This article aims to review the physiopathology, diagnosis and treatment of cystic fibrosis-related dyslipidemia (CFD). Bibliographic searches of the Medline and Latin American and Caribbean Health Sciences Literature databases were made (year range, 1987-2007), and the most representative papers on the theme were selected. The characteristic symptoms of CFD are hypertriglyceridemia-with or without hypocholesterolemia-and essential fatty acid deficiency. The principal CFD risk factors are pancreatic insufficiency, high-carbohydrate diet, liver diseases, inflammatory state and corticosteroid therapy. There are no specific recommendations regarding screening, which is typically performed based on the diagnosis, and at regular intervals, and more frequently in individuals belonging to high-risk groups. Treatment includes a balanced diet, micronutrient supplementation, and regular physical exercise according to individual tolerance. In the great majority of the cases, CFD-related hypertriglyceridemia does not reach values for which the use of hypolipidemic drugs is indicated. We conclude that there are few articles in the literature regarding the frequency, etiology and management of CFD. Preventive and therapeutic recommendations for hypertriglyceridemia are extrapolated from studies in individuals without cystic fibrosis. Further research is necessary to investigate the association of essential fatty acid deficiency and the physiopathology of cystic fibrosis . Since hypertriglyceridemia is an important risk factor for coronary artery disease, prospective studies will contribute for a better understanding of the natural history of this condition and define how to prevent and treat it.

Objectives:There are few studies reporting lipid profile in cystic fibrosis (CF) and most of them are in adult Caucasians. Therefore, the aim of this study was to evaluate the lipid profile of racially admixtured youths with CF.Materials and Methods:A cross-sectional survey conducted between August and September 2009 at a reference service for CF, evaluating clinical and laboratory data.Results:Forty-six patients aged from 6 years to 16 years and 2 months (median: 9 years and 10 months; 65.2% males) were evaluated. Of these, 26% were Whites, 54.4% Mulattoes and 19.6% Blacks. There were no diabetics, one patient had glucose intolerance and three had insulin resistance. Pancreatic sufficiency was present in 74% and malnutrition in 26% of the patients. The lipid profile revealed hypertriglyceridemia in 56%, hypercholesterolemia in 17.4% and hypocholesterolemia in 46.5%. In 30.4% of the patients, hypertriglyceridemia and hypocholesterolemia was observed. The serum levels of high density lipoprotein (HDL) were low in 56.5% and the low density lipoprotein (LDL) elevated in 15.2% of the patients.Conclusions:The lipid profile of this sample of Brazilian racially admixtured patients with CF showed a higher prevalence of hypertriglyceridemia and hypocholesterolemia. There was no association of dyslipidemia with the various racial groups, nutritional status, pancreatic sufficiency or glucose tolerance.

The low prevalence of carbohydrate intolerance in this population is probably a result of their young age. Another possibility is the low frequency of the ΔF508 mutation. Although not conclusive, these data suggest that in addition to age, the genotype:phenotype ratio may influence the development of glucose intolerance in patients with CF.