Dyslipidemia in racially admixtured children with cystic fibrosis

Abstract

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.

Cystic fibrosis rabbits develop spontaneous hepatobiliary lesions and CF-associated liver disease (CFLD)-like phenotypes

“…While many CF rabbits developed hypertriglycemia, a common lipid disorder in CF patients, we note that the cholesterol profiles of the CF rabbits differ from those of human patients. In CF patients, the frequency of hypercholesterolemia (<20%) is much lower than that of hypocholesterolemia (up to 50%) ( 62 ). In CF rabbits, however, the majority are associated with elevated cholesterol levels.…”

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

“…[138][139][140][141][142][143][144][145] The levels of HDL-c and triglycerides did not correlate with the severity of genotype, pancreatic status, diabetes, and dietary fat intake. [140][141][142]145,146 However, HDL-c values were lower in adolescents with CF than in those of younger children with CF. 133 Adult males with CF are also more likely to have HDL-c below the recommended minimum compared with adult females with CF.…”

“…Conventional evaluation of lipids in children and adults with CF have revealed changes associated with increased CVD risk, including a significantly lower concentration of high‐density lipoprotein cholesterol (HDL‐c) and higher triglycerides compared with controls or population means 138–145 . The levels of HDL‐c and triglycerides did not correlate with the severity of genotype, pancreatic status, diabetes, and dietary fat intake 140–142,145,146 . However, HDL‐c values were lower in adolescents with CF than in those of younger children with CF 133 .…”