We investigated plasma sphingomyelin (CerPCho) and ceramide (Cer) levels in pediatric patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Plasma samples were obtained from CF (n = 19) and PCD (n = 7) patients at exacerbation, discharge, and stable periods. Healthy children (n = 17) of similar age served as control. Levels of 16–24 CerPCho and 16–24 Cer were measured by LC–MS/MS. Concentrations of all CerPCho and Cer species measured at exacerbation were significantly lower in patients with CF than PCD. 16, 18, 24 CerPCho, and 22, 24 Cer in exacerbation; 18, 24 CerPCho, and 18, 20, 22, 24 Cer at discharge; 18, 24 CerPCho and 24 Cer at stable period were significantly lower in CF patients than healthy children (p < 0.001 and p < 0.05). All CerPCho and Cer levels of PCD patients were significantly higher except 24 CerPCho and 24 Cer during exacerbation, 24 CerPCho at discharge, and 18, 22 CerPCho levels at stable period (p < 0.001 and p < 0.05) compared with healthy children. There was no significant difference among exacerbation, discharge, and stable periods in each group for Cer and CerPCho levels. This is the first study measuring plasma Cer and CerPCho levels in PCD and third study in CF patients. The dramatic difference in plasma levels of most CerPCho and Cer species found between two diseases suggest that cilia pathology in PCD and CFTR mutation in CF seem to alter sphingolipid metabolism possibly in opposite directions.

中文翻译：

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小儿血浆神经酰胺和鞘磷脂在原发性睫状运动障碍中增加，但在囊性纤维化中则减少。

我们调查了患有囊性纤维化（CF）和原发性睫状运动障碍（PCD）的小儿患者的血浆鞘磷脂（CerPCho）和神经酰胺（Cer）水平。在病情恶化，出院和稳定期从CF（n = 19）和PCD（n = 7）患者获得血浆样本。年龄相似的健康儿童（n = 17）作为对照。通过LC-MS / MS测量了16-24 CerPCho和16-24 Cer的水平。CF患者加重时测得的所有CerPCho和Cer种类的浓度均显着低于PCD。病情加重时出现16、18、24 CerPCho和22、24 Cer；放电时为18、24 CerPCho和18、20、22、24 Cer；CF患者稳定期的18、24 CerPCho和24 Cer显着低于健康儿童（p<0.001和p <0.05）。与健康儿童相比，所有PCD患者的CerPCho和Cer水平均显着升高，除了加重期的24 CerPCho和24 Cer，出院时的24 CerPCho以及稳定期的18、22 CerPCho水平（p <0.001和p <0.05）。Cer和CerPCho水平的加重，放电和稳定期之间无显着差异。这是第一项测量PCD中血浆Cer和CerPCho水平的研究，第三项针对CF患者的研究。在两种疾病之间发现的大多数CerPCho和Cer物种的血浆水平存在显着差异，表明PCD的纤毛病理和CF中的CFTR突变似乎改变了鞘脂的代谢，可能是相反的方向。