Salyer W R, Salyer D C
J Clin Pathol. 1974 Jul;27(7):558-9. doi: 10.1136/jcp.27.7.558.
Type I hyperoxaluria results from reduced activity of alpha-ketoglutarate: glyoxylate carboligase, which is necessary for the synergistic decarboxylation of glyoxylate and alpha-ketoglutarate to alpha-hydroxy-beta-keto-adipate. Since thiamine pyrophosphate is a cofactor in the reaction, thiamine deficiency might be expected to result in tissue oxalosis. However, there was no significant increase in the incidence of renal oxalosis in 15 patients with Wernicke's encephalopathy at necropsy compared with controls. It is possible that hyperoxaluria was present in these thiamine-deficient patients but at a urine concentration below that necessary for calcium oxalate deposition. It is also possible that the severity of the thiamine deficit required for hyperoxaluria exceeds that for the neuronal and cardiac manifestations.
I型高草酸尿症是由于α-酮戊二酸:乙醛酸羧化酶活性降低所致,该酶对于乙醛酸和α-酮戊二酸协同脱羧生成α-羟基-β-酮己二酸是必需的。由于焦磷酸硫胺素是该反应的辅助因子,因此维生素B1缺乏可能会导致组织草酸沉着症。然而,与对照组相比,15例韦尼克脑病患者尸检时肾草酸沉着症的发生率并未显著增加。这些维生素B1缺乏的患者可能存在高草酸尿症,但尿中浓度低于草酸钙沉积所需的浓度。也有可能高草酸尿症所需的维生素B1缺乏严重程度超过了神经元和心脏表现所需的程度。