BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease is the most common inheritable kidney disease, frequently thought to become symptomatic in adulthood. However, patients with autosomal dominant polycystic kidney disease may develop signs or symptoms during childhood, in particular hypertension. Although ambulatory BP monitoring is the preferred method to diagnose hypertension in pediatrics, data in children with autosomal dominant polycystic kidney disease are limited. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Our retrospective multicenter study was conducted to collect ambulatory BP monitoring recordings from patients with autosomal dominant polycystic kidney disease age <18 years old. Basic anthropometric parameters as well as data on kidney function, BP treatment, and kidney ultrasound were also collected. RESULTS: Data from 310 children with autosomal dominant polycystic kidney disease with a mean age of 11.5±4.1 years old were collected at 22 European centers. At the time when ambulatory BP monitoring was performed, 95% of children had normal kidney function. Reference data for ambulatory BP monitoring were available for 292 patients. The prevalence rates of children with hypertension and/or those who were treated with antihypertensive drugs were 31%, 42%, and 35% during daytime, nighttime, or the entire 24-hour cycle, respectively. In addition, 52% of participants lacked a physiologic nocturnal BP dipping, and 18% had isolated nocturnal hypertension. Logistic regression analysis showed a significant association between a categorical cyst score that was calculated on the basis of the number of cysts >1 cm per kidney and daytime hypertension (odds ratio, 1.70; 95% confidence interval, 1.21 to 2.4; P=0.002), nighttime hypertension (odds ratio, 1.31; 95% confidence interval, 1.05 to 1.63; P=0.02), or 24-hour hypertension (odds ratio, 1.39; 95% confidence interval, 1.08 to 1.81; P=0.01). Kidney length, expressed as SD score, was also significantly associated with nighttime hypertension (odds ratio, 1.23; 95% confidence interval, 1.06 to 1.42; P=0.10). CONCLUSIONS: These data indicate high prevalence of hypertension in children with autosomal dominant polycystic kidney disease starting at young ages.

Arterial Hypertension Unit Department of Pediatric Cardiology and Cardiac Surgery Bambino Gesù Children's Hospital Istituto di Ricovero e Cura a Carattere Scientifico Rome Italy

Centro Materno Infantil do Norte Centro Hospitalar do Porto Abel Salazar Biomedical Sciences Institute University of Porto Porto Portugal

Department of Inherited and Acquired Kidney Diseases Research and Clinical Institute for Pediatrics Pirogov Russian National Research Medical University Moscow Russia

Department of Medical Biometry Institute of Medical Biometry and Informatics University of Heidelberg Heidelberg Germany

Department of Nephrology Kidney Transplantation and Arterial Hypertension The Children's Memorial Health Institute Warsaw Poland

Department of Pediatric Nephrology Assistance Publique Hôpitaux de Paris Necker Enfant Malades Descartes University Paris France

Department of Pediatric Nephrology Cerrahpasa Faculty of Medicine Istanbul University Istanbul Turkey

Department of Pediatric Nephrology Medical University in Lublin Lublin Poland

Department of Pediatric Nephrology University Hospitals Leuven Leuven Belgium

Department of Pediatrics 2nd Medical Faculty University Hospital Motol Charles University Prague Prague Czech Republic

Department of Pediatrics and Nephrology Medical University of Warsaw Warsaw Poland

Department of Pediatrics Cliniques Universitaires Saint Luc Université Catholique de Louvain Brussels Belgium

Department of Pediatrics of the Faculty of Medicine Comenius University in Bratislava and the University Children's Hospital Bratislava Bratislava Slovakia

Division of Nephrology Cliniques Universitaires Saint Luc Université Catholique de Louvain Brussels Belgium

Division of Nephrology Department of Pediatric Subspecialties and

Division of Pediatric Nephrology and Rheumatology Department of Pediatrics Ankara University Medical School Ankara Turkey

Division of Pediatric Nephrology Assistance Publique Hôpitaux de Paris Robert Debré Sorbonne University Paris France

Division of Pediatric Nephrology Department of Pediatrics Faculty of Medicine Hacettepe University Ankara Turkey

Institute of Clinical Medicine Vilnius University Vilnius Lithuania

Institute of Physiology University of Zurich Zurich Switzerland; and

Nephrology Department University Children's Hospital and School of Medicine University of Belgrade Serbia

Nephrology Department University Children's Hospital Belgrade Serbia

Pediatric Nephrology Department Fundeni Clinical Institute Bucharest Romania

Pediatric Nephrology Division Center for Pediatrics and Adolescent Medicine Heidelberg University Hospital Heidelberg Germany

Pediatric Nephrology Service University Hospital Vall d'Hebrón Universidad Autonoma de Barcelona Barcelona Spain

Pediatric Nephrology Unit Bordeaux University Hospital Institut National de la Santé et de la Recherche Médicale Unité Mixte de Recherche 1219 Bordeaux France

Pediatric Nephrology Unit Centro Hospitalar São João Porto Portugal

Polycystic Kidney Disease Research Group Laboratory of Pediatrics Department of Development and Regeneration Gynaecology Pediatrics and Urology Katholieke Universiteit Leuven Leuven Belgium

Reference Centre of Hereditary Renal Diseases of the Child and Adult Assistance Publique Hôpitaux de Paris Necker Enfants Malades Paris France

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