title: Idiopathic Arterial Calcification of Infancy: Case Report.
abstract: Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Zagazig University, Zagazig, Egypt.
authors.forename: Tarek Hamed
authors.initials: TH
authors.lastname: Attia
authors.affiliation: Department of Pediatrics, Al-hammadi Hospital, Riyadh, Saudi Arabia.
authors.forename: Mohamed Maisara
authors.initials: MM
authors.lastname: Abd Alhamed
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, Zagazig University, Zagazig, Egypt.
authors.forename: Mohamed Fouad
authors.initials: MF
authors.lastname: Selim
authors.affiliation: Department of Radiology, Faculty of Medicine, Banha University, Banha, Egypt.
authors.forename: Mohamed Salah
authors.initials: MS
authors.lastname: Haggag
authors.affiliation: Department of cardiology, Faculty of Medicine, Tanta University, Tanta, Egypt.
authors.forename: Diaa
authors.initials: D
authors.lastname: Fathalla
mesh_terms: D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D061205:Vascular Calcification / Q000000981:diagnostic imaging*
keywords: CT; Idiopathic arterial calcification of infancy; arterial calcification of infancy; arterial calcifications; arteries; autosomal recessive; cardiomyopathy; polyhydramnios