Cystic fibrosis vitamin supplementation

Nutrient malabsorption also occurs in the genetic disease cystic fibrosis. This disease arises from a mutation in the chloride ion transporter, and results in pancreatic insufficiency as well a.s respiratory difficulties. Treatment of the malabsorption symptoms of cystic fibrosis involves supplementation with pancreatic enzymes and synthetic water-soluble versions of the fat-soluble vitamins. The labored breathing and respiratory infections due to the disease cannot be treated by dietary intervention. 

Cystic fibrosis is the most common lethal autosomal-recessive disease, in which oxidative stress takes place at the airway surface [274]. This disease is characterized by chronic infection and inflammation. Enhanced free radical formation in cystic fibrosis has been shown as early as 1989 [275] and was confirmed in many following studies (see references in Ref. [274]). Contemporary studies also confirm the importance of oxidative stress in the development of cystic fibrosis. Ciabattoni et al. [276] demonstrated the enhanced in vivo lipid peroxidation and platelet activation in this disease. These authors found that urinary excretion of the products of nonenzymatic lipid peroxidation PGF2 and TXB2 was significantly higher in cystic fibrotic patients than in control subjects. It is of importance that vitamin E supplementation resulted in the reduction of the levels of these products of peroxidation. Exhaled ethane, a noninvasive marker of oxidative stress, has also been shown to increase in cystic fibrosis patients [277]. 

Exocrine pancreatic insufficiency is most commonly caused by cystic fibrosis, chronic pancreatitis, or pancreatic resection. When secretion of pancreatic enzymes falls below 10% of normal, fat and protein digestion is impaired and can lead to steatorrhea, azotorrhea, vitamin malabsorption, and weight loss. Pancreatic enzyme supplements, which contain a mixture of amylase, lipase, and proteases, are the mainstay of treatment for pancreatic enzyme insufficiency. Two major types of preparations in use are pancreatin and pancrelipase. Pancreatin is an alcohol-derived extract of hog pancreas with relatively low concentrations of lipase and proteolytic enzymes, whereas pancrelipase is an enriched preparation. On a per-weight basis, pancrelipase has approximately 12 times the lipolytic activity and more than 4 times the proteolytic activity of pancreatin. Consequently, pancreatin is no longer in common clinical use. Only pancrelipase is discussed here. 

Blood plasma of children with cystic fibrosis was found to have decreased TAC (by 16%) in spite of increased concentrations of ascorbic acid, uric acid, and thiol groups (L4). In another study TAC of children with cystic fibrosis was normal, but these children received vitamin supplementation in doses prescribed in international guidelines (a-tocopherol <10 years, 100 mg daily, and >10 years, 200 mg daily retinol 2.5 mg daily ascorbic acid 100-200 mg daily) (M2). Other authors found TAC values for nonhospitalized patients (1.40 0.20 mM) not different from laboratory control values (1.35 0.11 mM), but greater than values for hospitalized patients (1.09 0.17 mM). TAC in CF children correlated positively with anthropometric values (height, weight, body mass index) and pulmonary function (forced expiratory volume in 1 sec), but not with age (L3). 

A pharmacological role for vitamin E may exist in claudication arising from peripheral vascular disease. Studies with small numbers of patients having cystic fibrosis, glucose-6-phosphate dehydrogenase deficiency, and sickle cell anemia conditions associated with decreased erythrocyte half-lives showed that many had chemical evidence of vitamin E deficiency. Administration of vitamin E supplements (400-800 lU/d) significantly increased red cell survival time. Claims that doses of vitamin E 10-20 times the RDA are beneficial for treatment of skin disorders, fibrocystic breast disease, sexual dysfunction, cancer, baldness, and other disorders have not been substantiated. 

