title: Nutritional outcome in children with severe generalized recessive dystrophic epidermolysis bullosa: a short- and long-term evaluation of gastrostomy and enteral feeding.
abstract: BACKGROUND
  Generalized recessive dystrophic epidermolysis bullosa (RDEB) is often complicated by high nutritional difficulties with risks of malnutrition.
  
  
  OBJECTIVE
  To provide information regarding the benefits of enteral feeding by gastrostomy (GTF), energy and protein requirements, tolerance, growth and pubertal development in children with RDEB.
  
  
  METHODS
  Twenty-four patients were referred over a 7-year period in a retrospective study. Gastrostomy placement was decided in patients unable to feed orally and/or presenting loss in weight and height of at least 1 SD compared with their best growth level, despite regular nutritional advice. Weight and height were expressed as Z-scores. Catch-up growth following GTF onset was studied.
  
  
  RESULTS
  Gastrostomies were performed in 11 children (aged 9·0±5·8years), and one young man aged 18years. The body weight Z-score was -2·3±1·0, height Z-score 1·1±1·1, weight-for-height was 81±11% and height-for-age 95± 4%. At onset, GTF provided 74±21% and 180±81% of the recommended dietary allowance (RDA) for energy and proteins, respectively. At study update (53±20months), GTF provided 91±29% and 205±100% of RDA for energy and proteins, respectively. Weight-for-height reached 92±15% and height-for-age 98±5%. A normal puberty was obtained when GT was performed before the age of 10years. Skin was not improved.
  
  
  CONCLUSIONS
  Malnutrition was observed in 50% of the children with generalized RDEB. Protein and energy needs are particularly high. GTF is well tolerated and helps with catch-up growth and puberty. It must be considered before malnutrition onset, and, if necessary, before puberty.
authors: Department of Paediatric Gastroenterology and Nutrition, French Reference Centre for Rare Digestive Diseases, Nutrition Support Team, Department of Dermatology, French Reference Centre of Genetic Skin disorders (MAGEC) and Department of Paediatric Surgery, Necker-Enfants Malades Hospital, AP-HP, University René Descartes Paris V, 149 rue de Sèvres, 75015 Paris, France.
authors: V
authors: V
authors: Colomb
authors: E
authors: E
authors: Bourdon-Lannoy
authors: C
authors: C
authors: Lambe
authors: F
authors: F
authors: Sauvat
authors: S
authors: S
authors: Hadj Rabia
authors: D
authors: D
authors: Teillac
authors: Y
authors: Y
authors: De Prost
authors: C
authors: C
authors: Bodemer
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002149:Energy Intake; D004750:Enteral Nutrition / Q000379:methods*; D016108:Epidermolysis Bullosa Dystrophica / Q000628:therapy*; D005260:Female; D005774:Gastrostomy / Q000379:methods*; D006130:Growth Disorders / Q000628:therapy; D006801:Humans; D008297:Male; D009752:Nutritional Status; D017060:Patient Satisfaction; D011788:Quality of Life; D012189:Retrospective Studies; D016896:Treatment Outcome; D015430:Weight Gain