Serum Lipoprotein Concentrations in Cystic Fibrosis

Vaughan, WJ; Lindgren, Frank T.; Jb, Whalen; Abraham, S.

doi:10.1126/science.203033

Cited by 31 publications

(22 citation statements)

References 24 publications

(9 reference statements)

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“…Pencharz and Durie also found that a high-energy, high-fat diet in adults with cystic fibrosis did not increase serum lipid levels in patients with pancreatic insufficiency (38); this is of relevance to the current study in which the majority of participants exhibited malabsorption. Other studies have shown lower mean total cholesterol levels in patients with cystic fibrosis (39,40). With the exception of those few patients with pancreatic sufficiency in whom a long-term, high-fat intake may be associated with increased cardiovascular risk, the current recommendation for a high-fat diet (38,41) does not seem to result in an unfavorable metabolic profile in these patients.…”

Section: Resultsmentioning

confidence: 98%

Nutrient Status of Adults with Cystic Fibrosis

Gordon¹,

Anderson²,

Herlyn³

et al. 2007

Journal of the American Dietetic Association

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Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This crosssectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants' mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%-25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were Copyright © 2007 Significant advances in the management of cystic fibrosis (CF) have increased the lifespan for patients with this common disease (1 in 3,900 white live births) (1). Previous research has suggested that patients with cystic fibrosis who have a good nutritional status have the best chance of maintaining and improving health (1-3). Nutritional therapy itself may also contribute to a better pulmonary status (4,5). A 1992 consensus report (1) emphasized the importance of detecting malnutrition in these patients to ensure optimal therapeutic management. A 2003 expert report on bone disease in cystic fibrosis cited both malnutrition and physical inactivity as risk factors for poor bone health (6).Osteoporosis is a common health problem as patients with cystic fibrosis survive longer (6-11), and one study of patients with this disease showed that decreased bone density was more prevalent in the most malnourished patients (8). A subnormal body mass index (BMI), among other factors, can compromise bone density (6,11,12). A high fracture risk and kyphosis are also documented in these patients (12). Serum vitamin D concentrations may be an important predictor of skeletal health, but the relationship between vitamin D status and bone density remains unclear in this disease (6,9-15).Most reports on nutrition in cystic fibrosis have been reviews, or include data collected in children (16)(17)(18)(19)(20)(21)(22)(23). In this study, anthropometric, nutritional, and lifestyle va...

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Section: Resultsmentioning

confidence: 98%

Nutrient Status of Adults with Cystic Fibrosis

Gordon¹,

Anderson²,

Herlyn³

et al. 2007

Journal of the American Dietetic Association

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“…LDL and HDL are decreased in serum from CF patients, along with apoB, apoA1 and cholesterol, while VLDL and triglycerides are augmented, according to a pioneering study [44]. A decreased synthesis of apoB and apoA-I in intestinal epithelium from CF patients has recently been confirmed [45], suggesting a direct effect of CFTR dysfunction on malabsorption and/or abnormal formation of lipoproteins.…”

Section: Discussionmentioning

confidence: 94%

A Novel Lipidomic Strategy Reveals Plasma Phospholipid Signatures Associated with Respiratory Disease Severity in Cystic Fibrosis Patients

et al. 2009

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The aim of this study was to search for lipid signatures in blood plasma from cystic fibrosis (CF) patients using a novel MALDI-TOF-ClinProTools™ strategy, initially developed for protein analysis, and thin layer chromatography coupled to MALDI-TOF (TLC-MALDI). Samples from 33 CF patients and 18 healthy children were subjected to organic extraction and column chromatography separation of lipid classes. Extracts were analyzed by MALDI-TOF, ion signatures were compared by the ClinProTools™ software and by parallel statistical analyses. Relevant peaks were identified by LC-MSn. The ensemble of analyses provided 11 and 4 peaks differentially displayed in CF vs healthy and in mild vs severe patients respectively. Ten ions were significantly decreased in all patients, corresponding to 4 lysophosphatidylcholine (18∶0, 18∶2, 20∶3, and 20∶5) and 6 phosphatidylcholine (36∶5, O-38∶0, 38∶4, 38∶5, 38∶6, and P-40∶1) species. One sphingolipid, SM(d18∶0), was significantly increased in all patients. Four PC forms (36∶3, 36∶5, 38∶5, and 38∶6) were consistently downregulated in severe vs mild patients. These observations were confirmed by TLC-MALDI. These results suggest that plasma phospholipid signatures may be able to discriminate mild and severe forms of CF, and show for the first time MALDI-TOF-ClinProTools™ as a suitable methodology for the search of lipid markers in CF.

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“…Cystic fibrosis patients have decreased low‐density (LDL) and high‐density (HDL) lipoprotein cholesterol and increased plasma triglyceride levels. Such were the results of one of the earliest studies carried out on plasma of 15 children with cystic fibrosis in 1978 1 . Consecutive studies largely confirmed these results 2 .…”

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confidence: 60%

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

Worgall¹

2010

Respirology

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Serum Lipoprotein Concentrations in Cystic Fibrosis

Cited by 31 publications

References 24 publications

Nutrient Status of Adults with Cystic Fibrosis

Nutrient Status of Adults with Cystic Fibrosis

A Novel Lipidomic Strategy Reveals Plasma Phospholipid Signatures Associated with Respiratory Disease Severity in Cystic Fibrosis Patients

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

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