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Thoracic aortic calcification in 3 children with candidiasis-endocrinopathy syndrome
A Shikata1, T Sugimoto, K Kosaka
1Department of Pediatrics, Kyoto Prefectural University of Medicine, Japan.
Insights
Childhood-onset thoracic aortic calcification is linked to mucocutaneous candidiasis and endocrine issues. This vascular calcification pattern is a key feature of the candidiasis-endocrinopathy syndrome.
Area of Science:
- Vascular biology
- Pediatric endocrinology
- Immunology
Background:
- The candidiasis-endocrinopathy syndrome is a rare disorder characterized by recurrent mucocutaneous candidiasis and autoimmune endocrine deficiencies.
- Aortic calcification in children is uncommon and its association with specific syndromes requires further investigation.
Abstract:
Three cases are reported of the association of childhood onset of thoracic aortic calcification with mucocutaneous candidiasis, endocrine dysfunction and recurrent non-fungal pulmonary disease. The aortic calcification affects the thoracic aorta and the low lumbar aorta and common iliac arteries, sparing the mid-lumbar aorta and its major branches. Ischemic signs and symptoms of the head and neck and lower limbs are absent. This peculiar, slowly progressive vascular calcification, although unexplained to date, appears to be a non-random part of the more common candidiasis-endocrinopathy syndrome.