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Blood pressure in children with renal cysts and diabetes syndrome

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Abstract

Cystic kidney diseases such as autosomal recessive or dominant polycystic kidney disease (ARPKD and ADPKD) are associated with high prevalence of arterial hypertension. On the contrary, studies on hypertension in children with renal cysts and diabetes (RCAD) syndrome caused by abnormalities in the HNF1B gene are rare. Therefore, the primary aim of our study was to investigate the prevalence of high blood pressure in children with RCAD syndrome due to HNF1B gene abnormalities and secondary to search for possible risk factors for development of high blood pressure. Data on all children with genetically proven RCAD syndrome from three pediatric nephrology tertiary centers were retrospectively reviewed (office blood pressure (BP), ambulatory blood pressure monitoring (ABPM), creatinine clearance, renal ultrasound, echocardiography, albuminuria/proteinuria). High blood pressure was defined as BP ≥ 95th percentile of the current ESH 2016 guidelines and/or by the use of antihypertensive drugs. Thirty-two children with RCAD syndrome were investigated. Three children received ACE inhibitors for hypertension and/or proteinuria. High blood pressure was diagnosed using office BP in 22% of the children (n = 7). In the 7 performed ABPM, 1 child (14%) was diagnosed with hypertension and one child with white-coat hypertension. Creatinine clearance, proteinuria, albuminuria, body mass index, enlargement, or hypodysplasia of the kidneys and prevalence of HNF1B-gene deletion or mutation were not significantly different between hypertensive and normotensive children.

Conclusion: High blood pressure is present in 22% of children with RCAD syndrome.

What is Known:

• Arterial hypertension is a common complication in children with polycystic kidney diseases.

What is New:

• High office blood pressure is present in 22% and ambulatory hypertension in 14% of children with renal cyst and diabetes (RCAD) syndrome.

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Acknowledgements

We would like to thank Jan Janda for critical reading of our manuscript.

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Authors and Affiliations

  1. Department of Pediatrics, 2nd Faculty of Medicine, Charles University Prague, Prague, Czech Republic

    Tomáš Seeman, Kveta Blahova, Filip Fencl & Stepanka Pruhova

  2. Department of Pediatrics, Friedrich-Schiller-University Jena, Jena, Germany

    Friederike Weigel & Ulrike John-Kroegel

  3. Department of Pediatrics, Dr. von Hauner Children´s Hospital, University Hospital, LMU Munich, Munich, Germany

    Tomáš Seeman, Katharina Hermes, Richard Klaus, Bärbel Lange-Sperandio & Veit Grote

Authors
  1. Tomáš Seeman
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  2. Friederike Weigel
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  3. Kveta Blahova
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  4. Filip Fencl
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  5. Stepanka Pruhova
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  6. Katharina Hermes
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  7. Richard Klaus
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  8. Bärbel Lange-Sperandio
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  9. Veit Grote
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  10. Ulrike John-Kroegel
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Contributions

TS and UJK made substantial contributions to the conception and design of the work. TS collected, analyzed, and interpreted the clinical data and wrote the manuscript; FW, BW, and FF collected the clinical data. KH, RK, and BLS collected clinical data and wrote part of the manuscript and VG made the statistical analyses.

Corresponding author

Correspondence to Tomáš Seeman.

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Ethics approval

The present study was conducted in accordance with the principles outlined in the Declaration of Helsinki with approval from the Ethics Committee of LMU Munich and CHU Prague. Formal written informed consent could not be obtained from the patients’ parents or patients due to the retrospective design of the study.

Conflict of interest

The authors declare no competing interests.

Additional information

Communicated by Gregorio Paolo Milani

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Seeman, T., Weigel, F., Blahova, K. et al. Blood pressure in children with renal cysts and diabetes syndrome. Eur J Pediatr 180, 3599–3603 (2021). https://doi.org/10.1007/s00431-021-04165-1

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  • DOI: https://doi.org/10.1007/s00431-021-04165-1

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