Renal and Lung Cysts in Birt-Hogg-Dubé Syndrome: A Continuum of the Same Disorder.
birt-hogg-dubé
lung cysts
pneumothorax
renal cell carcinoma
renal cysts
syndrome
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
Oct 2021
Oct 2021
Historique:
accepted:
18
10
2021
entrez:
22
11
2021
pubmed:
23
11
2021
medline:
23
11
2021
Statut:
epublish
Résumé
Birt-Hogg-Dubé (BHD) syndrome is a rare autosomal-dominant disorder, affecting multiple organs, mostly the skin, lungs, and kidneys. The prevalence of BHD syndrome is difficult to define given the rarity of the disease. Patients present most often with primary spontaneous pneumothorax. Renal tumors are a characteristic finding in BHD, and are often bilateral and multifocal and of the chromophobe and oncocytoma variant. Very scarce reports have highlighted the presence of simple renal cysts, as the only phenotypical renal manifestation, in BHD patients. Herein, we highlight two novel cases of bilateral multiple renal and pelvic cysts, in two females with genetically proven BHD syndrome, doubting a potential association with BHD syndrome.
Identifiants
pubmed: 34804727
doi: 10.7759/cureus.18878
pmc: PMC8599113
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e18878Informations de copyright
Copyright © 2021, Degheili et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
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