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Renal and Lung Cysts in Birt-Hogg-Dubé Syndrome: A Continuum of the Same Disorder
- Source :
- Cureus
- Publication Year :
- 2021
- Publisher :
- Cureus, Inc., 2021.
-
Abstract
- 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.
- Subjects :
- renal cell carcinoma
Pathology
medicine.medical_specialty
Pulmonology
pneumothorax
business.industry
Urology
General Engineering
Chromophobe cell
Disease
syndrome
urologic and male genital diseases
medicine.disease
Birt–Hogg–Dubé syndrome
lung cysts
renal cysts
Pneumothorax
Nephrology
Renal cysts
Renal cell carcinoma
medicine
birt-hogg-dubé
Oncocytoma
business
Lung cysts
Subjects
Details
- ISSN :
- 21688184
- Database :
- OpenAIRE
- Journal :
- Cureus
- Accession number :
- edsair.doi.dedup.....368a125ed9f52880e923b3b7b4482cd8