Idiopathic Arterial Calcification Of Infancy Research Articles

Idiopathic Arterial Calcification: Experience from a Single Center in South India

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. We hereby report a series of 20 cases seen over a period of 10 years. Nineteen cases were reported as idiopathic arterial calcification and one was reported as arterial calcification in a twin reversed arterial perfusion sequence. The sites of calcification were mainly aortic valve and root of aorta which was involved in 100% of cases followed by pulmonary artery up to its branches in 18 (90%) cases. There was recurrence in the subsequent pregnancies in three of the cases. Genetic association with ENPP1 was proved in one case. There was no survival in all the cases. Thus, the prognosis of idiopathic arterial calcification is very poor with very few long-term survivors.

Idiopathic Arterial Calcification of Infancy- Peritoneal Dialysis for Treatment of Hypertension

Idiopathic infantile arterial calcification (IIAC) is a rare autosomal recessive disease usually diagnosed postmortem. The clinical presentation is not typical, but usually implies refractory hypertension and cardiorespiratory failure. We present a case of a newborn with IIAC who had fetal hydrops and refractory hypertension which normalized soon after initialization of peritoneal dialysis. With this case report, we wanted to highlight that peritoneal dialysis may be beneficial an effective therapeutic option for patients with IIAC and severe refractory hypertension. Until now, peritoneal dialysis was never performed in the treatment of patients with IIAC.

A Tale of Two Sisters: Idiopathic Arterial Calcification of Infancy in Siblings

Idiopathic arterial calcification of infancy (IACI) is a rare disorder, characterized by extensive arterial calcification of large and medium sized arteries. We present two cases of IACI in siblings including the prenatal, postnatal and postmortem imaging, which is a hallmark of this disease.

Bisphosphonate-related enamel hypoplasia in a child with idiopathic arterial calcification of infancy

We report bisphosphonate-related enamel hypoplasia as a rare side effect in a child with idiopathic arterial calcification of infancy.

Non-atherosclerotic coronary artery disease associated with sudden cardiac death

BackgroundThe concept of non-atherosclerotic coronary artery pathology in sudden cardiac death (SCD) has not been given the attention it deserves.ObjectiveTo determine the incidence of non-atherosclerotic coronary artery pathology in SCD...

Clinical images: Joint involvement in idiopathic arterial calcification of infancy

Clinical images: Joint involvement in idiopathic arterial calcification of infancy

The difficulty in diagnosing idiopathic arterial calcification of infancy, its variation in presentation, and the importance of autopsy

Idiopathic calcification of the coronary arteries is a rare hereditary condition of infancy. Complications include cardiac ischaemia, cardiac failure, and systemic hypertension. We present three patients with this condition, which in each case masqueraded as other cardiac diseases, with no indication of the specific diagnosis prior to autopsy.A 5 month old female presented with respiratory failure and hypertension and died within 24 hours of admission. All the coronary arteries were thick-walled, with narrow lumens. The aorta, great vessels, and renal arteries also showed thickening of the wall. Histology confirmed calcium in the internal elastic lamina of all vessels. The second patient was a female baby of 2 months, diagnosed with a large ventricular septal defect. She died suddenly prior to surgery. At autopsy, the orifice of the right coronary artery was reduced to a pinhole. The coronary arteries showed white patches of calcification, with associated ventricular infarction. The third patient was an 11 year old female who presented with cardiac failure, and had been diagnosed with dilated cardiomyopathy. Two weeks later, she died suddenly. The coronary arteries were patent, but firm with calcification and narrowed, with associated ventricular infarction. Our experience shows that idiopathic arterial calcification of infancy should always be considered in infants and children presenting with hypertension, cardiac failure, or sudden death.

Anesthetic management of a patient with idiopathic arterial calcification of infancy and fused cervical spine

Anesthetic management of a patient with idiopathic arterial calcification of infancy and fused cervical spine

New Findings in Idiopathic Arterial Calcification of Infancy Detected by MDCT

2Department of Pediatrics, University of Arkansas and Arkansas Children’s Hospital, Little Rock, AR 72202. rtery morphology and cardiac function were evaluated in an infant with idiopathic arterial calcification using MDCT. New morphologic findings identified included a thickened myocardium with a heterogeneous enhancement pattern consistent with a cardiomyopathy and a ring appearance caused by high-attenuation aortic wall calcification and lumen enhancement separated by low-attenuation thickened intima.

