Nephro-urological complications of epidermolysis bullosa in paediatric patients

Autor: M.J. Dillon, P.G. Duffy, D.J. Atherton, S.M.H. Chan
Rok vydání: 2007
Předmět:
Zdroj: British Journal of Dermatology. 156:143-147
ISSN: 1365-2133
0007-0963
Popis: 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.
Databáze: OpenAIRE