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Pediatr Nephrol. 2016 Dec;31(12):2317-2325. Epub 2016 Oct 20.

Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry.

Author information

1
Department of Pediatric Nephrology, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
2
ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, PO Box 22700, 1100 DE, Amsterdam, The Netherlands. m.bonthuis@amc.uva.nl.
3
ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, PO Box 22700, 1100 DE, Amsterdam, The Netherlands.
4
Department of Pediatrics, Rikshospitalet, Oslo University Hospital, Oslo, Norway.
5
Department of Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
6
Nephrogenetics Laboratory, Department of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
7
Department of Pediatrics, Bordeaux University Hospital, Bordeaux, France.
8
Department of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, University of Heidelberg, Heidelberg, Germany.
9
Department of Pediatric Nephrology, Emma Children's Hospital AMC, University of Amsterdam, Amsterdam, The Netherlands.

Abstract

BACKGROUND:

Congenital nephrotic syndrome (CNS) of the Finnish type, NPHS1, is the most severe form of CNS. Outcomes of renal replacement therapy (RRT) in NPHS1 patients in Europe were analysed using data from the ESPN/ERA-EDTA Registry. As NPHS1 is most prevalent in Finland and the therapeutic approach differs from that in many other countries, we compared outcomes in Finnish and other European patients.

METHODS:

NPHS1 mutations were confirmed in 170 children with CNS who initiated RRT (dialysis or renal transplantation) between 1991 and 2012. Finnish (n = 66) and non-Finnish NPHS1 patients (n = 104) were compared with respect to treatment policy, age at first RRT and renal transplantation (RTX), patient and graft survival, estimated glomerular filtration rate (eGFR) and growth. Age-matched patients with congenital anomalies of the kidney and urinary tract (CAKUT) served as controls.

RESULTS:

Finnish NPHS1 patients were significantly younger than non-Finnish patients, both at the start of RRT and at the time of RTX. We found similar overall 5-year patient survival on RRT (91 %) and graft survival (89 %) in both NPHS1 groups and CAKUT controls. At the start of RRT, height standard deviation score (SDS) was higher in Finnish patients than in non-Finnish patients (mean [95 % CI]: -1.31 [-2.13 to -0.49] and -3.0 [-4.22 to -1.91], p < 0.01 respectively), but not at 5 years of age. At 5 years of age height and body mass index (BMI) SDS were similar to those of CAKUT controls.

CONCLUSIONS:

Overall, 5-year patient and graft survival of both Finnish and non-Finnish NPHS1 patients on RRT were excellent and comparable with CAKUT patients with equally early RRT onset and was independent of the timing of RRT initiation and RTX.

KEYWORDS:

Congenital nephrotic syndrome; Dialysis; Graft survival; Kidney transplantation; NPHS1; Pediatrics

PMID:
27761660
DOI:
10.1007/s00467-016-3517-z
[Indexed for MEDLINE]

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