title: Nephro-urological complications of epidermolysis bullosa in paediatric patients.
abstract: A small but important proportion of patients with epidermolysis bullosa (EB) may develop significant renal and urological complications which can have a major impact on their morbidity and mortality. During the last 10 years, five of a large group of children with EB under our care, with either dystrophic or junctional types of disease, experienced major nephro-urological complications. Two patients with recessive dystrophic EB (REDB) developed macroscopic haematuria - one had renal failure and underwent a renal biopsy showing IgA nephropathy. A third patient with RDEB also developed renal failure and his biopsy demonstrated postinfectious glomerulonephritis/type III membranoproliferative (mesangiocapillary) glomerulonephritis. Both patients with renal failure underwent peritoneal dialysis. Two patients with junctional EB developed obstructive uropathies, which required bladder reconstruction and the fashioning of a Mitrofanoff channel in one.
authors.affiliation: Department of Dermatology, Great Ormond Street Hospital for Children, London WC1N 3JH, UK. s.chan@ich.ucl.ac.uk
authors.forename: S M H
authors.initials: SM
authors.lastname: Chan
authors.forename: M J
authors.initials: MJ
authors.lastname: Dillon
authors.forename: P G
authors.initials: PG
authors.lastname: Duffy
authors.forename: D J
authors.initials: DJ
authors.lastname: Atherton
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004820:Epidermolysis Bullosa / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D051437:Renal Insufficiency / Q000209:etiology*; D016896:Treatment Outcome; D014570:Urologic Diseases / Q000209:etiology*