Plasma calcium oxalate supersaturation in children with primary hyperoxaluria and end-stage renal failure
Plasma calcium oxalate supersaturation in children with primary hyperoxaluria and end-stage renal failure
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DOI：
10.1046/j.1523-1755.1999.00546.x
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发表时间：
1999-07-01
影响因子：
19.6
通讯作者：
Langman, CB
中科院分区：
文献类型：
--
作者：
Hoppe, B;Kemper, MJ;Langman, CB
背景。患有原发性高草酸尿症（PH 1）型的儿童在终末期肾病（ESRD）中发生系统性草酸盐中毒的风险很高，因为内源性草酸盐的产生超过了透析治疗去除的草酸盐。随着草酸盐的积累，草酸钙 (CaOx) 会发生组织沉积。然而，患有其他原因导致 ESRD 的儿童尽管血浆草酸盐 (P-Ox) 水平升高，但不易发生 CaOx 沉积。方法。我们的研究目的是检查这些观察结果的潜在机制。我们测量了 7 名 PH 1 引起的 ESRD 儿童和 33 名非 PH 相关 ESRD 儿童的 P-Ox、硫酸盐、柠檬酸盐，并计算了 CaOx 饱和度 (β(CaOx))。 6 名 PH 1 和 22 名非 PH 患者进行维持性血液透析 (HD)：此时分析 HD 前和后的水平，并在 12 个月内对 5 名 PH 1 和 14 名非 PH 患者重复两次。 12 名接受腹膜透析 (PD) 的患者（其中一名 PH 1）仅采集一次样本。肝肾或肾移植后，重复测定血浆水平。结果。 PH患者的平均P-Ox（125.7+/-17.9μmol/L）高于非PH患者（44.2+/-3.3μmol/L，P<10（-4））。所有其他测定的阴离子在两组之间没有差异。与非 PH 儿童 (1.56 +/- 0.12 单位，P < 10(-4)) 相比，PH 1 儿童的 beta(CaOx) 较高（4.71 +/- 0.69 相对单位）。 P-Ox 和 beta(CaOx) 在 PH 1 组 (r = 0.98: P < 2 x 10(-4)) 和非 PH 组 (r = 0.98, P < 10(-4)) 中均相关。在非 PH 儿童中，P-Ox 和 β(CaOx) 随着时间的推移保持稳定，而在 PH 1 患者中，经过六个月更积极的透析后，观察到 P-Ox 和 β(CaOx) 略有下降。在所有 PH 1 患者中以及 33 名非 PH 患者中的 25 名患者中，β(CaOx) 均处于过饱和状态（超过 1）。 HD 后 β(CaOx) 在所有 PH 1 患者中均保持在 1 以上，但在 22 名非 PH 患者中只有 2 名患者。在非 PH 儿童中。肾移植成功后三周内，P-Ox 和 beta(CaOx) 降至正常。而两名PH患者肝肾联合移植后7个月，该水平仍保持较高水平。结论。患有 ESRD 的 PH 1 儿童的系统性草酸盐中毒是由于较高的 P-Ox 和 β(CaOx) 水平所致。即使在严重的 HD 后，由于 β(CaOx) 在 PH 1 中仍保持过饱和，因此草酸盐积累增加，并发生 CaOx 组织沉积。因此，充分减少 P-Ox 和 beta(CaOx) 在 PH 1 中至关重要，并且只能通过早期、抢先、肝肾联合移植或单独肝移植来实现。
Background. Children with primary hyperoxaluria type (PH 1) are at great risk to develop systemic oxalosis in endstage renal disease (ESRD), as endogenous oxalate production exceeds oxalate removal by dialytic therapy. As oxalate accumulates, calcium oxalate (CaOx) tissue deposition occurs. Children with other causes of ESRD, however, are not prone to CaOx deposition despite elevated plasma oxalate (P-Ox) levels.Methods. Our study objective was to examine the potential mechanisms for these observations. We measured P-Ox, sulfate, citrate, and calculated CaOx saturation (beta(CaOx)) in 7 children with ESRD caused by PH 1 and in 33 children with non-PH-related ESRD. Maintenance hemodialysis (HD) was performed in 6 PH 1 and 22 non-PH patients: Pre- and post-HD levels were analyzed at this point and were repeated twice within 12 months in 5 PH 1 and 14 non-PH patients. Samples were obtained only once in 12 patients (one PH 1) on peritoneal dialysis (PD). After liver-kidney or kidney transplantation, plasma levels were measured repetitively.Results. The mean P-Ox was higher in PH (125.7 +/- 17.9 mu mol/liter) than in non-PH patients (44.2 +/- 3.3 mu mol/liter, P < 10(-4)). All other determined anions did not differ between the two groups. beta(CaOx) was higher in PH 1 (4.71 +/- 0.69 relative units) compared with non-PH children (1.56 +/- 0.12 units, P < 10(-4)). P-Ox and beta(CaOx) were correlated in both the PH 1 (r = 0.98: P < 2 x 10(-4)) and the non-PH group (r = 0.98, P < 10(-4)). P-Ox and beta(CaOx) remained stable over time in the non-PH children, whereas an insignificant decline was observed in PH 1 patients after six months of more aggressive dialysis. beta(CaOx) was supersaturated (more than 1) in all PH 1 and in 25 out of 33 non-PH patients. Post-HD beta(CaOx) remained more than 1 in all PH 1, but in only 2 out of 22 non-PH patients. In non PH children. P-Ox and beta(CaOx) decreased to normal within three weeks after successful kidney transplantation. whereas the levels still remained elevated seven months after combined liver-kidney transplantation in two PH patients.Conclusion. Systemic oxalosis in PH 1 children with ESRD is due to higher P-Ox and beta(CaOx) levels. As beta(CaOx) remained supersaturated in PH 1 even after aggressive HD, oxalate accumulation increases, and CaOx tissue deposition occurs. Therefore, sufficient reduction of P-Ox and beta(CaOx) is crucial in PH 1 and might only be achieved by early, preemptive, combined liver-kidney transplantation or liver transplantation alone.