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. 2022 Nov 29;16(6):985-995.
doi: 10.1093/ckj/sfac251. eCollection 2023 Jun.

Autosomal dominant polycystic kidney disease in young adults

Collaborators, Affiliations

Autosomal dominant polycystic kidney disease in young adults

Víctor Martínez et al. Clin Kidney J. .

Abstract

Background: The clinical manifestations of autosomal dominant polycystic kidney disease (ADPKD) usually appear in adulthood, however pediatric series report a high morbidity. The objective of the study was to analyze the clinical characteristics of ADPKD in young adults.

Methods: Family history, hypertension, albuminuria, estimated glomerular filtration rate (eGFR) and imaging tests were examined in 346 young adults (18-30 years old) out of 2521 patients in the Spanish ADPKD registry (REPQRAD). A literature review searched for reports on hypertension in series with more than 50 young (age <30 years) ADPKD patients.

Results: The mean age of this young adult cohort was 25.24 (SD 3.72) years. The mean age at diagnosis of hypertension was 21.15 (SD 4.62) years, while in the overall REPQRAD population was aged 37.6 years. The prevalence of hypertension was 28.03% and increased with age (18-24 years, 16.8%; 25-30 years, 36.8%). Although prevalence was lower in women than in men, the age at onset of hypertension (21 years) was similar in both sexes. Mean eGFR was 108 (SD 21) mL/min/1.73 m2, 38.0% had liver cysts and 3.45% of those studied had intracranial aneurysms. In multivariate analyses, hematuria episodes and kidney length were independent predictors of hypertension (area under the curve 0.75). The prevalence of hypertension in 22 pediatric cohorts was 20%-40%, but no literature reports on hypertension in young ADPKD adults were found.

Conclusions: Young adults present non-negligible ADPKD-related morbidity. This supports the need for a thorough assessment of young adults at risk of ADPKD that allows early diagnosis and treatment of hypertension.

Keywords: ADKPD; children; glomerular filtration rate; hypertension; young adults.

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Conflict of interest statement

V.M. has received speaker fees or travel support from Takeda, Amgen, Sanofi-Genzyme, Kyowa Kirin, Otsuka and Reata. A.O. is the former CKJ Editor-in-Chief and has received grants from Sanofi and consultancy or speaker fees or travel support from Advicenne, Astellas, AstraZeneca, Amicus, Amgen, Fresenius Medical Care, GSK, Bayer, Sanofi-Genzyme, Menarini, Mundipharma, Kyowa Kirin, Alexion, Freeline, Idorsia, Chiesi, Otsuka, Novo-Nordisk, Sysmex and Vifor Fresenius Medical Care Renal Pharma, and is Director of the Catedra Mundipharma-UAM of diabetic kidney disease and the Catedra Astrazeneca-UAM of chronic kidney disease and electrolytes. R.T. is an Associate Editor for CKJ and has received consultancy or speaker fees or travel support from Advicenne, AstraZeneca, Amicus, Amgen, Sanofi-Genzyme, Kyowa Kirin, Alexion, Chiesi, Otsuka and Reata.

Figures

Graphical Abstract
Graphical Abstract
Figure 1:
Figure 1:
Prevalence of hypertension in 22 studies from the literature reporting the prevalence of hypertension in >50 young ADPKD patients. A metanalysis or 14 prior studies is indicated. Details of the 22 studies are shown in Supplementary data, Table S2. Note that most reports refer to children or very young adults (≤21 years old).
Figure 2:
Figure 2:
Prevalence of hypertension in ADPKD patients by age group in REPQRAD.

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