SILVIA MOULIN RIBEIRO FONSECA
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Instituto do Câncer do Estado de São Paulo, Hospital das Clínicas, Faculdade de Medicina - Médico
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- Cardiovascular Manifestations of Erdheim-Chester's Disease: A Case Series(2018) COSTA, Isabela Bispo Santos da Silva; ABDO, Andre Neder Ramires; BITTAR, Cristina Salvadori; FONSECA, Silvia Moulin Ribeiro; MORAES, Aline Sabrina Holanda Teixeira; KALIL FILHO, Roberto; PEREIRA, Juliana; HAJJAR, Ludhmila AbrahaoErdheim-Chester Disease is a rare entity, classified as an inflammatory myeloid neoplasm, with an unknown incidence, occurring preferentially in men after 50 years of age. Classically, it has a multisystemic presentation, with the skeletal system being the most frequently affected (90% of the patients), followed by genitourinary involvement in 60% of cases and central nervous system in the pituitary and diabetes insipidus in 25% of the cases. Cardiovascular manifestations are present in more than half of the patients, with aortic infiltration and atrial pseudotumor being the most common forms.