Sud Kamal, Swaminathan Sundararaman, Varma Neelam, Kohli Harbir Singh, Jha Vivekanand, Gupta Krishan Lal, Sakhuja Vinay
Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Nephrology (Carlton). 2004 Dec;9(6):422-5. doi: 10.1111/j.1440-1797.2004.00317.x.
Combined liver and kidney transplantation is the ideal treatment for patients with end-stage renal failure secondary to primary hyperoxaluria and systemic oxalosis, with a functioning liver providing replacement of the deficient enzyme and a functioning kidney providing the route of excretion for the oxalate crystals. Pancytopenia from bone marrow infiltration of oxalate crystals is a rare complication of primary hyperoxaluria, and its reversal following transplant has not been described. We report the first case of pancytopenia from marrow infiltration by oxalate crystals reversing following a successful kidney transplant alone. Although kidney alone transplants do not provide the best chance of survival or quality of life as compared to a combined kidney and liver transplant, a well functioning kidney transplant is able to take care of the systemic oxalate load and ameliorate, at least for a period of time, the systemic complications of oxalosis.
肝肾联合移植是治疗原发性高草酸尿症继发终末期肾衰竭和全身性草酸osis的理想方法,功能正常的肝脏可替代缺乏的酶,功能正常的肾脏可为草酸盐晶体提供排泄途径。草酸盐晶体骨髓浸润导致的全血细胞减少是原发性高草酸尿症的一种罕见并发症,移植后其逆转情况尚未见报道。我们报告了首例仅成功进行肾移植后,草酸盐晶体骨髓浸润导致的全血细胞减少得到逆转的病例。尽管与肝肾联合移植相比,单纯肾移植不能提供最佳的生存机会或生活质量,但功能良好的肾移植能够处理全身草酸盐负荷,并至少在一段时间内改善草酸osis的全身并发症。