Vitamins and cystic fibrosis

Vitamins

Alison Morton, Sue Wolfe, Helen White and Kisten Tremlett. Jan, 2001. Vitamins [online]. Seacroft and St James's University Hospitals, Leeds, UK. Available from http://www.cysticfibrosismedicine.com

Malabsorption of fat soluble vitamins is likely in most patients with cystic fibrosis, particularly those who are pancreatic insufficient (Congdon et al, 1981). All patients have plasma levels checked annually and all receive appropriate supplements of the fat soluble vitamins A, D and E. These vitamins have been found to be deficient in early diagnosed infants (Reardon et al, 1984; Sokol et al, 1989; Marcus et al, 1991). Pancreatic sufficient patients need to be regularly monitored to ensure continuing adequacy of plasma vitamin levels. The recommended daily supplements which usually achieve normal plasma levels in infancy are vitamin A 4000 iu (120 mcg), vitamin D 400 iu (10 mcg) and vitamin E 37 - 75 iu (25 - 50 mg). The recommended doses for children over 1 year of age are vitamin A 8000 iu, vitamin D 800 iu and vitamin E 100 - 200 mg. These doses are considerably higher than the usual dietary intake.

Fasting plasma levels of the fat soluble vitamins A, D and E, total lipids, vitamin E:lipid ratio, and a prothrombin time, should be checked as part of an annual assessment. Prothrombin levels do not always correlate with plasma vitamin K levels (De Montalambert et al, 1992). A plasma sample that has only 50% of the normal concentration of prothrombin still has a normal prothrombin time (Suttie, 1992). There is increasing interest in PIVKA II (protein induced by vitamin K absence or antagonism) levels (Alexander et al, 1998). Retinol binding protein and plasma zinc levels may provide useful additional information to aid interpretation of low plasma vitamin A levels.

Vitamin A: Vitamin A deficiency may cause night blindness in older patients (Rayner et al, 1989) and can progress to severe xerophthalmia if not checked (Campbell et al, 1998). Clinical status improves when low levels of vitamin A detected at assessment are corrected (Rayner et al, 1992). When supplements are adequate and blood levels are routinely checked at annual assessment we found no ophthalmological evidence of vitamin A deficiency (Ansari et al, 1999). There is currently much interest in the role of beta carotene as an antioxidant. Hypervitaminosis A has been recognised in patients with cystic fibrosis. Care is needed in interpretation of low measured vitamin levels. Oral supplements should not be increased indiscriminately. Causes of low vitamin A levels other than malabsorption should be considered and plasma retinol binding protein and zinc levels should be measured.

Vitamin D: Vitamin D deficiency may cause rickets which is very rare (Scott et al, 1977) and osteomalacia (Friedman et al, 1985). Clinical evidence of vitamin D deficiency is rare in cystic fibrosis. Osteoporosis, osteopenia and low levels of vitamin D metabolites are being increasingly recognised in children and adults with cystic fibrosis (Stamp & Geddes, 1993; Bachrach et al, 1994, Bhudhikanok et al, 1996, Henderson et al, 1996, Conway et al, 1998).

Vitamin E: Vitamin E deficiency may cause neurological problems in older CF individuals (Sitrin et al, 1987). Correction of vitamin E deficiency improves haemoglobin levels (Kelleher et al, 1987). Vitamin E is an antioxidant and protects cell membranes from oxidative damage, because of this role vitamin E may be important in controlling the progression of lung disease in cystic fibrosis. Recent studies have suggested that cystic fibrosis patients have inadequate antioxidants defences to cope with elevated oxidant stress (Brown et al,1996). Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress and higher levels of supplementation may be required.

Vitamin K: Regular vitamin K supplements are not given unless there is chronic liver disease, a prolonged prothrombin time or a proposed surgical operation when an oral daily supplement of 5-10mg is given for a week prior to the operation, or 10 mg intramuscularly prior to the procedure. Subclinical vitamin K deficiency (as shown by elevated PIVKA II levels) is almost universal in pancreatic insufficient children with CF (Rashid et al, 1999; Alexander et al, 1998). Vitamin K is required for the formation of osteocalcin which is involved in bone metabolism. The role of subclinical vitamin K deficiency in osteopenia and osteoporosis in CF has not been fully investigated but may be very important and is being researched. In Leeds the vitamin K status is routinely assessed.

Routine supplementation of water soluble vitamins is unnecessary (Congdon et al, 1981; Peters and Rolles, 1993).

References

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Conway SP, Morton A, White H, Oldroyd B, Westmacott C, Smith AH. Bone mineral density status and risk factors for osteopenia and osteoporosis in an adult CF population. Thorax 2000; in press.

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