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Seeman, Tomas; Jansky, Petr; Filip, Fencl; Blahova, Kveta und Jaros, Adam (2021): Increasing prevalence of hypertension during long-term follow-up in children with autosomal dominant polycystic kidney disease. In: Pediatric Nephrology, Bd. 36, Nr. 11: S. 3717-3723

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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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