Plasma Ceramides and Sphingomyelins of Pediatric Patients Increase in Primary Ciliary Dyskinesia but Decrease in Cystic Fibrosis.

Publisher: Wiley Subscription Services, Inc Country of Publication: United States NLM ID: 0060450 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1558-9307 (Electronic) Linking ISSN: 00244201 NLM ISO Abbreviation: Lipids Subsets: MEDLINE

Publication: 2018- : Hoboken, NJ : Wiley Subscription Services, Inc.
Original Publication: Chicago, American Oil Chemists' Society.

Ceramides/*blood ; Ciliary Motility Disorders/*blood ; Ciliary Motility Disorders/*genetics ; Cystic Fibrosis/*blood ; Sphingomyelins/*blood; Adolescent ; Case-Control Studies ; Child ; Chromatography, Liquid ; Cystic Fibrosis/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Female ; Humans ; Male ; Microtubule-Associated Proteins/genetics ; Mutation ; Prospective Studies ; Tandem Mass Spectrometry

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.
(© 2020 AOCS.)

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THD-2016-12638 International Scientific Research Projects Coordination Unit of Hacettepe University

Keywords: Ceramide; Cystic fibrosis; Inflammation; Primary ciliary dyskinesia; Sphingolipid; Sphingomyelin

0 (CFTR protein, human)
0 (Ceramides)
0 (DNAAF3 protein, human)
0 (Microtubule-Associated Proteins)
0 (Sphingomyelins)
126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator)

Date Created: 20200303 Date Completed: 20210609 Latest Revision: 20210609

20221213

10.1002/lipd.12230

32120452