Cystic fibrosis malnutrition

The causes of human copper deficiency include (1) low intake - malnutrition, total parenteral nutrition (TPN) (2) high loss - cystic fibrosis, nephrotic syndromes and (3) genetic factors — Menkes disease. Copper deficiency may also be associated with chronic malabsorption, a situation which is made much worse in cases of gastric and bowel resection. Several special diets, including powdered milk, liquid protein and standard hospital diets are a means of inducing copper deficiency. The amount of copper in US food has decreased steadily since 1942, and may be related to the rising incidence of coronary artery disease. A copper deficiency may also occur as the result of the use of chelators for other purposes for example, diethyl dithiocarbamate is an in vivo metabolite of ANTABUSE (disulfiram). 

Selenium sulfide is used as an antiseborrheic agent and as a shampoo in the treatment of Tinea versicolor. Selenite is also found in mineral supplements and is used in parenteral nutrition. However, selenium has gained undeserved popularity as a constituent of health foods and alternative tonics, perhaps because selenium deficiency has been implicated in the pathogenesis of some forms of malnutrition in children. However, even in children with selenium deficiency the benefit to harm balance has not been estabhshed. Indeed, in protein deficiency it seems to be particularly toxic. Nor is there any serious basis for its reputation as a remedy for cystic fibrosis, to prevent aging, or as a sexual stimulant. 

Short stature also occurs with several conditions that are not associated with a trne GH deficiency or insnfficiency. These conditions inclnde intranterine growth restriction constitntional growth delay malnutrition malabsorption of nntrients associated with inflammatory bowel disease, celiac disease, and cystic fibrosis chronic renal failure skeletal and cartilage dysplasia and genetic syndromes... 

Lai H, Kosorok MR, Sondel SA, et al. Growth status in children with cystic fibrosis based on the national Cystic Fibrosis Patient Registry data Evaluation of various criteria used to identify malnutrition. J Pediatr 1998 132 478 85. 

Pencharz PB, Durie PR. Pathogenesis of malnutrition in cystic fibrosis, and its treatment. CUn Nutr 2000 19 387-394. 

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). 

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