[Diffuse arterial calcified elastopathy. A case report].
Diffuse arterial calcified elastopathy is a very rare and little known hereditary disease, characterized by diffuse calcifications of the arterial wall. It seems common in North Africa and in the Caucasian region. Its incidence appears to be underestimated in Morocco. Clinical pattern is dominated by renovascular hypertension often associated with symptoms of heart failure. Risk of sudden death from myocardial infarction is particularly important. Thus, the diagnosis of diffuse arterial calcified elastopathy must always be suspected in front of an apparently unexplained heart failure or renovascular hypertension occurring in an infant. We report a case of diffuse arterial calcified elastopathy discovered in a neonatal intensive care unit, during management of a cardiogenic shock in a 3-months old infant. This observation demonstrates the importance of systematic measurement of the arterial tension, family screening and the impact of the ultrasound in the detection of vascular calcifications. Treatment remains essentially symptomatic.
Unité de réanimation néonatale, centre national de référence en néonatologie et nutrition, hôpital d'Enfants de Rabat, Bloc S 18, secteur 17 Hay-Riad, Rabat, Morocco. asmae-zakaria@hotmail.fr
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A
Zakaria
M
M
Kabiri
M
M
Kabbaj
A
A
Barkat
N
N
Lamdouar Bouazzaoui
D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000235:genetics*; D002114:Calcinosis / Q000175:diagnosis / Q000235:genetics*; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000235:genetics; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000235:genetics*; D003937:Diagnosis, Differential; D004452:Echocardiography; D004547:Elastic Tissue / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006978:Hypertension, Renovascular / Q000175:diagnosis / Q000235:genetics; D007223:Infant; D009018:Morocco; D009397:Nephrocalcinosis / Q000175:diagnosis / Q000235:genetics; D012770:Shock, Cardiogenic / Q000175:diagnosis / Q000235:genetics*; D018608:Ultrasonography, Doppler