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Nutritional outcome in children with severe generalized recessive dystrophic epidermolysis bullosa: a short‐ and long‐term evaluation of gastrostomy and enteral feeding
Author(s) -
Colomb V.,
BourdonLannoy E.,
Lambe C.,
Sauvat F.,
Hadj Rabia S.,
Teillac D.,
De Prost Y.,
Bodemer C.
Publication year - 2012
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2011.10592.x
Subject(s) - medicine , enteral administration , malnutrition , gastrostomy , pediatrics , epidermolysis bullosa , parenteral nutrition , head circumference , surgery , dermatology , birth weight , pregnancy , biology , genetics
Summary Background Generalized recessive dystrophic epidermolysis bullosa (RDEB) is often complicated by high nutritional difficulties with risks of malnutrition. Objectives 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 ± 5·8 years), and one young man aged 18 years. 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 ± 20 months), 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 10 years. Skin was not improved. Conclusion 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.