Abstract
Introduction
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Kidney cysts form over the course of the disease and kidney function slowly declines, usually leading to kidney failure in middle to late adulthood. However, some symptoms, such as hypertension or proteinuria, can be present at an earlier age. In this study, we aimed to quantify early complications in children over time.
Methods
All 69 children with ADPKD from our pediatric nephrology center who met inclusion criteria (follow-up ≥ 1 year and ≥ 2 recorded visits) were studied. Analysis of changes in kidney size, cyst count, estimated glomerular filtration rate (eGFR), urinary protein excretion, and blood pressure was performed.
Results
The median time of follow-up was 6.3 years (range 8.4–14.8). Over the follow-up, kidneys grew from 109 to 115% of expected length (p < 0.0001), number of cysts increased at a rate of 0.8 cyst/kidney/year, and the prevalence of hypertension increased significantly from 20 to 38% (p < 0.015). The eGFR and absolute urinary protein excretion remained stable.
Conclusions
This study shows that children with ADPKD suffer from increasing prevalence of hypertension during the course of the disease parallel to the increasing number of kidney cysts and size despite normal and stable kidney function and proteinuria.
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Acknowledgments
The authors thank the members of the Section Pediatric Nephrology of the Czech Pediatric Society for contributing cases: Dr. Adolfová, Dr. Barochová, Dr. Barošová, Dr. Bendáková, Dr. Biolek, Dr. M. Bláhová, Dr. Bosáková, Dr. Boumová, Dr. Brzobohatý, Dr. Doležalová, Prof. Doležel, Dr. Dostálková, Dr. Duroňová, Doc. Dušek, Dr. Flögelová, Dr. Geier, Dr. Gelnarová, Dr. Gut, Dr. Holá, Dr. Jiřičková, Dr. Kmoníčková, Dr. Kolský, Dr. Konečná, Dr. Kreisinger, Dr. Krupka, Dr. Kuběna, Dr. Kubín, Dr. Langer, Dr. Leichmannová, Dr. Libánská, Dr. Malina, Dr. Nebesář, Dr. Nedbalová, Dr. Němec, Dr. Němečková, Dr. Odzganová, Dr. Osvaldová, Dr. Pánek, Dr. Petrželka, Prof. Podracká, Dr. Prachařová, Dr. Prokopová, Dr. Rambousek, Dr. Rohanová, Dr. Rosenbergová, Dr. Rota, Dr. Ručková, Dr. Sadílková, Doc. Skálová, Dr. Sládková, Dr. Stupka, Dr. Svobodová, Dr. Šilhánková, Dr. Šikut, Dr. Šimánková, Dr. Šimková, Dr. Širůček, Dr. Šohajová, Dr. Štarha, Dr. Šuláková, Dr. Váchová, Dr. Valeš, Dr. Vondrák, Dr. Záhorský, Dr. Zaoral, Dr. Zasadilová, Dr. Žaba. The authors thank also Ass. Prof. Kotaška, Dr. Fořtová, Dr. Hříbal and Ing. Štekrová for clinical contributions and molecular genetic testing.
Funding
This work is supported by Ministry of Health, Czech Republic, conceptual development of research organization, and University Hospital Motol, Prague, Czech Republic 00064203.
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TS made substantial contributions to the conception and design of the work and to the acquisition, analysis, and interpretation of data and drafted the work; PJ, FF, and KB made contributions to the acquisition, analysis, and interpretation of data and approved the version to be published; AJ made contributions to the acquisition, analysis, and interpretation of data, drafted the work, and approved the version to be published.
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Ethical approval was waived by local Ethics Committee of University Hospital Motol in view of the retrospective nature of the study, and all the procedures being performed were part of the routine care.
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The authors declare no competing interests.
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Seeman, T., Jansky, P., Filip, F. et al. Increasing prevalence of hypertension during long-term follow-up in children with autosomal dominant polycystic kidney disease. Pediatr Nephrol 36, 3717–3723 (2021). https://doi.org/10.1007/s00467-021-05104-w
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DOI: https://doi.org/10.1007/s00467-021-05104-w