Etiology and outcome of fetuses with functional heart disease.

The aim of this study was to assess the etiology and outcome of fetuses with functional heart disease as detected by echocardiography. In total, 51 fetuses (median gestation age of 28.6 weeks) were included. The inclusion criteria were hydrops (n = 14), pericardial effusion (PE; n = 9), tricuspid valve regurgitation (TR; n = 8), hypertrophic cardiomyopathy (HCM; n = 7) and dilated cardiomyopathy (DCM; n = 7). Antenatal management was performed for 17 of 51 fetuses (33%): two abortions, nine digoxin administrations, three thoracocenteses, one pericardial puncture, one blood transfusion and one ascites centesis. The etiology of functional heart disease was twin pregnancy in 18, fetal lung lesions in five, maternal diabetes in five, fetal anemia in four, extracardiac or chromosomal abnormalities in three, infection in three, teratoma or arteriovenous malformation in four, indomethacin administration in two, endocardial fibroelastosis in two, maternal anaphylaxia in one, idiopathic arterial calcification of infancy (IACI) in one, pregnancy-induced hypertension (PIH) in one, and unknown in two fetuses. There was no significant difference between fetuses with and without treatment (53% vs. 79%; p = 0.06). There were two stillbirths and 12 postnatal deaths (29%). Among 35 surviving infants, 85% were free of symptoms in the follow-up (mean 3.9 years). These findings indicate that a functional heart disease in utero is associated with very varying etiology and high mortality. Improved understanding of the hemodynamic findings may lead to treatment that is more successful.

Etiology and outcome of fetuses with functional heart disease

Background. The aim of this study was to assess the etiology and outcome of fetuses with functional heart disease as detected by echocardiography. Methods. In total, 51 fetuses (median gestation age of 28.6 weeks) were included. The inclusion criteria were hydrops (n = 14), pericardial effusion (PE; n = 9), tricuspid valve regurgitation (TR; n = 8), hypertrophic cardiomyopathy (HCM; n = 7) and dilated cardiomyopathy (DCM; n = 7). Antenatal management was performed for 17 of 51 fetuses (33%): two abortions, nine digoxin administrations, three thoracocenteses, one pericardial puncture, one blood transfusion and one ascites centesis. Results. The etiology of functional heart disease was twin pregnancy in 18, fetal lung lesions in five, maternal diabetes in five, fetal anemia in four, extracardiac or chromosomal abnormalities in three, infection in three, teratoma or arteriovenous malformation in four, indomethacin administration in two, endocardial fibroelastosis in two, maternal anaphylaxia in one, idiopathic arterial calcification of infancy (IACI) in one, pregnancy-induced hypertension (PIH) in one, and unknown in two fetuses. There was no significant difference between fetuses with and without treatment (53% vs. 79%; p = 0.06). There were two stillbirths and 12 postnatal deaths (29%). Among 35 surviving infants, 85% were free of symptoms in the follow-up (mean 3.9 years). Conclusions. These findings indicate that a functional heart disease in utero is associated with very varying etiology and high mortality. Improved understanding of the hemodynamic findings may lead to treatment that is more successful.

P164: Ultrasound prenatal diagnosis of Idiopathic Arterial Calcification of Infancy: a case report and review of the literature

P164: Ultrasound prenatal diagnosis of Idiopathic Arterial Calcification of Infancy: a case report and review of the literature

Pulmonary arterial and intracranial calcification in the recipient of a twin–twin transfusion

Pulmonary arterial and intracranial calcifications are rarely found in children. A female infant, the recipient of a twin-twin transfusion syndrome was found, by ultrasound and computed tomography, to have both pulmonary arterial and intracerebral calcification. A rare condition, termed idiopathic arterial calcification of infancy, is the likely cause. This condition carries a poor prognosis and is usually fatal.

