Background <p>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.</p> Materials and methods <p>Clinical records of radiologically confirmed cases of CFE and HSK, attending the Pediatric nephrology clinic at a tertiary care center were reviewed retrospectively.</p> Results <p>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&#xa0;ml/min/1.73m<sup>2)</sup> and mean diethylenetriamine pentaacetate (DTPA) GFR (78.18 ± 21.21&#xa0;ml/min) at presentation (p &lt; 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.</p> Conclusions <p>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.</p>

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

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

摘要

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/1.73m2) 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.