Uncontrolled hypertension in a child with Hurler syndrome

Abstract

A 15-year-old girl with Hurler syndrome was evaluated for recurrent hypertensive episodes with peak systolic pressures in excess of 175mmHg. CTangiography demonstrates post-ductal long-segment aortic stenosis and beading of the descending aorta extending to the iliac bifurcation (Figs. 1 and 2). Hurler syndrome is a mucopolysaccharidosis secondary to a deficit of lysosomal enzyme, alpha-L-iduronidase sulfate with characteristic skeletal, visceral, neurological and cardiovascular anomalies [1]. Hypertension has been described in Hurler syndrome in up to 32% of patients [2]. Mechanisms suggested are congenital or acquired architectural anomalies, atherosclerotic aortoiliac occlusive disease and