Reduced absorption and enhanced synthesis of cholesterol in patients with cystic fibrosis: a preliminary study of plasma sterols
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Monica Gelzo
und
Giuseppe Castaldo
Abstract
Background:
Low cholesterol is typically observed in the plasma of patients with cystic fibrosis (CF) contrasting with the subcellular accumulation of cholesterol demonstrated in CF cells and in mice models. However, the homeostasis of cholesterol has not been well investigated in patients with CF.
Methods:
We studied the plasma of 26 patients with CF and 33 unaffected controls campesterol and β-sitosterol as markers of intestinal absorption and lathosterol as a marker of de novo cholesterol biosynthesis by gas chromatography (GC-FID and GC-MS).
Results:
Plasma campesterol and β-sitosterol results were significantly (p=0.01) lower while plasma lathosterol was significantly higher (p=0.001) in patients with CF as compared to control subjects. Plasma cholesterol results were significantly lower (p=0.01) in CF patients.
Conclusions:
Our data suggest that the impaired intestinal absorption of exogenous sterols in patients with CF stimulates the endogenous synthesis of cholesterol, but the levels of total cholesterol in plasma remain lower. This may be due to the CFTR dysfunction that reduces cholesterol blood excretion causing the accumulation of cholesterol in liver cells and in other tissues contributing to trigger CF chronic inflammation.
Acknowledgments
Grants from Regione Campania (DGRC 28/2/14) and by POR Campania (FSE 2007-13, project CREME) are gratefully acknowledged.
Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
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©2016 Walter de Gruyter GmbH, Berlin/Boston
Artikel in diesem Heft
- Frontmatter
- Editorials
- The Theranos phenomenon, scientific transparency and freedom of speech
- Holotranscobalamin: in the middle of difficultly lies opportunity
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- Mini Review
- Quantitative nucleic acid amplification by digital PCR for clinical viral diagnostics
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- Reduced absorption and enhanced synthesis of cholesterol in patients with cystic fibrosis: a preliminary study of plasma sterols
- An International Standard for holotranscobalamin (holoTC): international collaborative study to assign a holoTC value to the International Standard for vitamin B12 and serum folate
- A technical and clinical evaluation of a new assay for inhibin A and its use in second trimester Down syndrome screening
- Investigation on the ability of first trimester glycodelin and angiopoietin-2 to predict small-for-gestational age pregnancies at delivery
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- Congress Abstracts
- 5th Slovenian Congress of Clinical Chemistry and Laboratory Medicine
Artikel in diesem Heft
- Frontmatter
- Editorials
- The Theranos phenomenon, scientific transparency and freedom of speech
- Holotranscobalamin: in the middle of difficultly lies opportunity
- Review
- Laboratory and clinical risk assessment to treat myelodysplatic syndromes
- Mini Review
- Quantitative nucleic acid amplification by digital PCR for clinical viral diagnostics
- Genetics and Molecular Diagnostics
- Hybrid minigene splicing assay verified the pathogenicity of a novel splice site variant in the dystrophin gene of a Chinese patient with typical Duchenne muscular dystrophy phenotype
- General Clinical Chemistry and Laboratory Medicine
- Prospective validation of an automated chemiluminescence-based assay of renin and aldosterone for the work-up of arterial hypertension
- Sex steroid hormone stability in serum tubes with and without separator gels
- Reduced absorption and enhanced synthesis of cholesterol in patients with cystic fibrosis: a preliminary study of plasma sterols
- An International Standard for holotranscobalamin (holoTC): international collaborative study to assign a holoTC value to the International Standard for vitamin B12 and serum folate
- A technical and clinical evaluation of a new assay for inhibin A and its use in second trimester Down syndrome screening
- Investigation on the ability of first trimester glycodelin and angiopoietin-2 to predict small-for-gestational age pregnancies at delivery
- Plasma total C-terminal agrin fragment (tCAF) as a marker for kidney function in patients with chronic kidney disease
- Hematology and Coagulation
- Thirty-minutes’ exposure to smartphone call triggers neutrophil activation in vitro
- Performance of the XN-2000 WPC channel-flagging to differentiate reactive and neoplastic leukocytosis
- Differences in lupus anticoagulant final conclusion through clotting time or Rosner index for mixing test interpretation
- Reference Values and Biological Variations
- Derivation of level-specific reference change values (RCV) from a health screening database and optimization of their thresholds based on clinical utility
- Cancer Diagnosis
- BRAF analysis before surgery for papillary thyroid carcinoma: correlation with clinicopathological features and prognosis in a single-institution prospective experience
- Letters to the Editor
- Significant increase of serum prostate-specific antigen after exercise
- Serum delipidation but not high-speed centrifugation is effective in clearing lipemia interference in serum lipase activity measurement
- A relationship between absolute monocyte count and C-reactive protein in patients with migraine undergoing no pharmacological therapy
- Validation of the “Vacutainer® urinalysis preservative plus urine tube” for the determination of albumin and protein
- Performance evaluation of a novel automated HIV Ag/Ab chemiluminescence immunoassay
- SLC26A4 genotypes associated with enlarged vestibular aqueduct malformation in south Italian children with sensorineural hearing loss
- Is cystatin C level altered in women with polycystic ovary syndrome?
- Venous thromboembolism in a patient with persistent inhibitor to coagulation factor V – a case report
- Free light chains nephelometric assay: human urine stability in different storage conditions
- Comparing the viscoelastomeric fibrin polymerization assays FIBTEM® (ROTEM) vs. Functional Fibrinogen® (TEG): or why is a higher threshold for fibrinogen substitution better than a lower one?
- Reply to: Comparing the visco-elastomeric fibrin polymerization assays FIBTEM® (ROTEM) vs. Functional Fibrinogen® (TEG): or why is a higher threshold for fibrinogen substitution better than a lower one? By Schöchl et al.
- Congress Abstracts
- 5th Slovenian Congress of Clinical Chemistry and Laboratory Medicine
