Cardiovascular Manifestations of Erdheim-Chester's Disease: A Case Series | Zendy

Isabela Bispo Santos da Silva Costa | Zendy; André Abdo | Zendy; Cristina Salvadori Bittar | Zendy; Silvia Moulin Ribeiro Fonseca | Zendy; Aline Sabrina Holanda Teixeira Moraes | Zendy; Roberto Kalil Filho | Zendy; Juliana Pereira | Zendy; Ludhmila Abrahão Hajjar | Zendy

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Cardiovascular Manifestations of Erdheim-Chester's Disease: A Case Series

Author(s) -

Isabela Bispo Santos da Silva Costa,

André Abdo,

Cristina Salvadori Bittar,

Silvia Moulin Ribeiro Fonseca,

Aline Sabrina Holanda Teixeira Moraes,

Roberto Kalil Filho,

Juliana Pereira,

Ludhmila Abrahão Hajjar

Publication year - 2018

Publication title -

arquivos brasileiros de cardiologia

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.4

H-Index - 53

eISSN - 1678-4170

pISSN - 0066-782X

DOI - 10.5935/abc.20180218

Subject(s) - medicine , erdheim–chester disease , diabetes insipidus , genitourinary system , presentation (obstetrics) , disease , osteosclerosis , histiocytosis , pathology , pediatrics , dermatology , surgery

Erdheim-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.

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