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Nutcracker syndrome in pediatrics: initial findings and long-term follow-up results

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Abstract

Background

Nutcracker syndrome (NCS) describes a set of symptoms and signs resulting from compression of the left renal vein (LRV). There is a lack of knowledge about its natural course, diagnosis, and management, especially in children. Herein, we present our single-center experience with a large number of patients who have long-term follow-up results.

Methods

All patients with NCS diagnosed between January 2011 and March 2021 were included and their data were obtained retrospectively.

Results

A total of 123 NCS patients (85 females) were included. The median age at the time of diagnosis was 12 (IQR 10–14) years, and BMI percentiles were below 5% in 38% of the cases. At the time of diagnosis, two-thirds of the patients were asymptomatic. The most common laboratory finding was nephritic proteinuria (98%), followed by microscopic hematuria (16%). Signs of LRV compression were significantly more evident in upright position Doppler ultrasonography (DUS) examination. All patients have been followed conservatively; hematuria and/or proteinuria resolved in 43 of the 108 patients (40%) within 35.8 ± 25.8 months of follow-up. Control DUS was performed in 52 patients after a mean period of 39.1 ± 21.3 months. The median peak velocity and diameter ratios of the LRV in the upright position were found to be decreased significantly when compared to the initial assessment (p < 0.05). Normal DUS findings were noted in 13 patients at the final evaluation.

Conclusions

In unexplained proteinuria and/or hematuria, NCS should be considered, especially in asthenic adolescents. Our results support conservative management in children as the first-line treatment approach.

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Data availability

All data generated or analyzed during this study are included in this article. Further inquiries can be directed to the corresponding author.

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Authors and Affiliations

  1. Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey

    Iryna Akdemir

  2. Division of Pediatric Nephrology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey

    Ece Mekik Akar, Songül Yılmaz, Nilgün Çakar & Zeynep Birsin Özçakar

  3. Division of Pediatric Radiology, Department of Radiology, Ankara University School of Medicine, Ankara, Turkey

    Suat Fitöz

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  1. Iryna Akdemir
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Contributions

Iryna Akdemir designed the study, performed data collection, interpreted the results, and drafted the manuscript. Ece Mekik Akar contributed to data collection, performed statistical data analysis, interpreted the results, and drafted the manuscript. Songül Yılmaz contributed to data collection and provided both scientific and grammatical revisions to the manuscript. Nilgün Çakar provided both scientific and grammatical revisions to the manuscript. Suat Fitöz interpreted the results and provided both scientific and grammatical revisions to the manuscript. Zeynep Birsin Özçakar designed the study, interpreted the results, and provided both scientific and grammatical revisions to the manuscript. All authors have read and approved the final version of the manuscript.

Corresponding author

Correspondence to Zeynep Birsin Özçakar.

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The study was approved by the Ethics Committee of Ankara University School of Medicine with a report numbered I5-338–21.

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The authors declare no competing interests.

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Akdemir, I., Mekik Akar, E., Yılmaz, S. et al. Nutcracker syndrome in pediatrics: initial findings and long-term follow-up results. Pediatr Nephrol 39, 799–806 (2024). https://doi.org/10.1007/s00467-023-06150-2

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