Dislipidemia relacionada à fibrose cística

Abstract

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.

Dyslipidemia in racially admixtured children with cystic fibrosis

“…[620] In the present report, hypercholesterolemia was found in 17.4% of the patients (of whom 38% did not present pancreatic insufficiency). Of the patients without pancreatic insufficiency, only three presented hypercholesterolemia (with TC levels ranging between 171 and 179 mg/dL).…”

“…[56] The mechanisms postulated to explain this disturbance are: diet rich in carbohydrates and poor in fats, diabetes, liver disease, elevation of pro-inflammatory cytokines and use of corticosteroids, probably all acting in a combined manner. [5–7] Recently, lung transplantation was described as being associated with hyperlipidemia in CF patients, probably related to cyclosporine use.…”

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

“…Additionally, essential fatty acid deficiency may not always be present clinically with its typical deficiency symptoms, such as dermatitis and hair loss. Low linoleic acid levels have been noted to be linked to poorer pulmonary status and growth faltering in infants and children 126–129 . Low docosahexaenoic acid levels with high arachidonic acid levels can be associated with low bone mineral density in pwCF 129–132 .…”

“…Low linoleic acid levels have been noted to be linked to poorer pulmonary status and growth faltering in infants and children. [126][127][128][129] Low docosahexaenoic acid levels with high arachidonic acid levels can be associated with low bone mineral density in pwCF. [129][130][131][132] Because essential fatty acid deficiency may not present in CF with typical physical signs, those at high risk should be evaluated using a triene:tetraene ratio with dietary supplementation recommended if the ratio is elevated.…”

Bortezomib a safety treatment for patients with Multiple Myeloma and Cystic Fibrosis

“…In 2003 a diagnosis of CF was made by studying the mutations of the gene encoding the CFTR protein, which functions as a chloride channel within a number of epithelial tissues, typically involved in this disease. He showed a CFTR exocrine pancreatic insufficiency and fat malabsorption, as in 90% of adult CF patient,6 and a CF-related diabetes (40% of adults)7 treated with insulin. As CF-related bone disease he presented osteoporosis, common in adult CF patients,8 treated with calcium and biphosphonates.…”