Watts R W, Calne R Y, Williams R, Mansell M A, Veall N, Purkiss P, Rolles K
Q J Med. 1985 Oct;57(222):697-703.
A case is reported of a patient with renal failure and developing systemic and renal oxalosis due to pyridoxine-resistant type I primary hyperoxaluria. In spite of vigorous haemodialysis and hydration before and after operation, an allografted cadaveric kidney failed because of oxalate deposits in the transplant. The patient was treated by combined hepatic and renal transplantation. The liver allograft functioned well but the kidney had poor function due to primary acute tubular necrosis aggravated by steroid-associated acute pancreatitis, systemic cytomegalovirus infection and high cyclosporin A levels. The patient died from generalised cytomegalovirus infection. The early course after operation was associated with a reduced rate of oxalate production, which would slow the rate of oxalate deposition in the tissues. The size of the oxalate metabolic pool was also diminished. These observations are compatible with the grafted liver having corrected the metabolic lesion.
报道了1例因I型原发性高草酸尿症对吡哆醇耐药而出现肾衰竭并发展为全身性和肾性草酸沉着症的患者。尽管在手术前后进行了积极的血液透析和补液，但移植的尸体肾因移植肾中草酸盐沉积而功能衰竭。该患者接受了肝肾联合移植治疗。肝移植功能良好，但由于原发性急性肾小管坏死因类固醇相关性急性胰腺炎、全身性巨细胞病毒感染和高环孢素A水平而加重，肾移植功能不佳。患者死于全身性巨细胞病毒感染。术后早期病程与草酸盐生成率降低有关，这将减缓草酸盐在组织中的沉积速度。草酸盐代谢池的大小也减小了。这些观察结果与移植肝脏纠正了代谢病变相符。
