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Arteriopathy in children with acquired immune deficiency syndrome
1Department of Pathology, Children's Hospital of New Jersey, Newark 07107.
Insights
Arterial lesions, including fibrosis and calcification, are distinctive in children with acquired immune deficiency syndrome (AIDS). These vascular changes may contribute to organ damage in pediatric AIDS patients.
Area of Science:
- Pathology
- Pediatric Medicine
- Vascular Biology
Background:
- Acquired immune deficiency syndrome (AIDS) can affect multiple organ systems.
- Arterial pathology in pediatric AIDS is not well-characterized.
- Understanding vascular complications is crucial for managing pediatric AIDS.
Purpose of the Study:
- To describe the pathologic arterial features in children with AIDS.
- To differentiate pediatric AIDS arteriopathy from other vascular conditions.
- To explore potential pathogenetic mechanisms and clinical implications.
Main Methods:
- Autopsy examination of arteries from various organs (heart, lungs, kidneys, spleen, intestine, brain) in 6 pediatric AIDS cases.
- Histopathologic analysis of intimal and medial arterial layers.
- Correlation of vascular findings with clinical presentation and organ pathology.
Main Results:
- Small and medium-sized arteries commonly affected.
- Intimal fibrosis, medial calcification, and luminal narrowing observed.
- Vasculitis and perivasculitis noted in the brain associated with AIDS encephalopathy.
- One case showed coronary artery aneurysm with thrombosis and myocardial infarction.
- Lesions resemble idiopathic arterial calcification of infancy but are distinct.
Conclusions:
- Pediatric AIDS is associated with a distinctive arteriopathy.
- Vascular inflammation in the brain may link to AIDS encephalopathy.
- Arterial lesions can contribute to organ damage in pediatric AIDS.
- Pediatricians should consider arterial involvement in pediatric AIDS cases.
Abstract:
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.