Computed tomographic findings of tuberous sclerosis with pulmonary lymphangioleiomyomatosis and renal angiomyolipomas: a case report

The authors describe a case of a 31-year-old female with tuberous sclerosis, a genetic, rare, variably expressed disease. Clinical symptoms were chest pain, and progressive dyspnea. Computed tomography scan of the chest showed bilateral, diffuse, small thin-walled cysts scattered throughout the lung...

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Detalles Bibliográficos
Autores principales: Vianna, Flavia Gavinho, Marchiori, Edson, Zanetti, Glaucia, Mano, Claudia Mauro, Sarcinelli-Luz, Branca, Carvalho, Juliana Franca, Assed, Carla, Santos, Isabella Guedes, Santos, Alair Augusto, Vianna, Alberto Domingues
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2009
Materias:
Research article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2827144/
https://www.ncbi.nlm.nih.gov/pubmed/20184711
http://dx.doi.org/10.4076/1757-1626-2-9238
Descripción
Sumario:The authors describe a case of a 31-year-old female with tuberous sclerosis, a genetic, rare, variably expressed disease. Clinical symptoms were chest pain, and progressive dyspnea. Computed tomography scan of the chest showed bilateral, diffuse, small thin-walled cysts scattered throughout the lungs characteristic for pulmonary lymphangioleiomyomatosis. Computed tomography scan of the abdomen revealed enlarged, heterogeneous kidneys, with low density tumors corresponding to angiomyolipomas. Pulmonary lymphangioleiomyomatosis and bilateral renal angiomyolipomas are some presentations of tuberous sclerosis and the coexistence of both conditions may cause devastating morbidity and mortality.

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