Grise P, Le Luyer B, Mitrofanoff P
Chir Pediatr. 1983;24(6):411-2.
A case of oxalate lithiasis associated with Abetalipoproteinemia is reported. The excessive absorption of dietary oxalate in this patient with fat malabsorption occurs because unabsorbed fatty acids in the bowel lumen combine with calcium ions which would otherwise impair oxalate absorption by forming insoluble calcium oxalate crystals. The medical management include dietary fat restriction and supplements of the fat soluble vitamins A, E and K. The prognostic is poor particularly in the neurological and retinal component of this disease.
报告了一例与无β脂蛋白血症相关的草酸盐结石病例。该脂肪吸收不良患者出现膳食草酸盐过度吸收,是因为肠腔内未吸收的脂肪酸与钙离子结合,否则钙离子会通过形成不溶性草酸钙晶体而损害草酸盐吸收。医学处理包括限制膳食脂肪以及补充脂溶性维生素A、E和K。预后较差,尤其是在该疾病的神经和视网膜方面。