Death of a Patient with Turner Syndrome Caused by Arotic Dissection and Rupture after Hemodialysis / 대한신장학회지

ABSTRACT

The main cause of death in hemodialysis patients is cardiovascular disease. Aortic dissection with rupture is one of the most life threatening cardiovascular diseases. The risk of developing aortic dissection can be increased by renal failure or hemodialysis and the morbidity also increases in Turner syndrome. Here, we report a case of Turner syndrome patient who died from ruptured aortic dissection after hemodialysis, and review relevant literature. A 59 year-old female presented to the emergency department complaining of severe dyspnea and general weakness. Six months ago, she had undergone an operation at another hospital due to horseshoe kidney and staghorn calculus. After the operation, acute renal failure developed, so she received hemodialysis. The chest x-ray image taken at the time of admission showed mediastinal widening compared to image taken 6 months ago. Aortic dissection was diagnosed by chest computer tomograph. Chromosomal analysis was performed and the study revealed the mosaicism of Turner syndrome (45,X/ 46,XX). The genetic defect, renal failure and especially hemodialysis were thought as important risk factors in the development of aortic dissection in this patient. Although storongly advised to have emergency operation, the patient only wanted conservative management. Two months later, the patient died from aorta rupture.