Patel Maya, Andronikou Savvas, Solomon Rustum, Sinclair Paul, McCulloch Mignon
Department of Paediatric Radiology, Red Cross Children's Hospital, Cape Town, South Africa.
Pediatr Radiol. 2004 Aug;34(8):652-5. doi: 10.1007/s00247-004-1166-z. Epub 2004 Mar 17.
Idiopathic arterial calcification in infancy is usually fatal with death in early life and diagnosis at post mortem. This report describes a unique, late presentation with hypertension and cardiac failure in a child aged 33 months, found to have widespread arterial calcification at radiological imaging. The calcium-phosphate axis was normal and there was no other demonstrable cause for calcification. Additionally, the histological features of arterial calcification at renal biopsy paralleled the findings in infants with this disorder. The late presentation in this case is unusual and has not been previously reported. Ultrasound and CT are sensitive for calcification, and the disease should be suspected in children presenting with cardiac or respiratory manifestations and features of arterial calcification, where no metabolic cause is established.
婴儿期特发性动脉钙化通常是致命的,在生命早期死亡,死后才得以诊断。本报告描述了一名33个月大儿童的独特晚期表现,伴有高血压和心力衰竭,在影像学检查中发现有广泛的动脉钙化。钙磷轴正常,没有其他可证实的钙化原因。此外,肾活检时动脉钙化的组织学特征与患有这种疾病的婴儿的发现相似。该病例的晚期表现不常见,此前未见报道。超声和CT对钙化敏感,对于出现心脏或呼吸表现以及动脉钙化特征且未确定代谢原因的儿童,应怀疑患有此病。