Nephro-urological complications of epidermolysis bullosa in paediatric patients.
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.
Chan
S M H
SM
Department of Dermatology, Great Ormond Street Hospital for Children, London WC1N 3JH, UK. s.chan@ich.ucl.ac.uk
Dillon
M J
MJ
Duffy
P G
PG
Atherton
D J
DJ
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*