Subject(s)
Aortic Diseases/diagnosis , Arterial Occlusive Diseases/diagnosis , Calcinosis/diagnosis , Chromosome Aberrations , Heart Failure/etiology , Phosphoric Diester Hydrolases/genetics , Pyrophosphatases/genetics , Aortic Diseases/genetics , Arterial Occlusive Diseases/genetics , Calcinosis/genetics , Fatal Outcome , Female , Heart Failure/diagnosis , Heart Failure/physiopathology , Humans , InfantABSTRACT
BACKGROUND: Kawasaki disease (KD) is an infantile febrile illness of unknown origin characterized by clinical, laboratory and histopathologic features of systemic vasculitis. METHODS AND RESULTS: We report a 3-month-old female infant with incomplete KD who suddenly died despite intravenous immunoglobulin, aspirin, steroid and heparin treatment. Postmortem examination confirmed the echocardiographically detected giant coronary aneurysms and showed occlusive thrombosis in the giant aneurysm of the left anterior descending coronary artery, associated with neoangiogenesis, macrophage infiltration and immunostaining for tissue factor (a strong initiator of the coagulation cascade), thrombopoietin receptor and tumour necrosis factor-alpha. CONCLUSIONS: These findings show the association of angiogenesis, tumor necrosis factor-alpha and procoagulant factors, with macrophage infiltration in coronary artery aneurysms of a fatal infantile KD.
