Vitamin status in treated patients with cystic fibrosis.

Abstract

SUMMARY The water-soluble (BI, B2, B6, C, folic acid) and fat-soluble vitamin (A, carotene, E, and D) status of 36 patients with cystic fibrosis was assessed and compared with a control group of 21 age-matched normal children. Twenty-seven of the patients were receiving vitamin supplements (except folic acid and vitamin E) at the time of investigation. Vitamin BI, B2, and B6 status was adequate in all patients, and there was little evidence of folic acid deficiency. Vitamin C stores might not have been adequate in some of these patients, despite daily supplements with 50 mg of the vitamin. Steatorrhoea, often severe, was present in most of them. Serum carotene and vitamin E concentrations were low in over 90 % of patients and were related to the severity of steatorrhoea. Vitamin A was low in over 40% of the patients despite daily vitamin supplements of 4000 IU and correlated with the serum retinol-binding protein level. Serum 25-OH cholecalciferol was low in some patients whether or not they were receiving a daily supplement of 400 IU vitamin D. In a short-term supplementation trial with water-miscible preparations of vitamins A and E in 14 patients, the serum levels of both vitamins responded well to 2 weeks of treatment with 50 mg vitamin E and 4000 IU vitamin A. Except for serum vitamin A, which was lowest in patients with the poorest clinical grading, the other vitamins were not influenced by the clinical grade of the patients.

Prevalence of vitamin K deficiency in cystic fibrosis

“…The study was approved by the ethics review boards of the participating hospitals. On the basis of the characteristic clinical features and abnormal Prevalence of vitamin K deficiency in cystic fibrosis [1][2][3] Mohsin Rashid, Peter Durie, Maureen Andrew, Daina Kalnins, Janey Shin, Mary Corey, Elizabeth Tullis, and Paul B Pencharz sweat test results, all 98 patients were known to have CF. Fecal fat balance studies (72 h) had been done routinely on these patients to assess the presence and degree of steatorrhea.…”

“…Patients with cystic fibrosis (CF) are at risk of developing deficiencies of fat-soluble vitamins (A, D, E, and K) because of pancreatic insufficiency, hepatobiliary disease, or both (1,2). Vitamin K is widely distributed in many foods (vitamin K 1 , or phylloquinone) and is also produced by intestinal bacteria (vitamin K 2 , or menaquinone).…”

Vitamin therapy in cystic fibrosis–a review and rationale

“…Levels have been measured in one study (2), in which most children received vitamin C as part of their multivitamin regime. Mean leucocyte vitamin C levels were similar to controls but the range was wider with more CF children in the 'low normal' range; levels were unrelated to dinical score or, in fact, to supplementation.…”

“…Although clinical fat-soluble vitamin deficiency states have been recognized for almost as long as CF (I), many vitamin supplements have clearly been shown to be unnecessary (2). There has been a tendency though for across-the-board multi-vitamin supplements to continue.…”

Fat-soluble vitamins in cystic fibrosis

“…The vitamin status of thirty-six older patients in Leeds was investigated in 1981 in order to assess the adequacy of conventional vitamin supplementation and the effect of short-term supplementation with water-miscible forms of vitamin A and E (Congden et al 1981). At that time patients were not receiving vitamin E supplements and serum vitamin E levels were low in 90% of patients and inversely correlated with the severity of the steatorrhoea, but responded well to treatment with 50 mg water-miscible vitamin E daily for 2 weeks.…”