RENOVASCULAR HYPERTENSION AND VASCULAR ANOMALIES IN ALAGILLE-SYNDROME

Alagille syndrome (AS) is characterized by the association of at least three of the following five abnormalities: chronic cholestasis, perip heral pulmonary artery stenosis, vertebral arch defects, embryotoxon, and typical facies. In addition to urological abnormalities, tubuloint erstitial nephritis, renal tubular acidosis, and mesangiolipidosis hav e been noted in AS, The usual manifestations of such renal pathologies rarely include hypertension. We report five patients with at least fo ur of the five major features of AS who developed secondary hypertensi on of renovascular origin 3.5-28 years after the initial diagnosis of AS. Angiography demonstrated uni- or bilateral renal artery stenosis a nd various other abnormalities of the main arteries in all five patien ts: aorta (3 cases), celiac artery (4 cases), superior mesenteric arte ry (1 case), subclavian artery (1 case). Our findings underscore the v alue of arterial blood pressure monitoring in patients with AS. If hyp ertension occurs, a renovascular origin should be sought, The diffuse vascular abnormalities which appeared to be a feature of AS in these p atients should prompt larger studies of vascular abnormalities in AS.