Aorto-pulmonary calcification: An unusual manifestation of idiopathic calcification of infancy evident antenatally

Idiopathic arterial calcification of infancy represents a clinical spectrum involving calcification of large and medium-sized blood vessels with an unknown etiology. Its complications include severe systemic hypertension and cardiomyopathy. A twin infant with a variant of idiopathic arterial calcification was diagnosed antenatally by the detection of hyperechogenicity of the proximal aorta and central pulmonary vessels. The calcification was apparently isolated to these vessels and was associated with polyhydramnios and hypertrophic cardiomyopathy. Because antenatal diagnosis is possible, we suggest that there should be a high index of suspicion for idiopathic arterial calcification when there is sonographic hyperechogenicity of vessel walls, evidence of polyhydramnios, cardiomyopathy, or a family history of idiopathic arterial calcification.

Idiopathic arterial calcification of infancy: Prenatal and postnatal effects of therapy in an infant

In an infant with idiopathic arterial calcification of infancy, prenatal diagnosis of arterial calcification was made by ultrasonography and allowed initiation of therapy in utero. Etidronate therapy produced apparent radiographic and ultrasonographic improvement in the degree of vascular calcification but did not prevent the lethal progression of intimal vascular occlusive disease.

Idiopathic arterial calcification of infancy

We describe two twin sisters in whom calcification of different arteries was detected in the first weeks of life. Transient renal insufficiency, arterial hypertension, and skeletal abnormalities were also observed. One child had anasarca and heart decompensation at birth. Prenatal infarction of one kidney had occurred in the same infant. A kidney biopsy showed calcium deposits in all the layers of the arteries. Most findings in these patients are compatible with idiopathic arterial calcification of infancy (IACI). Investigation of calcium and phosphorus metabolism revealed spontaneously receding hypercalciuria, increased intraerythrocytic calcium levels, and transient X-ray abnormalities of the long bones. Treatment initially consisted of biphosphonate and later, the calcium antagonist flunarizin. A progressive diminution of the arterial calcification was observed in the course of both treatments.

Idiopathic arterial calcification of infancy: a case with prolonged survival

We describe a patient with idiopathic arterial calcification of infancy and the following unusual features of the disease: (1) prolonged survival until age 11 years, (2) discordance between the extent of vascular calcification and clinical manifestations of arterial luminal occlusion, (3) a large area of myocardial calcification, (4) symptoms of cerebral-vascular insufficiency, and (5) spontaneous resolution of hypertension.

Arteriopathy in children with acquired immune deficiency syndrome.

Pathologic features of the arteries of different organs (heart, lungs, kidneys, spleen, intestine, brain) seen at autopsy in 6 children with acquired immune deficiency syndrome (AIDS) are described. Small and medium-sized arteries, which were the most commonly involved, showed intimal fibrosis with fragmentation of elastic tissue, fibrosis and calcification of media with variable luminal narrowing, and a vasculitis or perivasculitis that was seen only in the brain in association with AIDS encephalopathy. In 1 case aneurysms of the right coronary artery with thrombosis and myocardial infarction were seen. Vascular inflammation, seen only in the brain, may be related to the agent associated with AIDS encephalopathy. The fibrocalcific arterial lesions most closely resemble idiopathic arterial calcification of infancy, but because of differences in age incidence, clinicopathologic and immunologic features, and the size and distribution of the involved arteries, the arterial lesions of pediatric AIDS appear to constitute a distinctive arteriopathy. Infection, secondary to immunodeficiency and resulting in increased exposure to endogenous and exogenous elastases, may be the pathogenesis. Luminal narrowing caused by arterial lesions may play a contributory role in the pathogenesis of the atrophy, cell depletion, scarring, and necrosis or infarction found in organs of children with AIDS. Pediatricians should be alerted to the possibility of arterial involvement in pediatrics AIDS.

Hypertension as the major problem of idiopathic arterial calcification of infancy

We describe two infants with severe hypertension associated with idiopathic arterial calcification of infancy. In both children, blood pressure control was refractory to aggressive antihypertensive therapy. There was radiologic and laboratory evidence of renovascular disease requiring the use of specific renin antagonists and later nephrectomy, resulting in moderate improvement of hypertension. Although on occasion there is a familial incidence, in the vast majority of cases the diagnosis hinges on a high index of suspicion.

Idiopathic arterial calcification of infancy: S. G. Kirchner, R. M. Heller, A. G. Kasselberg. South Med J 72:1328–1330, (October), 1979

Idiopathic arterial calcification of infancy: S. G. Kirchner, R. M. Heller, A. G. Kasselberg. South Med J 72:1328–1330, (October), 1979