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Cardiofaciocutaneous syndrome with KRAS gene mutation presenting as chylopericardium
Shogo Akahoshi1, Akinori Hirano2, Hiroki Nagamine1
1Department of Cardiology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Abstract:
A 12-year-old female patient with cardiofaciocutaneous syndrome in the presence of a KRAS gene mutation had episodes of pericardial effusion on ultrasound, later confirmed to be chylopericardium, which resolved after a lymphangiography. We discussed herein the pathophysiological background of this rare case and the efficacy of lymphangiography in the treatment of chylopericardium.
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