Author/Authors :
Babaoğlu, Kadir Kocaeli University - Faculty of Medicine - Department of Pediatric Cardiology, Turkey , Uyan, Zeynep Seda Kocaeli University - Faculty of Medicine - Department of Pediatric Cardiology, Turkey , Binnetoğlu, Köksal Kocaeli University - Faculty of Medicine - Department of Pediatric Cardiology, Turkey , Yıldız, Cenk Eray Istanbul University - Institute of Cardiology - Department of Cardiovascular Surgery, Turkey , Sarper, Nazan Kocaeli University - Faculty of Medicine - Department of Pediatric Hematology, Turkey
Abstract :
Wiskott- Aldrich Syndrome (WAS) is a recessive genetic disorder linked to the X-chromosome characterized by immune deficiency, eczema and thrombocytopenia. To the best of our knowledge, a few cases of vasculitis or aneurysmal formation have been reported in this syndrome, but the association has not been well established. We report a patient with WAS and extensive aortitis causing severe aneurysmal dilatation in the everywhere of the aorta who underwent successful first stage operation involving replacement of ascending aorta.
