Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature
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ORIGINAL ARTICLE
Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature Loubna el Bakkali & Robert Rodrigues Pereira & Dirk J. Kuik & Johannes C. F. Ket & Joanna A. E. van Wijk
Received: 29 October 2010 / Revised: 27 February 2011 / Accepted: 28 February 2011 / Published online: 1 May 2011 # The Author(s) 2011. This article is published with open access at Springerlink.com
Abstract Nephrotic syndrome (NS) is a clinical diagnosis with proteinuria, hypoalbuminaemia and oedema. NS is rare in children, and its incidence in The Netherlands is unknown. The aim of this study was to estimate the incidence of idiopathic NS in the Netherlands. All paediatric patients (age 0–18 years) with a newly diagnosed NS in the Netherlands were registered by the Dutch Pediatric Surveillance Unit during the years 2003 until 2006, secondary NS was excluded. All paediatricians filled out questionnaires about the first clinical findings of the patients and incidences were calculated. A literature review on incidences of childhood NS was conducted. The incidence of NS in children in the Netherlands in the years 2003 until 2006 was 1.52/ 100, 000 children/ year. The median age at diagnosis was 3.88 years with a mean age of 5.08 years. A significant male:female ratio of 2.04:1 was found. This prospective study of NS in the Netherlands revealed an incidence of 1.52:100, 000 children/year, and is similar to the incidences found all over the world. L. el Bakkali (*) : J. A. E. van Wijk Department of Paediatric Nephrology, VU University Medical Center, PO Box 7057, 1007 MB, Amsterdam, The Netherlands e-mail: [email protected] R. Rodrigues Pereira Dutch Paediatric Surveillance Unit, Leiden, The Netherlands D. J. Kuik Department of Epidemiology and Biostatistics, VU University Medical Center, Amsterdam, The Netherlands J. C. F. Ket Medical Library, VU University, Amsterdam, The Netherlands
Keywords Children . Epidemiology . Incidence . Nephrotic syndrome . Prospective study . Review of literature
Introduction Nephrotic syndrome (NS) is the most common glomerular disorder of childhood. It is defined by proteinuria, hypoalbuminaemia, oedema and hyperlipidaemia [1]. Most (80%) children have a minimal-change NS (MCNS). Other diagnoses, which could be the cause of NS, are focal segmental glomerulosclerosis (FSGS), congenital NS (CNS) and NS due to glomerulonephritis or other glomerulopathies. Although NS is relatively rare, it accounts for mortality and considerable morbidity in young children. The prognosis of NS in children correlates with the spectrum of responsiveness to steroid therapy, from steroid-sensitive NS (SSNS) to steroid-resistant NS (SRNS). SRNS is the most common acquired cause of end-stage renal disease (ESRD) in children [2]. The aim of this study was to determine the incidence of NS in The Netherlands. All new cases of NS in The Netherlands of children 0–18 years of age were prospectively registered from 1 January 2003 till 1 January 2007. The second aim was to compare these results w
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