title: Hypertension and aortorenal disease in Alagille syndrome.
abstract: Alagille syndrome is a rare congenital multisystem disorder that may involve heart disease and pulmonary or peripheral artery stenosis. We report the clinical and radiological presentation of five adult patients with Alagille syndrome, hypertension and renal artery stenosis. All had systolic hypertension and a narrowing of the abdominal aorta, corresponding to a secondary midaortic syndrome. Renovascular disease progressed during follow-up, with increases in blood pressure, decreases in glomerular filtration rate and/or kidney atrophy. A literature review identified several anecdotal reports of Alagille syndrome associated with hypertension, renal artery stenosis and/or midaortic syndrome. We discuss this condition, focusing on diagnosis, differential diagnosis, associated conditions and management. Cardiologists, nephrologists and radiologists should be aware of this rare cause of renovascular hypertension and of the need for clinical, biological and echographic follow-up.
authors.affiliation: Hypertension Unit and National Referral Centre for Rare Vascular Diseases, Université Paris-Descartes, Assistance Publique Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France. joeelie.salem@gmail.com
authors.forename: Joe-Elie
authors.initials: JE
authors.lastname: Salem
authors.forename: Eric
authors.initials: E
authors.lastname: Bruguiere
authors.forename: Laurence
authors.initials: L
authors.lastname: Iserin
authors.forename: Anne
authors.initials: A
authors.lastname: Guiochon-Mantel
authors.forename: Pierre-François
authors.initials: PF
authors.lastname: Plouin
mesh_terms: D000293:Adolescent; D016738:Alagille Syndrome / Q000150:complications* / Q000503:physiopathology; D001018:Aortic Diseases / Q000150:complications* / Q000503:physiopathology; D001794:Blood Pressure; D002675:Child, Preschool; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D006973:Hypertension / Q000150:complications* / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D007674:Kidney Diseases / Q000150:complications* / Q000503:physiopathology; D008297:Male