Lipoprotein patterns in adult cystic fibrosis: A cause for concern or marker for survival?

Worgall, Tilla S.

doi:10.1111/j.1440-1843.2010.01788.x

Cited by 2 publications

(2 citation statements)

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“…For a long time, it has been well recognized that lipoprotein and plasma triglyceride levels are altered in CF patients [36,37]. Even though reduced HDL levels are observed in pediatric CF patients, the situation for adult CF patients is not fully understood [38][39][40]. Additionally, there is increasing evidence that membrane lipid composition is also altered in association with CFTR mutations, making cells more susceptible to pathogen-induced inflammations [1,41,42].…”

Section: Discussionmentioning

confidence: 99%

Plasma Levels of the Bioactive Sphingolipid Metabolite S1P in Adult Cystic Fibrosis Patients: Potential Target for Immunonutrition?

et al. 2020

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Recent research has linked sphingolipid (SL) metabolism with cystic fibrosis transmembrane conductance regulator (CFTR) activity, affecting bioactive lipid mediator sphingosine-1-phosphate (S1P). We hypothesize that loss of CFTR function in cystic fibrosis (CF) patients influenced plasma S1P levels. Total and unbound plasma S1P levels were measured in 20 lung-transplanted adult CF patients and 20 healthy controls by mass spectrometry and enzyme-linked immunosorbent assay (ELISA). S1P levels were correlated with CFTR genotype, routine laboratory parameters, lung function and pathogen colonization, and clinical symptoms. Compared to controls, CF patients showed lower unbound plasma S1P, whereas total S1P levels did not differ. A positive correlation of total and unbound S1P levels was found in healthy controls, but not in CF patients. Higher unbound S1P levels were measured in ΔF508-homozygous compared to ΔF508-heterozygous CF patients (p = 0.038), accompanied by higher levels of HDL in ΔF508-heterozygous patients. Gastrointestinal symptoms were more common in ΔF508 heterozygotes compared to ΔF508 homozygotes. This is the first clinical study linking plasma S1P levels with CFTR function and clinical presentation in adult CF patients. Given the emerging role of immunonutrition in CF, our study might pave the way for using S1P as a novel biomarker and nutritional target in CF.

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Section: Discussionmentioning

confidence: 99%

Plasma Levels of the Bioactive Sphingolipid Metabolite S1P in Adult Cystic Fibrosis Patients: Potential Target for Immunonutrition?

et al. 2020

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“…The aetiology of this dyslipidaemia and its clinical significance remains unclear, with concerns about the potential for increased cardiovascular risks. In the accompanying editorial Worgall reflects that as normalization of the hypertriglyceridaemia is seen in patients over 40 years with CF, lower triglyceride levels may be a marker for longer survival 96 . Further studies will be needed to clarify the aetiology and risks associated with these metabolic profiles in the future.…”

Section: Cystic Fibrosismentioning

confidence: 99%