Plasma calcium-oxalate saturation in children with renal insufficiency and in children with primary hyperoxaluria

Plasma calcium-oxalate saturation in children with renal insufficiency and in children with primary hyperoxaluria
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DOI:
10.1046/j.1523-1755.1998.00066.x
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发表时间:
1998-09-01
影响因子:
19.6
通讯作者:
Langman, CB
Langman, CB
中科院分区:
医学1区
文献类型:
--
作者:
Hoppe, B;Kemper, MJ;Langman, CB

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背景资料。草酸钙(CaOx)沉积和全身性草酸中毒在慢性肾功能衰竭(CRI)儿童中并不常见,但在I型高草酸血症(PH-1)儿童中很常见。我们假设血浆CaOx饱和度(β(CaOx))存在差异,其决定因素可以解释这种差异。因此,在常规生化检测的基础上,对17例肾功能正常的PH-1患者、54例慢性肾衰患儿(S-Cr0.9~5.9 mg/dl)和50例健康儿童(NL)测定血浆草酸(P-Ox)、柠檬酸(P-Cit)和硫酸盐(P-Sulf)(血浆阴离子),并计算β(CaOx)。用离子色谱法分析血浆中的阴离子,并用基于PC的溶液平衡程序计算β(CaOx)。与NL组比较,PH-1组和CRI组各阴离子水平及β(CaOx)均升高,PH-1组P-Ox、P-Cit和β(CaOx)均高于CRI组(P&lt;0.05),CRI组P-Sulf高于CRI组(P<0.01)。β(CaOx)与P-Ox呈正相关(r=0.63~0.95,P&lt;10(-4))。在CRI患者中,P-Ox和β(CaOx)均与GFR下降呈负相关。P-Cit和P-Sulf对β(CaOx)无影响。虽然7例慢性肾衰患者和4例PH-1患者存在过饱和(β(CaOx)和GT;L),但眼科检查仅在PH-1患者中可疑CaOx沉积,1例PH患者因草酸盐骨病而确诊为全身性草酸中毒。在PH-1中,即使在肾功能正常的情况下,P-Ox和β(CaOx)也会升高,这增加了CaOx结晶沉积的可能性。因此,更有效的降低β-钙(CaOx)的治疗对于降低全身性草酸中毒的风险至关重要。对于CRI未知但可能具有保护性物质的儿童,尽管P-Ox和β(CaOx)水平升高,通常达到过饱和水平,但仍有助于预防系统性牛津病的风险。
Background. Calcium-oxalate (CaOx) deposition and systemic oxalosis are uncommon in children with chronic renal failure (CRI), but frequent in children with primary hyperoxaluria type I (PH-1). We hypothesized a difference in plasma CaOx saturation (beta(CaOx)) and its determining factors would explain this discrepancy.Methods. Therefore, in addition to common biochemical measurements, plasma-oxalate (P-Ox), citrate (P-Cit) and sulfate (P-Sulf) (plasma anions) were measured and beta(CaOx) was calculated in 17 PH-1 patients with normal renal function receiving pyridoxine and citrate therapy, in 54 children with CRI (S-Cr 0.9 to 5.9 mg/dl), and in 50 healthy children (NL). Plasma anions were analyzed by ion-chromatography and beta(CaOx) was calculated using a PC-based program for solution equilibria.Results. Compared to NL, all plasma anion levels and beta(CaOx) were higher in PH-1 and CRI; P-Ox, P-Cit and beta(CaOx) were higher in PH-1 than in CRI (P < 0.05), but P-Sulf was higher in CRI (P < 0.01). beta(CaOx) and P-Ox were correlated in all groups (r = 0.63 to 0.95, P < 10(-4)). P-Ox and beta(CaOx) were both inversely correlated to a decrease in GFR in CRI patients. P-Cit and P-Sulf did not influence beta(CaOx). Although supersaturation (beta(CaOx) > l) was found in 7 CRI and in 4 PH-1 patients, eye examinations were suspicious for CaOx depositions only in the PH-1 patients, while systemic oxalosis was confirmed in one PH patient because of oxalate osteopathy.Conclusions. In PH-1, P-Ox and beta(CaOx) are elevated even with normal renal function, which increases the likelihood of CaOx crystal deposition. Therefore, more effective therapy to decrease beta(CaOx) is crucial to reduce the risk of systemic oxalosis. In children with CRI unknown, but presumably protective substances, help prevent the risk of systemic oxalosis, despite increased P-Ox and beta(CaOx) levels, often to supersaturation levels.