ABSTRACT
BACKGROUND: Children with cystic fibrosis are at risk of fat-soluble vitamin deficiency. CFTR modulators positively effect nutritional status. This study aimed to assess changes in serum vitamins A, D & E after starting ETI therapy to ensure levels were not abnormally high. METHODS: Retrospective review of annual assessment data over 3½ years, before and after starting ETI in a specialist paediatric CF centre, including vitamin levels. RESULTS: 54 eligible patients were included, aged 5-15 yrs (median age 11.5). Median time to post measurements was 171 days. Median vitamin A was increased (1.38 to 1.63 µmol/L, p<0.001). Three patients (6%) had high vitamin A post-ETI, compared with none at baseline; and 2 (4%) had low levels compared to 4 (8%) at baseline. No changes in vitamins D&E. CONCLUSIONS: This study found increased vitamin A, sometimes to high levels. We recommend testing levels within 3 months of starting ETI.
Subject(s)
Cystic Fibrosis , Vitamin A , Humans , Child , Cystic Fibrosis/diagnosis , Cystic Fibrosis/drug therapy , Cystic Fibrosis/genetics , Vitamins , Cystic Fibrosis Transmembrane Conductance Regulator , Benzodioxoles , Mutation , Aminophenols/adverse effectsABSTRACT
In view of the temporal relation between elevated concentrations of glucocorticoids and prostaglandins (PG) at the time of parturition, we have examined the effects of dexamethasone on PGE2 output by mixed cell preparations from human placentae at term maintained in short-term (48 or 96 h) culture. Dexamethasone inhibited placental PGE2 output in a dose-dependent fashion. The effect on placental cells was more marked than on short-term cultures of amnion cells and was not influenced by the presence of progesterone. Dexamethasone also inhibited stimulated PGE2 output after addition of arachidonic acid. These results suggest that glucocorticoids inhibit placental PG output by a mechanism involving attenuation of PG synthase activity or expression and do not support a direct causal role for elevated maternal or fetal glucocorticoids at term on increased placental PG biosynthesis.