Description
- Cystic Fibrosis is an inherited disease of the exocrine glands. It affects the
pancreas, respiratory system, intestines, liver and sweat glands. Patients experience
chronic respiratory infections, pancreatic insufficiency, and increased electrolytes
in sweat. The pancreatic insufficiency leads to nutrient deficiencies, which can
further impair the bodys ability to combat the respiratory infections. 1
Causes
- Cystic fibrosis is a genetic disease. It is inherited as an autosomal recessive
trait. 2
At Risk
- Children of parents who both carry the recessive gene have a 25% chance of inheriting
this disease.
Prevention and Management
General:
- Currently there is no way to cure cystic fibrosis. Medical interventions are
intended to help the patient lead as normal a life as possible.
Nutritional Influences:
- Obstruction of the pancreatic ducts results in a deficiency of trypsin, amylase
and lipase which prevents the conversion and absorption of fat and protein. This
interferes with the absorption of fat soluble vitamins, A, D, E and K. Diets should
include supplements of these vitamins. 3
- One study suggests that cystic fibrosis patients have inadequate antioxidant
defenses to cope with the increased oxidative stress that these patients regularly
experience. 4
- Efficient antioxidant supplementation may decrease lung inflammation in cystic
fibrosis. 5
- Supplementation may be necessary to maintain normal levels of beta carotene.
The observed increased levels of lipid peroxidation may be due to low levels of
beta-carotene.6 These low levels may be increased with supplementation.7
- Patients with malabsorption syndromes may develop vitamin E deficiencies and
tailored treatment may include vitamin E supplements.8 Patients
supplemented with at least 100 mg of vitamin E had normal concentrations of vitamin
E in their erythrocytes. Patients that were unsupplemented or who received amounts
of vitamin E less than 100 mg had levels below normal.9
Additional Information
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Abstracts
Lepage G, Champagne J, Ronco N, Lamarre A, Osberg I, Sokol RJ, Roy CC. Supplementation
with carotenoids corrects increased lipid peroxidation in children with cystic
fibrosis. Am J Clin Nutr 1996 Jul;64(1):87-93. Evidence of lipid peroxidation
previously documented in cystic fibrosis (CF) implies an imbalance between free
radical generation and antioxidant defense mechanisms. The aim of the present
study was to examine the relation between plasma concentrations of malondialdehyde,
a marker of lipid peroxidation, and the exogenous antioxidant line of defense.
Malondialdehyde concentrations (90.2 +/- 4.7 nmol/L) in 25 children with CF aged
9.6 +/- 0.8 y were higher (P < 0.001) than concentrations (69.1 +/- 2.6 nmol/L)
in 17 children used as control subjects and were not correlated with any marker
of disease severity. In contrast with their all-rac-alpha-tocopherol status,
which was normal as a result of routine supplementation with a 200-mg dose of
all-rac-alpha-tocopheryl acetate/d, beta-carotene was very low. A 2-mo open trial
in which 12 children with CF aged 11.5 +/- 0.8 y were given 4.42 mg (8.23 mumol)
beta-carotene three times per day led to normalization of the malondialdehyde
concentration in all but 1 patient, in conjunction with an increase of plasma beta carotene from 0.08 +/- 0.03 to 3.99 +/- 0.92 mumol/L. Their plasma concentrations
were inversely correlated (r = -0.54, P = 0.006) [corrected] with malondialdehyde
when the values measured pre- and posttreatment were pooled. We conclude that
beta-carotene deficiency contributes to lipid peroxidation in CF and that supplementation
may eventually prove to be a useful adjunct for the management of the disease.
References
1 Tabers Cyclopedic Medical Dictionary. 16th ed. Philadelphia:FA Davis Company;
1985. p 120.
2 Diseases. 2nd ed. Springhouse (PA):Springhouse Corporation; 1993. p 898.
3 Diseases. 2nd ed. Springhouse (PA):Springhouse Corporation; 1993. p 882.
4 Brown RK et al. Pulmonary dysfunction in cystic fibrosis is associated
with oxidative stress. EurRespir J 1996 Feb;9(2):334-9.
5 Winklhofer-Roob BM, Response to oral beta-carotene supplementation in
patients with cystic fibrosis: a 16-month follow-up study. Acta Paediatr Oct;84(10)1132-6.
6 Winklhofer-Roob BM et al. Neutrophil elastase/alpha 1-proteinase inhibitor
complex levels decrease in plasma of cystic fibrosis patients during long-term oral
beta-carotene supplementation. Pediartr Res 1996 Jul;40(1):130-4.
7 Lepage G, et al., Suplementation with carotenoids corrects increased lipid
peroxidation in children with cystic firosis. Am J Clin Nutr 1996 Jul;64(1):87-93.
8 Tanyel MC; Mancano LD, Neurologic findings in vitamin E dificiency. Am
Fam Physician 1997 Jan;55(1):197-201.
9 Perters SA; Kelly FJ, Vitamin E supplementation in cystic fibrosis. J
Pediatr Gastroenterol Nutr 1996 May;22(4):341-5.
