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Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group

Boyer, O; Schaefer, F; Haffner, D; Bockenhauer, D; Holtta, T; Berody, S; Webb, H; ... Vivarelli, M; + view all (2021) Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group. Nature Reviews Nephrology , 17 pp. 277-289. 10.1038/s41581-020-00384-1. Green open access

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Abstract

Congenital nephrotic syndrome (CNS) is a heterogeneous group of disorders characterized by nephrotic-range proteinuria, hypoalbuminaemia and oedema, which manifest in utero or during the first 3 months of life. The main cause of CNS is genetic defects in podocytes; however, it can also be caused, in rare cases, by congenital infections or maternal allo-immune disease. Management of CNS is very challenging because patients are prone to severe complications, such as haemodynamic compromise, infections, thromboses, impaired growth and kidney failure. In this consensus statement, experts from the European Reference Network for Kidney Diseases (ERKNet) and the European Society for Paediatric Nephrology (ESPN) summarize the current evidence and present recommendations for the management of CNS, including the use of renin–angiotensin system inhibitors, diuretics, anticoagulation and infection prophylaxis. Therapeutic management should be adapted to the clinical severity of the condition with the aim of maintaining intravascular euvolaemia and adequate nutrition, while preventing complications and preserving central and peripheral vessels. We do not recommend performing routine early nephrectomies but suggest that they are considered in patients with severe complications despite optimal conservative treatment, and before transplantation in patients with persisting nephrotic syndrome and/or a WT1-dominant pathogenic variant.

Type: Article
Title: Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group
Open access status: An open access version is available from UCL Discovery
DOI: 10.1038/s41581-020-00384-1
Publisher version: https://doi.org/10.1038/s41581-020-00384-1
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
Keywords: Focal segmental glomerulosclerosis, Paediatric kidney disease
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Renal Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10124512
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