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Idiopathic Infantile Arterial Calcification (IIAC) also known as Arterial Calcification of Infancy, Generalised Infantile Arterial Calcification (GACI), Idiopathic Arterial Calcification of Infancy (IACI), Occlusive Infantile Arterial Calcification, Occlusive Infantile Arteriopathy is an extremely rare, usually fatal genetic disorder, caused by mutations in the ENPP1 gene in 75% of the subjects. The condition affects infants during the first 6 months of life. This condition is inherited as an autosomal recessive pattern. It is characterized by generalised calcification of the arterial internal elastic lamina, leading to rupture of the lamina and occlusive changes in the tunica intima with stenosis and decreased elasticity of the vessel wall. Most infants die of vaso-occlusive disease, especially of the coronary arteries. == Symptoms == Clinical presentation is variable. First symptoms usually occur at birth but can take place in the first 6 months of life or in utero.〔 *Decreased fetal activity *Gestation with an antenatal diagnosis of hydrops fetalis *Polyhydramnios *Low biophysical profile *Marked cyanosis *Edema *Severe hypertension *No or fade pulses *Refusal of feeds *Tachypnea *Vomiting *Abdominal distension *General arterial rigidity *Cardiac failure (most common clinical finding) *Strain pattern 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Idiopathic infantile arterial calcification」の詳細全文を読む スポンサード リンク
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