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

Albumins / therapeutic use Antibiotic Prophylaxis Anticoagulants / therapeutic use Combined Modality Therapy Diuretics / therapeutic use Fluid Therapy Genetic Markers Genetic Testing Humans Infections / etiology Nephrectomy Nephrotic Syndrome / complications Thrombosis / etiology

Journal

Nature reviews. Nephrology
ISSN: 1759-507X
Titre abrégé: Nat Rev Nephrol
Pays: England
ID NLM: 101500081

Informations de publication

Date de publication:
04 2021
Historique:
accepted: 30 11 2020
pubmed: 31 1 2021
medline: 2 4 2021
entrez: 30 1 2021
Statut: ppublish

Résumé

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.

Identifiants

DOI: 10.1038/s41581-020-00384-1 PMID: 33514942 PMC: PMC8128706
pubmed: 33514942
doi: 10.1038/s41581-020-00384-1
pii: 10.1038/s41581-020-00384-1
pmc: PMC8128706
doi:

Substances chimiques

Albumins 0
Anticoagulants 0
Diuretics 0
Genetic Markers 0

Types de publication

Consensus Development Conference Journal Article Practice Guideline Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

277-289

Commentaires et corrections

Type : ErratumIn

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Auteurs

Olivia Boyer (O)

Department of Pediatric Nephrology, Reference center for Idiopathic Nephrotic Syndrome in Children and Adults, Imagine Institute, Paris University, Necker Hospital, APHP, Paris, France. olivia.boyer@aphp.fr.
Laboratory of Hereditary Kidney Diseases, Imagine Institute, INSERM U1163, Paris Descartes University, Paris, France. olivia.boyer@aphp.fr.
ORCID: 0000-0002-3957-1359

Franz Schaefer (F)

Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.

Dieter Haffner (D)

Department of Pediatric Kidney, Liver and Metabolic Diseases, Children's Hospital, Hannover Medical School, Hannover, Germany.
Center for Congenital Kidney Diseases, Center for Rare Diseases, Hannover Medical School, Hannover, Germany.
ORCID: 0000-0002-9601-7813

Detlef Bockenhauer (D)

UCL Department of Renal Medicine and Renal Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Tuula Hölttä (T)

Department of Pediatric Nephrology and Transplantation, The New Children's Hospital, HUS Helsinki University Hospital, Helsinki, Finland.

Sandra Bérody (S)

Department of Pediatric Nephrology, Reference center for Idiopathic Nephrotic Syndrome in Children and Adults, Imagine Institute, Paris University, Necker Hospital, APHP, Paris, France.

Hazel Webb (H)

UCL Department of Renal Medicine and Renal Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Marie Heselden (M)

Patient representative, London, UK.

Beata S Lipska-Zie Tkiewicz (BS)

Clinical Genetics Unit, Department of Biology and Medical Genetics, Medical University of Gdańsk, Gdańsk, Poland.
Centre for Rare Diseases, Medical University of Gdańsk, Gdańsk, Poland.
ORCID: 0000-0002-4169-9685

Fatih Ozaltin (F)

Department of Pediatric Nephrology and Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Ankara, Turkey.
ORCID: 0000-0003-1194-0164

Elena Levtchenko (E)

Division of Pediatric Nephrology, Department of Pediatrics, University Hospitals Leuven; Department of Development & Regeneration, University of Leuven, Leuven, Belgium.

Marina Vivarelli (M)

Division of Nephrology and Dialysis, Department of Pediatric Subspecialties, Bambino Gesù Pediatric Hospital Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Rome, Italy.

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Classifications MeSH

questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Albumines › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Thérapeutique › Traitement médicamenteux › Prémédication › Antibioprophylaxie › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Utilisations thérapeutiques › Agents hématologiques › Anticoagulants › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Thérapeutique › Association thérapeutique › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Effets physiologiques des médicaments › Diurétiques › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Thérapeutique › Traitement médicamenteux › Traitement par apport liquidien › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Phénomènes génétiques › Phénotype › Marqueurs génétiques › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Établissements, main d'oeuvre et services de soins de santé › Services de santé › Services de médecine préventive › Services de diagnostic › Dépistage génétique › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Infection › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Procédures de chirurgie opératoire › Procédures de chirurgie urogénitale › Procédures de chirurgie urologique › Néphrectomie › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Maladies urogénitales › Maladies urologiques › Maladies du rein › Néphrose › Syndrome néphrotique › Management of congenital nephrotic syndrome:...
questionsmedicales.fr › Maladies cardiovasculaires › Maladies vasculaires › Embolie et thrombose › Thrombose › Management of congenital nephrotic syndrome:...