Middle aortic syndrome in a boy with arteriohepatic dysplasia (Alagille syndrome).
Middle aortic syndrome is increasingly recognized as a cause of hypertension in adolescents and young adults. Alagille syndrome is a genetic disorder characterized by hepatic, eye, and bony abnormalities. Cardiovascular lesions occur in most of the patients, but narrowing of the abdominal aorta has not been previously recorded. We present an unusual association between middle aortic syndrome and Alagille syndrome, with a similar vascular lesion seen in two generations.
Shefler
A G
AG
Department of Pediatric Cardiology, John Radcliffe Hospital, Oxford OX3 9DU, UK.
Chan
M K
MK
Ostman-Smith
I
I
D000293:Adolescent; D000328:Adult; D016738:Alagille Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D001012:Aorta, Abdominal / Q000002:abnormalities / Q000601:surgery; D001017:Aortic Coarctation / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D007621:Karyotyping; D008297:Male; D012729:Sex Chromosome Aberrations / Q000175:diagnosis / Q000235:genetics*; D014680:Veins / Q000637:transplantation