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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic arterial calcification of infancy, or occlusive infantile arterial calcification, is a rare cause of arterial calcification. This condition is inherited as an autosomal recessive pattern, which is almost always fatal. In most of the 100 cases described in the literature, the diagnosis was made at autopsy; a few cases have been reported in which an antemortem diagnosis was made on the basis of radiographic or sonographic demonstration of arterial calcification. The clinical characteristics are extremely variable, and respiratory distress with cardiac failure is the most common finding. Hypertension that is refractory to treatment is present in most cases. Most infants die before the age of 6 months, and very few have survived for more than 1 year. Mortality in this condition is caused by myocardial ischemia due to coronary artery involvement or refractory cardiac failure. The purpose of this report is to describe the prenatal diagnosis of idiopathic arterial calcification. Fewer than 10 cases of antenatal detection of this condition have been reported in the literature. This case is 1 of 2 cases with the earliest diagnosis of this condition so far (Table 1).
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PMID:Antenatal detection of idiopathic arterial calcification with hydrops fetalis. 1279 64

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.
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PMID:Idiopathic Arterial Calcification of Infancy: Case Report. 2725 93