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肾脏融合异常患儿的结局:来自印度北部的单中心经验

Outcome of children with fusion anomalies of the kidney: a single center experience from North India.

作者信息

Taneja Sanjana, Dhull Rachita Singh, Saha Abhijeet, Agarwal Prajal, Kapoor Kanika

机构信息

Division of Pediatric Nephrology, Department of Pediatrics, Lady Hardinge Medical College and associated Kalawati Saran Children's Hospital, Room No 102, New Delhi, 201010, India.

Department of Pediatrics, VMMC & Safdarjung Hospital, New Delhi, India.

出版信息

Int Urol Nephrol. 2025 Aug 12. doi: 10.1007/s11255-025-04700-1.

Abstract

BACKGROUND

Crossed fused ectopia (CFE) and horseshoe kidney (HSK) are the fusion anomalies of the kidney. Due to paucity of data on these disorders from India, we assessed the clinico-radiological profile and outcomes of these children.

MATERIALS AND METHODS

Clinical records of radiologically confirmed cases of CFE and HSK, attending the Pediatric nephrology clinic at a tertiary care center were reviewed retrospectively.

RESULTS

Thirty one cases of fusion disorders of the kidney were studied (16 CFE, 15 HSK). The median age at diagnosis was 84 (37-123.5) months. Non-specific abdominal pain was the most common presenting complaint (42%). There was a significant difference between mean estimated GFR (97.23 ± 31.76 ml/min/1.73m and mean diethylenetriamine pentaacetate (DTPA) GFR (78.18 ± 21.21 ml/min) at presentation (p < 0.05). Hydronephrosis was seen in 13 (42%) children (3 obstructive, 10 non-obstructive). Seven patients (22.5%) showed scarring on dimercapto succinic acid (DMSA) scan. Four patients (12.9%) had urological and five patients (16%) had associated non-urological anomalies. Only 2 (6.5%) patients had hypertension (stage 1) at presentation. Though five patients (n = 14) showed declining renal function at the latest follow-up, there was no significant difference in the mean eGFR of the entire cohort at the one-year and the latest follow-up mark.

CONCLUSIONS

Most children with fusion anomalies of kidney are symptomatic at presentation and further treatment is guided by the presence of associated urological anomalies. These children have an overall good prognosis but close monitoring of renal functions is essential.

摘要

背景

交叉融合异位肾(CFE)和马蹄肾(HSK)是肾脏的融合异常。由于来自印度的关于这些疾病的数据匮乏,我们评估了这些儿童的临床放射学特征及预后。

材料与方法

回顾性分析了一家三级医疗中心儿科肾脏病门诊经放射学确诊的CFE和HSK病例的临床记录。

结果

研究了31例肾脏融合异常病例(16例CFE,15例HSK)。诊断时的中位年龄为84(37 - 123.5)个月。非特异性腹痛是最常见的就诊主诉(42%)。就诊时平均估算肾小球滤过率(97.23±31.76 ml/min/1.73m²)与平均二乙三胺五乙酸(DTPA)肾小球滤过率(78.18±21.21 ml/min)之间存在显著差异(p < 0.05)。13例(42%)儿童出现肾积水(3例梗阻性,10例非梗阻性)。7例患者(22.5%)在二巯基丁二酸(DMSA)扫描中显示瘢痕形成。4例患者(12.9%)有泌尿系统异常,5例患者(16%)有相关的非泌尿系统异常。仅2例(6.5%)患者在就诊时患有高血压(1期)。尽管5例患者(n = 14)在最近一次随访时肾功能下降,但整个队列在1年和最近一次随访时的平均估算肾小球滤过率无显著差异。

结论

大多数肾脏融合异常的儿童在就诊时有症状,进一步治疗取决于是否存在相关的泌尿系统异常。这些儿童总体预后良好,但密切监测肾功能至关重要。

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