Abstract

Freier S, Faber J, Goldstein R, Mayer M. Treatment of acrodermatitis enteropathica by intravenous amino acid hydrolysate. J Pediatr 1973;82:109-12. Freier et al described an infant who developed failure to thrive, a severe rash, and alopecia, as well as profound diarrhea. A sibling had died following a similar course. The diagnosis of congenital acrodermatitis enteropathica was made. She was treated initially with diiodohydroxyquinoline. She was then treated with intravenous amino acids and eventually a protein hydrolysate. Her skin and diarrhea improved with the treatment. These were considered standard treatment at the time. In retrospect, there must have been trace amounts of zinc in the hydrolysate. The infant described likely had the congenital form of acrodermatitis enteropathica, an autosomal-recessive trait involving a mutation in the zinc transporter gene. This has been localized to the SLC39A4 gene on chromosome 8. It is a rare condition, with an estimated prevalence of 1-9 per million. Acrodermatitis enteropathica was first described in 1936, but the role of zinc in treating acrodermatitis enteropathica was not recognized at the time of this article’s publication. It was an often-fatal condition. Human zinc deficiency was first described in the 1970 by Prasad—he described short patients with hypogonadism. Around the same time, parenteral nutrition increasingly was being used to support infants with severe gastrointestinal disorders. The initial solutions were often free of trace metals. There were soon increasing reports of severe rashes in infants receiving zinc-free total parenteral nutrition. It also was noted that zinc losses paralleled intestinal losses. Today, we are much more likely to see acquired acrodermatitis enteropathica. The advent of total parenteral nutrition heralded an increase in previously unrecognized nutrient deficiencies. The 2 most common were zinc and essential fatty acid deficiency; however, reports of copper, chromium, iodine, and biotin deficiency followed. There was increased recognition of the importance of trace nutrients in human health. Zinc deficiency may occur because of decreased intake, increased needs, malabsorption, increased losses, or impaired use. It may be primary or secondary, usually due to poor intake or chronic diarrhea. Recent pharmaceutical shortages have aggravated the possibility of deficiencies. Infants on prolonged exclusive breastfeeding may suffer deficiencies, and patients with prolonged diarrhea and/or severely restricted diets are also at risk.1Prasad A.S. Impact of the discovery of human zinc deficiency on health.J Am Coll Nutr. 2009; 28: 257-265Crossref Scopus (209) Google Scholar

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