Tuberous sclerosis with pulmonary lymphangioleiomyomatosis and renal angiomyolipomas. Computed tomographic findings: 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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Bibliographic Details
Published in:Cases journal 2009-09, Vol.2, p.9238-9238
Main Authors: Vianna, Flávia G, Marchiori, Edson, Zanetti, Gláucia, Mano, Claudia M, Sarcinelli-Luz, Branca, Carvalho, Juliana F, Assed, Carla, Santos, Isabella G, Santos, Alair A S M D, Vianna, Alberto D
Format: Article
Language:English
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Summary: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.
ISSN:1757-1626
DOI:10.1186/1757-1626-0002-0000009238