Disease Information for Acrodermatitis enteropathica: Definition

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  • Disease of Infants, usally; Hereditary sensitivity to zinc deficiency but also seen in severe zinc deficiency in most; age is usually 3 to 12 months; voluminous and steatorrheic diarrhea in kids with malnutrition, skin rash, low serum zinc levels-----------------------------------------

    Acrodermatitis Enteropathica synonyms are: AE; Brandt Syndrome ; Danbolt-Cross Syndrome; Zinc Deficiency, Congenital. Discussion: Acrodermatitis enteropathica (AE) is a disorder of zinc metabolism that occurs in one of two forms: an inborn (congenital) form and an acquired form; The inborn form of AE is a rare genetic disorder characterized by intestinal abnormalities that lead to the inability to absorb zinc from the intestine; The lack of zinc presents, characteristically, as: (1) skin inflammation with pimples (pustular dermatitis) occurring around the mouth and/or anus, (2) diarrhea, and (3) abnormal nails (nail dystrophy); In the acute phase, irritability and emotional disturbances are evident due to wasting (atrophy) of the brain cortex; It is important to recognize and treat this disorder; The acquired form of this disorder generates similar symptoms; Acquired AE sometimes results from special intravenous nutritional programs that are prepared without the appropriate amount of zinc; Supplemental zinc usually eliminates the symptoms;------------[NORD 2005]-----------------

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