[Secondary glomerulonephritis in neurofibromatosis type 1. Two case reports].
Síndrome nefrótico secundario en neurofibromatosis tipo 1. A propósito de dos casos.
Journal
Anales del sistema sanitario de Navarra
ISSN: 2340-3527
Titre abrégé: An Sist Sanit Navar
Pays: Spain
ID NLM: 9710381
Informations de publication
Date de publication:
05 Dec 2019
05 Dec 2019
Historique:
entrez:
21
12
2019
pubmed:
21
12
2019
medline:
1
7
2020
Statut:
epublish
Résumé
Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder with systemic clinical manifestations. There are few publications about the renal effects of this disease, with renal vascular disease and adrenal tumors being the most frequent forms of renal involvement, while cases describing glomerular effects are exceptional. Despite the lack of published information, common molecular mechanisms in both NF-1 and nephrotic syndrome, involving the mTOR pathway, were suggested to explain a possible association between both pathologies. We present two cases of renal involvement in the form of nephrotic syndrome in patients diagnosed with NF1. A 41-year-old female was diagnosed of NF-1 in the context of a nephrotic syndrome with resistance to steroid treatment; the renal biopsy revealed a diagnosis of minimal changes disease. The second case is other 71-year-old woman with a history of NF-1, who presented a nephrotic syndrome and secondary renal amyloidosis.
Types de publication
Case Reports
Langues
spa
Sous-ensembles de citation
IM