Laura Massella1, Djalila Mekahli2,3, Dušan Paripović4, Larisa Prikhodina5, Nathalie Godefroid6, Anna Niemirska7, Ayşe Ağbaş8, Karolina Kalicka9, Augustina Jankauskiene10, Malgorzata Mizerska-Wasiak11, Alberto Caldas Afonso12, Rémi Salomon13,14, Georges Deschênes15, Gema Ariceta16, Z Birsin Özçakar17, Ana Teixeira18, Ali Duzova19, Jérôme Harambat20, Tomáš Seeman21, Gabriela Hrčková22, Adrian Catalin Lungu23, Svetlana Papizh5, Amira Peco-Antic24, Stéphanie De Rechter2,3, Ugo Giordano25, Marietta Kirchner26, Teresa Lutz27, Franz Schaefer27, Olivier Devuyst28,29, Elke Wühl27, Francesco Emma30. 1. Division of Nephrology, Department of Pediatric Subspecialties, and. 2. Polycystic Kidney Disease Research Group, Laboratory of Pediatrics, Department of Development and Regeneration, Gynaecology Pediatrics and Urology (G-PURE), Katholieke Universiteit Leuven, Leuven, Belgium. 3. Department of Pediatric Nephrology, University Hospitals Leuven, Leuven, Belgium. 4. Nephrology Department, University Children's Hospital, Belgrade, Serbia. 5. Department of Inherited and Acquired Kidney Diseases, Research and Clinical Institute for Pediatrics, Pirogov Russian National Research Medical University, Moscow, Russia. 6. Department of Pediatrics, Cliniques Universitaires Saint Luc, Université Catholique de Louvain, Brussels, Belgium. 7. Department of Nephrology, Kidney Transplantation and Arterial Hypertension, The Children's Memorial Health Institute, Warsaw, Poland. 8. Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey. 9. Department of Pediatric Nephrology, Medical University in Lublin, Lublin, Poland. 10. Institute of Clinical Medicine, Vilnius University, Vilnius, Lithuania. 11. Department of Pediatrics and Nephrology, Medical University of Warsaw, Warsaw, Poland. 12. Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Abel Salazar Biomedical Sciences Institute, University of Porto, Porto, Portugal. 13. Department of Pediatric Nephrology, Assistance Publique Hôpitaux de Paris, Necker Enfant Malades, Descartes University, Paris, France. 14. Reference Centre of Hereditary Renal Diseases of the Child and Adult, Assistance Publique Hôpitaux de Paris, Necker Enfants Malades, Paris, France. 15. Division of Pediatric Nephrology, Assistance Publique Hôpitaux de Paris, Robert Debré, Sorbonne University, Paris, France. 16. Pediatric Nephrology Service, University Hospital Vall d'Hebrón, Universidad Autonoma de Barcelona, Barcelona, Spain. 17. Division of Pediatric Nephrology and Rheumatology, Department of Pediatrics, Ankara University Medical School, Ankara, Turkey. 18. Pediatric Nephrology Unit, Centro Hospitalar São João, Porto, Portugal. 19. Division of Pediatric Nephrology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey. 20. Pediatric Nephrology Unit, Bordeaux University Hospital, Institut National de la Santé et de la Recherche Médicale Unité Mixte de Recherche 1219, Bordeaux, France. 21. Department of Pediatrics, 2nd Medical Faculty, University Hospital Motol, Charles University Prague, Prague, Czech Republic. 22. Department of Pediatrics of the Faculty of Medicine, Comenius University in Bratislava and the University Children's Hospital Bratislava, Bratislava, Slovakia. 23. Pediatric Nephrology Department, Fundeni Clinical Institute, Bucharest, Romania. 24. Nephrology Department, University Children's Hospital and School of Medicine, University of Belgrade, Serbia. 25. Arterial Hypertension Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy. 26. Department of Medical Biometry, Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany. 27. Pediatric Nephrology Division, Center for Pediatrics and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany. 28. Institute of Physiology, University of Zurich, Zurich, Switzerland; and. 29. Division of Nephrology, Cliniques Universitaires Saint Luc, Université Catholique de Louvain, Brussels, Belgium. 30. Division of Nephrology, Department of Pediatric Subspecialties, and francesco.emma@opbg.net.
Abstract
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.
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.
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