1988
DOI: 10.1001/archderm.124.5.746
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Gastrointestinal manifestations of epidermolysis bullosa in children

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Cited by 15 publications
(31 citation statements)
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“…In particular, children with RDEB experienced GOR very frequently (43 ⁄57, 75%) and two of these were additionally found to have a hiatus hernia. 8,10,11 Nissen's fundoplication, although providing some relief in three of these children initially, did not appear to have lasting benefit. GOR was also particularly problematic for a quarter of children with Dowling-Meara EBS and was felt to have contributed to FTT in infancy in some of these patients.…”
Section: Discussionmentioning
confidence: 75%
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“…In particular, children with RDEB experienced GOR very frequently (43 ⁄57, 75%) and two of these were additionally found to have a hiatus hernia. 8,10,11 Nissen's fundoplication, although providing some relief in three of these children initially, did not appear to have lasting benefit. GOR was also particularly problematic for a quarter of children with Dowling-Meara EBS and was felt to have contributed to FTT in infancy in some of these patients.…”
Section: Discussionmentioning
confidence: 75%
“…Kindler syndrome is an autosomal recessive genodermatosis sharing many clinical features with EB, notably skin fragility, resulting from defects of actin-cytoskeleton adhesion via focal contacts as a result of mutations in the kindlin-1 gene (Table 1). 3 A number of gastrointestinal (GI) complications have been described in EB, notably the occurrence of oral mucosal involvement, 4-6 oesophageal strictures 5,7-12 and constipation 5,7,8,10,11,13 which may cause considerable morbidity for this patient group. Other features of gastrointestinal disease, including gastro-oesophageal reflux (GOR), failure to thrive (FTT; defined as weight loss across two centiles on a growth chart), protein-losing enteropathy (PLE) and diarrhoea have not been well described in EB.…”
Section: Discussionmentioning
confidence: 99%
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“…A relationship between RDEB and congenital megacolon or mucoinous cystadenoma has not previously been reported yet, so further evaluation is needed. Among all the types of epidermolysis bullosa, only patients with RDEB develop clinically apparent esophageal involvement (7,8), which is common among them, with an incidence of 6% (9). The most common site of involvement of the esophagus is the upper third (50%) followed by the lower third (25%); in 25% of cases, multiple sites are involved (7,8).…”
Section: Discussionmentioning
confidence: 99%