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肾磷阈在儿童X-连锁低磷性佝偻病诊治中的临床价值

Clinical value of renal phosphorus threshold in the diagnosis and treatment X-linked hypophosphatemic rickets in children
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摘要 目的探讨肾磷阈,即肾小管最大磷重吸收率与肾小球滤过率的比值(ratio of tubular maximum reabsorption of phosphate to glomerular filtration rate,TmP/GFR)在X-连锁低磷性佝偻病(X-linked hypophosphatemic rickets,XLH)患儿诊治中的临床价值。方法回顾性纳入2010年1月—2023年1月在南京医科大学附属儿童医院初诊为XLH的83例患儿,收集初诊及随访数据,探讨TmP/GFR与佝偻病严重程度、钙磷代谢指标及磷酸盐治疗量的相关性。根据是否发生肾钙质沉着症将患儿分为肾钙质沉着组(n=47)和非肾钙质沉着组(n=36),比较两组患儿的临床资料。采用多因素logistic回归分析探讨XLH患儿并发肾钙质沉着症的影响因素。使用受试者操作特征曲线(receiver operating characteristic curve,ROC曲线)评估TmP/GFR对XLH患儿并发肾钙质沉着症的预测价值。结果83例XLH患儿初诊时TmP/GFR为(0.78±0.21)mmol/L,个体差异很大(范围:0.28~1.24 mmol/L)。TmP/GFR与XLH患儿佝偻病严重程度无显著相关性(P>0.05)。甲状旁腺激素与TmP/GFR呈负相关(rs=-0.020,P=0.008),血磷(rs=0.384,P<0.001)、血钙(rs=0.251,P<0.001)及25羟维生素D(rs=0.179,P<0.001)与TmP/GFR呈正相关,TmP/GFR与碱性磷酸酶(rs=-0.002,P=0.960)及磷元素治疗剂量(rs=0.012,P=0.800)无显著相关性。肾钙质沉着组的血钙和TmP/GFR均明显低于非肾钙质沉着组(P<0.05),而甲状旁腺激素和尿钙浓度均明显高于非肾钙质沉着组(P<0.05)。多因素logistic回归分析显示,TmP/GFR和尿钙浓度与XLH患儿并发肾钙质沉着症密切相关(P<0.05)。ROC曲线分析显示,TmP/GFR、尿钙浓度以及两者联合检测预测XLH患儿并发肾钙质沉着症的曲线下面积分别为0.696、0.679、0.761。结论TmP/GFR可以作为诊断儿童XLH的一项重要指标,然而它并不具备反映佝偻病严重程度及活动性的能力,无法作为判断传统治疗疗效的指标。尿钙浓度和TmP/GFR对XLH患儿并发肾钙质沉着症具有良好的预测价值,可为临床评估XLH患儿发生肾钙质沉着症的风险提供参考。 Objective To explore the clinical value of the renal phosphorus threshold(ratio of tubular maximum reabsorption of phosphate to glomerular filtration rate,TmP/GFR)in the diagnosis and treatment of children with Xlinked hypophosphatemic rickets(XLH).Methods A retrospective study was conducted,including 83 children diagnosed with XLH at Children's Hospital of Nanjing Medical University from January 2010 to January 2023.Initial diagnosis and follow-up data were collected to investigate the correlation of TmP/GFR with the severity of rickets,calcium and phosphorus metabolism indicators,and the dosage of phosphate treatment.Children were divided into two groups based on the occurrence of renal calcification:the renal calcification group(n=47)and the non-renal calcification group(n=36).Clinical data between the two groups were compared.Multivariate logistic regression analysis was used to identify factors influencing renal calcification in XLH children.The predictive value of TmP/GFR for renal calcification in XLH children was evaluated using receiver operating characteristic(ROC)curves.Results In the 83 XLH children,the initial TmP/GFR was(0.78±0.21)mmol/L,with significant individual variation(range:0.28-1.24 mmol/L).TmP/GFR showed no significant correlation with the severity of rickets(P>0.05).Parathyroid hormone was negatively correlated with TmP/GFR(rs=-0.020,P=0.008),while blood phosphorus(rs=0.384,P<0.001),blood calcium(rs=0.251,P<0.001),and 25-hydroxyvitamin D(rs=0.179,P<0.001)were positively correlated with TmP/GFR.No significant correlation was found between TmP/GFR and alkaline phosphatase(rs=-0.002,P=0.960)or phosphate treatment dosage(rs=0.012,P=0.800).Blood calcium and TmP/GFR levels were significantly lower in the renal calcification group than in the non-renal calcification group(P<0.05),while parathyroid hormone and urine calcium levels were significantly higher in the renal calcification group(P<0.05).Multivariate logistic regression analysis indicated that TmP/GFR and urine calcium levels were closely associated with renal calcification in XLH children(P<0.05).ROC curve analysis revealed that the areas under the curve for TmP/GFR,urine calcium,and their combined detection predicting renal calcification in XLH children were 0.696,0.679,and 0.761,respectively.Conclusions TmP/GFR may serve as an important diagnostic indicator for pediatric XLH;however,it does not reflect the severity or activity of rickets and cannot be used to judge the efficacy of traditional treatment.Urine calcium and TmP/GFR are valuable predictors for renal calcification in XLH children.
作者 陶佳琪 陈颖 TAO Jia-Qi;CHEN Ying(Department of Nephrology,Children's Hospital of Nanjing Medical University,Nanjing 210008,China)
出处 《中国当代儿科杂志》 CAS CSCD 北大核心 2024年第9期926-932,共7页 Chinese Journal of Contemporary Pediatrics
关键词 X-连锁低磷性佝偻病 肾磷阈 肾钙质沉着症 儿童 X-linked hypophosphatemic rickets Renal phosphorus threshold Renal calcification Child
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