Ring E, Wendler H, Ratschek M, Zobel G
Department of Paediatrics, University of Graz, Austria.
Pediatr Radiol. 1989;20(1-2):131-3. doi: 10.1007/BF02010661.
A patient with primary hyperoxaluria type I in infancy is reported. He had renal insufficiency, but urolithiasis was absent. Demonstration of diffuse nephrocalcinosis by renal ultrasound contributed to early diagnosis. Prolonged survival leads to extensive extrarenal oxalate deposition. Repeated skeletal surveys showed the development and the progression of severe hyperoxaluria-related bone disease. Translucent metaphyseal bands with sclerotic margins, wide areas of rarefaction at the ends of the long bones, and translucent rims around the epiphyses and the tarsal bones were signs of disordered bone growth. Bone density generally increased with time indicating progressive sclerosis due to oxalate deposition in the previously normal bone structure.
本文报道了一名婴儿期患I型原发性高草酸尿症的患者。他存在肾功能不全，但无尿路结石。肾脏超声显示弥漫性肾钙质沉着有助于早期诊断。长期存活导致广泛的肾外草酸盐沉积。多次骨骼检查显示严重的高草酸尿症相关骨病的发生和进展。具有硬化边缘的半透明干骺端带、长骨末端的大片骨质稀疏区域以及骨骺和跗骨周围的半透明边缘是骨生长紊乱的迹象。骨密度通常随时间增加，表明由于草酸盐沉积在先前正常的骨结构中导致进行性硬化。