The answer is a. (Murray, pp 627-661. Scriver, pp 3897-3964. Sack, pp 121-138. Wilson, pp 287-320.) Vitamins A, D, E, and K are all fat-soluble. The physical characteristics of fat-soluble vitamins derive from the hydrophobic nature of the aliphatic chains composing them. The other vitamins listed are water-soluble, efficiently administered orally, and rapidly absorbed from the intestine. Fat-soluble vitamins must be administered intramuscularly or as oral emulsions (mixtures of oil and water). In intestinal disorders such as chronic diarrhea or malabsorption due to deficient digestive enzymes, fat-soluble vitamins are poorly absorbed and can become deficient. Supplementation of fat-soluble vitamins is thus routine in disorders like cystic fibrosis (219700), a cause of respiratory and intestinal disease that is the likely diagnosis in this child. 

BekerLT, Ahrens RA,FinkRJ,etal. Effect of vitamin K1 supplementation on vitamin K stams in cystic fibrosis patients. J Pediatr Gastroenterol Nutr 1997 24 512-517. 

Rovner, A. J., Stallings, V. A., Schall, J. I., Leonard, M. B., Zemel, B. S. 2007. Vitamin D insufficiency in children, adolescents, and young adults with cystic fibrosis despite routine oral supplementation. Am J Clin Nutr 86 1694—9. 

Stored ester reserves. It should be noted that there are reports indicating that continued dietary supplementation of alcoholic cirrhosis (Mobarhan et al.y 1981) or cystic fibrosis (Fulton et al, 1982) patients with vitamin A in high doses does not always cause a sustained rise in plasma levels, though it may correct abnormalities in dark adaptation. These findings indicate that in these subjects with diseased livers, an impaired RBP synthetic rate is a major contributing factor to low circulating levels of retinol. Furthermore, alcohol per se has been shown in baboons and rats to increase the rate at which retinol is catabolized by hepatic tissue (Sato and Lieber, 1981) and some data from rats suggest that alcohol may potentiate the sensitivity of tissues to vitamin A, even in the presence of normal blood levels (Leo and Lieber, 1982). 

Among humans, zinc supplementation of children with protein-energy malnutrition (PEM) was reported not to alter the response in vitamin A and RBP levels immediately following admission to a hospital, but was necessary to sustain higher plasma levels throughout the recovery period. Studies in children with cystic fibrosis and in normal adult humans with moderately depressed or adequate serum zinc levels failed to demonstrate a plasma retinol response to zinc supplementation (Palin et aL, 1979 Garry and Visconti, 1980). 

Plasma vitamin A and RBP levels have been investigated in patients with cystic fibrosis of the pancreas (Smith et al., 1972 Knopfle et al., 1975 Palin et al, 1979). Plasma vitamin A and RBP levels have been found to be lower than normal in patients with cystic fibrosis, despite the administration of oral vitamin A supplements adequate to maintain normal hepatic stores. In one study (Smith et al., 1972), the plasma vitamin A transport system was studied in 43 patients with cystic fibrosis receiving oral supplements of vitamin A and in 95 normal control subjects of a similar age range. The mean plasma concentrations of vitamin A and RBP were significantly lower in the patients than in the controls. Moreover, in cystic fibrosis patients each component of the transport system failed to show the normal age-related rise. It is not known whether these abnormalities of the retinol transport system are primary or secondary features of cystic fibrosis the abnormalities may, however, play a role in the pathophysiology of the disease. 

The effect of vitamin D supplementation on the frequency of vitamin D deficiency, respiratory outcomes and vitamin D toxicity in patients with cystic fibrosis has been assessed. No adverse events were reported [72 ]. 

Ferguson ]H, Chang AB. Vitamin D supplementation for cystic fibrosis. Cochrane Database Syst Rev April 18,2012 4 CD007298. 

The studies referred to above use LDL in an in vitro assay, but the ultimate antioxidant test is whether these compounds work in animals or humans. There are very few examples of an in vivo antioxidant action of a carotenoid in humans. The best recent examples come from studies with children suffering from cystic fibrosis who are known to have relatively low levels of carotenoids in their serum. These patients are supplemented with large amounts of vitamin E, but still show significantly elevated levels of malondialdehyde in their serum. Two groups have supplemented patients with this disease with p-carotene, and in each case, they reported a decrease in the malondialdehyde levels [27, 20] as well as increased resistance of LDL to oxidant stress [27]. 

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