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肾功能不全患儿和原发性高草酸尿症患儿的血浆草酸钙饱和度。

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

作者信息

Hoppe B, Kemper M J, Bökenkamp A, Langman C B

机构信息

Department of Pediatrics, Northwestern University, Children's Memorial Hospital Chicago, IL 60614, USA.

出版信息

Kidney Int. 1998 Sep;54(3):921-5. doi: 10.1046/j.1523-1755.1998.00066.x.

Abstract

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 (betaCaOx) and its determining factors would explain this discrepancy.

METHODS

Therefore, in addition to common biochemical measurements, plasma-oxalate (POx), citrate (PCit) and sulfate (PSulf) (plasma anions) were measured and betaCaOx was calculated in 17 PH-1 patients with normal renal function receiving pyridoxine and citrate therapy, in 54 children with CRI (SCr 0.9 to 5.9 mg/dl), and in 50 healthy children (NL). Plasma anions were analyzed by ion-chromatography and betaCaOx was calculated using a PC-based program for solution equilibria.

RESULTS

Compared to NL, all plasma anion levels and betaCaOx were higher in PH-1 and CRI; POx, PCit and betaCaOx were higher in PH-1 than in CRI (P < 0.05), but PSulf was higher in CRI (P < 0.01). BetaCaOx and POx were correlated in all groups (r = 0.63 to 0.95, P < 10(-4)). POx and betaCaOx were both inversely correlated to a decrease in GFR in CRI patients. PCit and PSulf did not influence betaCaOx. Although supersaturation (betaCaOx > 1) 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, POx and betaCaOx are elevated even with normal renal function, which increases the likelihood of CaOx crystal deposition. Therefore, more effective therapy to decrease betaCaOx 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 POx and betaCaOx levels, often to supersaturation levels.

摘要

背景

草酸钙(CaOx)沉积和全身性草酸中毒在慢性肾衰竭(CRI)儿童中并不常见,但在I型原发性高草酸尿症(PH - 1)儿童中却很常见。我们推测血浆CaOx饱和度(βCaOx)及其决定因素的差异可以解释这种差异。

方法

因此,除了常规生化检测外,我们还检测了17例接受吡哆醇和柠檬酸盐治疗且肾功能正常的PH - 1患者、54例CRI儿童(血清肌酐0.9至5.9mg/dl)和50例健康儿童(NL)的血浆草酸(POx)、柠檬酸盐(PCit)和硫酸盐(PSulf)(血浆阴离子),并计算βCaOx。血浆阴离子通过离子色谱法分析,βCaOx使用基于计算机的溶液平衡程序计算。

结果

与NL相比,PH - 1和CRI患者的所有血浆阴离子水平和βCaOx均较高;PH - 1患者的POx、PCit和βCaOx高于CRI患者(P < 0.05),但CRI患者的PSulf较高(P < 0.01)。所有组中βCaOx和POx均呈正相关(r = 0.63至0.95,P < 10⁻⁴)。CRI患者中POx和βCaOx均与肾小球滤过率降低呈负相关。PCit和PSulf不影响βCaOx。虽然在7例CRI患者和4例PH - 1患者中发现过饱和(βCaOx > 1),但仅在PH - 1患者中眼部检查怀疑有CaOx沉积,而1例PH患者因草酸骨病确诊为全身性草酸中毒。

结论

在PH - 1中,即使肾功能正常,POx和βCaOx也会升高,这增加了CaOx晶体沉积的可能性。因此,更有效的降低βCaOx的治疗对于降低全身性草酸中毒的风险至关重要。在CRI儿童中,未知但可能具有保护作用的物质有助于预防全身性草酸中毒的风险,尽管POx和βCaOx水平升高,且常常达到过饱和水平。

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