Nutrition Management of Patients with Epidermolysis Bullosa

Epiderrnolysis bullosa (EB), a heterogeneous group of rare, inherited disorders, is manifested by recurrent blistering of the skin induced by the slightest trauma. Little information exists regarding the nutrition management of patients with EB. This study presents information on growth, identifies potential nutrition problems, and provides guidelines for nutrition management of persons with EB. Eighty patients attending a dermatology clinic for EB patients are described. Severity of disease ranged from mild blistering of the knees, elbows, and feet to extensive blistering and scarring of the skin and entire gastrointestinal tract. Of the 18 children with EB simplex, which is a mild form of the disease, 4 (22%) were at nutritional risk. None of the 13 adults with EB simplex were underweight and 8 (62%) were overweight. Of the patients with the more severe forms of EB, 27 of the 35 (77%) children with dystrophic EB and 4 of the 7 (57%) children with junctional EB were at risk for malnutrition. Of the 7 adults with dystrophic EB, 6 (86%) were underweight. Common nutrition problems included protein-energy malnutrition, chewing and swallowing problems, constipation, anemia, and vitamin/mineral deficiencies. When nutrition care protocols address these problems, growth, development, and nutritional status can improve. For those with severe nutrition problems, gastrostomy feeding or similar nutrition therapies should be considered. J Am Diet Assoc. 1995; 95:575-579.     

Fish and Amphibian Embryos -- A Model System for Evaluating Teratogenicity

Fish and Amphibian Embryos—A Model System for EvaluatingTeratogenicity. Birge, W.J., Black, J.A., Westerman, A.G. andRamey, B.A. (1983). Fundam. Appl. Toxicol. 3: 237–242.Developmental stages of fish and amphibians were used to evaluatereproductive impairments produced by environmental contaminants.Using static-renewal and flow-through procedures, exposure wasinitiated at, or soon after, fertilization and maintained through4 days posthatching. Trace concentrations of many environmentaltoxicants produced high frequencies of mortality and teratogenesis.When developmental stages of the trout were exposed to inorganicmercury, 10% control-adjusted mortality was observed at 1 µg/Land frequencies of terata in hatched populations ranged from8% to 43% for exposure concentrations of 2 to 25 µg/L-In similar tests with the narrow-mouthed toad, 45% mortalityoccurred when mercury was administered at 1 µg/L; frequenciesof teratogenesis ranged from 8% to 17% at exposure concentrationsof 1 to 5 µg/L; and complete mortality occurred at 7 µg/L.Boron-induced teratogenesis in trout ranged from 5% at 1 µg/Lto 51% at higher exposure levels. When catfish embryos wereexposed to atrazine at 0.06, 4.8, and 46.7 mg/L, terata appearedin surviving populations at frequencies of 4%, 69%, and 100%,respectively. Fish and amphibian developmental stages constitutesimple and effective models for 1) investigating mechanismsof teratogenesis, 2) evaluating the impact of environmentaltoxicants on aquatic biota, and 3) identifying environmentalteratogens which may be of concern to human health.