Idiopathic Arterial Calcification in Infancy

Gower, N. D.; Pinkerton, John

doi:10.1136/adc.38.200.408

Cited by 15 publications

(3 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…IIAC is formally diagnosed based on characteristic pathologic findings. The most commonly reported findings on gross examination are: (i) myocardial hypertrophy (4, 18, 28, 42–46) from either unilateral or bilateral ventricular enlargement, (ii) subendocardial fibrosis (8, 31, 32, 47), thought secondary to tissue ischemia and (iii) firm and tortuous coronary arteries (4, 26, 42, 44, 46).…”

Section: Review Of the Literaturementioning

confidence: 99%

“…Intimal hyperplasia is a fundamental change in this disorder and plays a major role in vascular occlusion, leading to tissue ischemia and organ dysfunction. In addition, acute inflammatory changes within the intima and media are often seen at sites of calcification (4, 28, 36, 42, 45, 46, 48), either as eosinophilic infiltration or giant cell reaction. Electron microscopy shows that the intimal thickening is due to proliferation of both fibroblasts and smooth muscle cells (2) and confirms that the spicules deposited around the internal elastic lamina consist of a crystalline material consistent with hydroxyapatite (17, 48).…”

Section: Review Of the Literaturementioning

confidence: 99%

“…Others feel that an abnormality of calcium metabolism is to blame (44) with dystrophic calcification occurring in a previously injured arterial wall (30). Still others suggest that the primary defect is intimal fibrous proliferation (42), with elastic degeneration and calcification occurring as secondary phenomena because of a hemodynamic or pressure effect created by the intimal proliferation (24). The presence of inflammatory cells within the arterial walls at sites of calcification has led some authors to propose an allergic (46) or infectious (28) etiology, perhaps occurring antenatally (48).…”

Section: Review Of the Literaturementioning

confidence: 99%

See 2 more Smart Citations

Idiopathic infantile arterial calcification: Two case reports, a review of the literature and a role for cardiac transplantation

Glatz

Pawel

Hsu

et al. 2005

Pediatric Transplantation

View full text Add to dashboard Cite

Idiopathic infantile arterial calcification (IIAC) is a rare, but important, cause of rapidly progressive ischemic heart disease in children. In this paper, we report two recent cases of IIAC seen at tertiary referral hospitals. Both cases presented in infancy with signs of heart failure and, ultimately, died with the diagnosis of IIAC confirmed at postmortem examination. A thorough review of the literature reveals approximately 160 reported cases of IIAC. The clinical outcomes, radiographic findings and pathologic details are summarized. Proposed etiologic mechanisms are reviewed, including promising research into the role of inorganic pyrophosphate as a regulatory factor in the development of IIAC. Because of the typically fatal outcome of IIAC and the lack of proven therapies, the potential role for cardiac transplantation is discussed.

show abstract

Section: Review Of the Literaturementioning

confidence: 99%

Section: Review Of the Literaturementioning

confidence: 99%

Section: Review Of the Literaturementioning

confidence: 99%

See 1 more Smart Citation

Idiopathic infantile arterial calcification: Two case reports, a review of the literature and a role for cardiac transplantation

Glatz

Pawel

Hsu

et al. 2005

Pediatric Transplantation

View full text Add to dashboard Cite

show abstract

Left Ventricular Aneurysm in Rubella Heart Disease

Horst¹,

Gotsman²

1970

Arch Pediatr Adolesc Med

View full text Add to dashboard Cite

A 3-year-old patient with the rubella syndrome was studied by cardiac catheterization and cineangiography and was found to have an aneurysm of the left ventricle in addition to a persistent ductus arteriosus and hypoplasia with coarctations of the pulmonary arteries. The ductus arteriosus was ligated and divided, and residual myocardial dysfunction was demonstrated six months after surgery. The aneurysm is considered to be directly related to rubella myocarditis which occurred at an earlier age in association with other areas of myocarditis resulting in a myopathic disorder. Gregg1 first described the rela¬ tionship between rubella and congenital malformations, and nu¬ merous reports have appeared de¬ scribing the association between the rubella virus and cardiovascular dis¬ ease. Hastreiter et al2 found pulmo¬ nary artery anomalies in all of their cases, a patent ductus arteriosus in 75% of cases, and hypoplasia of the aortic isthmus in 16% of their series. Other cardiovascular diseases include myocarditis,3 renal artery stenosis," supravalvar aortic stenosis,5 and coro¬ nary artery sclerosis.6 Septal defects and other rarer malformations have also been described.7It is the purpose of this report to describe a child with the stigmata of the congenital rubella syndrome who also has an apical aneurysm of the left ventricle, a patent ductus arte¬ riosus, and pulmonary arterial hypo¬ plasia with coarctation confirmed by cardiac catheterization, selective cineangiocardiography, and surgery. A similar combination of lesions has not been reported previously. Report of a CaseAn Indian boy was seen initially at 17 months of age; he was deaf and had phys¬ ical and developmental retardation and microcephaly (skull circumference, 41 cm). The mother denied a history of rubella in pregnancy, but a diagnosis of the rubella syndrome was made on the clinical fea¬ tures and the demonstration of significant elevation of the titer of rubella neutral¬ izing antibodies. There was a hemaggluti¬ nation inhibiting antibody titer of 1:256 against rubella antigen. No rubella virus was isolated from a throat swab or urine. A continuous murmur was heard under the left clavicle and suggested that he had a persistent ductus arteriosus. The patient remained well until he was 3 years old when he presented with heart failure for the first time. He had bounding pulses, a large thrusting left ventricle, a loud, widely split but mobile second heart sound, an apical mid-diastolic murmur, and a loud continuous murmur under the left clavicle which radiated through to the back. The initial hemoglobin concentration was 5.5 gm/100 cc; the red cells showed a pattern of iron deficiency, and the ab¬ normality probably contributed to his heart failure. The electrocardiogram is shown in Fig 1 and the chest roentgeno¬ gram before ductal surgery and six months later in Fig 2. Blood was trans¬ fused, and heart failure was treated with digitalis. Catheterization of the left and right sides of the heart with selective angiocardiography was performed to

show abstract

Intimal hyperplasia of the aorta and renal vessels in an infant with hypertension

Formby

Emery

1969

The Journal of Pathology

View full text Add to dashboard Cite

Idiopathic Arterial Calcification in Infancy

Cited by 15 publications

References 3 publications

Idiopathic infantile arterial calcification: Two case reports, a review of the literature and a role for cardiac transplantation

Idiopathic infantile arterial calcification: Two case reports, a review of the literature and a role for cardiac transplantation

Left Ventricular Aneurysm in Rubella Heart Disease

Intimal hyperplasia of the aorta and renal vessels in an infant with hypertension

Contact Info

Product

Resources

About