Sustained pyridoxine response in primary hyperoxaluria type 1 recipients of kidney alone transplant.
Sustained pyridoxine response in primary hyperoxaluria type 1 recipients of kidney alone transplant.
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DOI：
10.1111/ajt.12706
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发表时间：
2014-06
期刊：
影响因子：
--
通讯作者：
Milliner DS
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文献类型：
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作者：
Lorenz EC;Lieske JC;Seide BM;Meek AM;Olson JB;Bergstralh EJ;Milliner DS
联合肾肝移植是大多数原发性1型高尿酸血症（PH 1）患者的首选移植选择，因为它消除了草酸盐产生的肝脏来源，提高了肾移植物的存活率。然而，PH 1患者G170 R突变纯合子可以开发正常的尿草酸盐水平与吡哆醇治疗，可能是候选人的肾脏单独移植。我们在1999年9月至2013年7月间移植的5例G170 R纯合子患者中检查了单独肾移植后吡哆醇治疗的疗效。所有患者在移植后均维持吡哆醇。移植时的中位年龄为39岁（范围33-67岁）。移植后中位随访时间为8.5年（范围0.2-13.9年）。随访结束时，4例移植物功能正常。一个移植失败13.9年后移植由于复发草酸盐肾病后急性内科疾病。组织草酸盐储存清除后，移植后尿草酸盐水平在大多数检查时间< 0.5 mmol/24 hr。仅在3/13例同种异体移植物活检中观察到草酸钙晶体。这一系列表明，一个亚组的PH 1患者表现出持续反应吡哆醇治疗后，肾移植。因此，对于G170 R纯合子突变的PH 1患者，应考虑吡哆醇联合单纯肾移植。
Combined kidney liver transplant is the preferred transplant option for most patients with primary hyperoxaluria type 1 (PH1) given that it removes the hepatic source of oxalate production and improves renal allograft survival. However, PH1 patients homozygous for the G170R mutation can develop normal urine oxalate levels with pyridoxine therapy and may be candidates for kidney alone transplant. We examined the efficacy of pyridoxine therapy following kidney alone transplant in five patients homozygous for G170R transplanted between 9/1999 and 7/2013. All patients were maintained on pyridoxine post-transplant. Median age at transplant was 39 years (range 33–67 years). Median follow-up post-transplant was 8.5 years (range 0.2–13.9 years). At the end of follow-up, 4 grafts were functioning. One graft failed 13.9 years post-transplant due to recurrent oxalate nephropathy following an acute medical illness. After tissue oxalate stores had cleared, post-transplant urine oxalate levels were < 0.5 mmol/24 hr the majority of times checked. Calcium oxalate crystals were noted in only 3/13 allograft biopsies. This series suggests that a subgroup of PH1 patients demonstrate sustained response to pyridoxine therapy following kidney alone transplant. Therefore, pyridoxine combined with kidney alone transplantation should be considered for PH1 patients with a homozygous G170R mutation.