of such species into the alveolar space; a ratio above 1 indicates higher values in the children with ABCA3 transporter deficiency, i.e. accumulation of such species in the alveolar space.</p

Subjects included into the study, their allocation to pulmonary disease categories and subcategories, subjects excluded and final number of subjects.

<p>Subjects included into the study, their allocation to pulmonary disease categories and subcategories, subjects excluded and final number of subjects.</p