DEFICIENCY OF VITAMIN-E AND VITAMIN-A IN CYSTIC-FIBROSIS IS INDEPENDENT OF PANCREATIC FUNCTION AND CURRENT ENZYME AND VITAMIN SUPPLEMENTATION

The aim of this study was to evaluate to what extent serum vitamins A and E in cystic fibrosis are affected by the underlying disease, pancr eatic sufficiency or insufficiency, meconium ileus, nutritional status , age and treatment (enzyme and vitamin supplementation). Serum vitami n A and E levels were determined by high performance liquid chromatogr aphy in 210 cystic fibrosis patients, subdivided according to clinical condition into four subgroups (unsupplemented pancreatic insufficienc y, supplemented meconium ileus, pancreatic sufficiency, supplemented p ancreatic insufficiency) and compared with 42 control subjects. Vitami n A and E levels were generally lower in cystic fibrobsis patients tha n in controls (P < 0.002 and P < 0.001 respectively). Subjects with pa ncreatic insufficiency regularly receiving enzyme and vitamin suppleme ntation had significantly lower vitamin A (P < 0.05) and vitamin E (P < 0.01) levels than controls. In subjects with pancreatic sufficiency only vitamin A was significantly lower than in controls (P < 0.01). Vi tamin levels were not age-dependent in cystic fibrosis, and no signifi cant correlation with standardized body weight (Z-score) was observed. Conclusion Cystic fibrosis patients show a clear tendency to vitamin A and E deficiency, irrespective of pancreatic function body weight an d standardized supplementation with pancreatic extract and liposoluble vitamins. Since the clinical significance of this deficiency is still not clear, longitudinal studies of cystic fibrosis patients with and without adequate vitamin supplementation are required.