Nutrition in dystrophic epidermolysis bullosa.
In dystrophic epidermolysis bullosa (EB), a combination of diminished food intake and increased nutritional requirements commonly leads to malnourishment. Adequate nutrition in these patients could provide many benefits, including improved growth, accelerated healing, decreased susceptibility to infection, and enhanced well-being. We assessed nutrition status of children with dystrophic EB, and evaluated the benefits of nutritional advice in those who were considered malnourished. The majority of patients had inadequate intakes of a wide spectrum of nutrients, including those who appeared to be relatively mildly affected. We were unable to secure persisting substantial improvements in dietary intake despite thorough counseling. Adequate nutrition in patients with dystrophic EB depends on active nutritional support starting from birth. The need for more invasive techniques of nourishment, such as nasogastric and gastrostomy feeding, may have to be considered earlier than is currently the case.
Allman
S
S
Dietetic Department, Hospital for Sick Children, London, UK.
Haynes
L
L
MacKinnon
P
P
Atherton
D J
DJ
D001827:Body Height; D001835:Body Weight; D002648:Child; D015362:Child Nutrition Disorders / Q000097:blood / Q000188:drug therapy / Q000209:etiology*; D002675:Child, Preschool; D002149:Energy Intake; D016108:Epidermolysis Bullosa Dystrophica / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007223:Infant; D007501:Iron / Q000097:blood / Q000627:therapeutic use; D008297:Male; D009751:Nutritional Requirements; D014815:Vitamins / Q000627:therapeutic use; D015032:Zinc / Q000097:blood / Q000627:therapeutic use
D014815:Vitamins; D007501:Iron; D015032:Zinc