Van den Bergh F A, Rietra P J, Kolk-Vegter A J, Bosch E, Tager J M
Acta Med Scand. 1976;200(4):249-56.
In a patient with Fabry's disease who had undergone kidney transplantation to correct uremia, the neutral glycosphingolipids and alpha-galactosidase activity have been measured in plasma and urine and, 9 months later, after the death of the patient, in autopsy material. After transplantation, there was no significant increase in alpha-galactosidase activity in plasma; the activity found never exceeded 3% of the mean control value. A striking parallelism was found during the follow-up period in the increase and decrease of trihexosylceramide and globoside and also of glucosylceramide and dihexosylceramide. The alpha-galactosidase activity in spleen and liver was as low as that observed in untreated Fabry hemizygotes. These data and those obtained from autopsy material provide evidence that renal transplantation does not lead to a specific enzymic breakdown of trihexosylceramide in Fabry patients. However, no trihexosylceramide accumulation was observed in the transplanted kidney.
在一名因尿毒症接受肾移植的法布里病患者中，已对其血浆和尿液中的中性糖鞘脂及α - 半乳糖苷酶活性进行了测定，9个月后，在患者死亡后，又对尸检材料进行了检测。移植后，血浆中的α - 半乳糖苷酶活性没有显著增加；所测得的活性从未超过平均对照值的3%。在随访期间，发现神经酰胺三己糖苷和红细胞糖苷脂以及葡糖神经酰胺和二己糖神经酰胺的增减呈现出显著的平行关系。脾脏和肝脏中的α - 半乳糖苷酶活性与未经治疗的法布里半合子中观察到的活性一样低。这些数据以及从尸检材料中获得的数据表明，肾移植并不会导致法布里病患者体内神经酰胺三己糖苷发生特定的酶促分解。然而，在移植肾中未观察到神经酰胺三己糖苷的蓄积。
