Overview ERKNet Guideline Projects

Updated 12 May 2025

Please find below the overview of all published, ongoing and planned guidelines

Title

Journal

Author

WG/TF/Collaboration

Diagnosis, management and treatment of the Alport syndrome

Nephrol Dial Transplant

2025

Torra et al.

Hereditary Glomerulopathies

ERA

ESPN

Diagnosis and management of spinal dysraphism in children and adolescentsEAU Guidelines Office 2025K. Abrahamson et al. 

eUROGEN

ERN-ITHACA

EAU

ESPU

Dent disease: Clinical Practice RecommendationsNephrol Dial Transplant 2025D. Bökenkamp et al. 

Metabolic Nephropathies

Tubulopathies

ESPN

International Expert Consensus Statement on the Diagnosis and Management of Congenital Nephrogenic Diabetes Insipidus (arginine vasopressin resistance)Nature Review Nephrology 2024E. Levtchenko et al.

Tubulopathies

ESPN

Clinical Practice Recommendations on Kidney Management in Methylmalonic Acidemia: an Expert Consensus Statement From ERKNet and MetabERNKidney International Reports 2024A. Servais et al.

Metabolic and Stone Disorders, Tubulopathies

Metab-ERN

Clinical practice recommendations for kidney involvement in tuberous sclerosis complex: a consensus statement by the ERKNet Working Group for Autosomal Dominant Structural Kidney Disorders and the ERA Genes & Kidney Working GroupNature Reviews Nephrology 2024D. Mekahli et al.

AD structural kidney disorders

ERA

Clinical practice recommendations on lipoprotein apheresis for children with homozygous familial hypercholesterolemia: an expert consensus statement from ERKNet and ESPN

 

Atherosclerosis, Volume 392, May 2024, 117525D. Reijman et al.

Pediatric CKD/Dialysis

ESPN

Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope

 

Nature Reviews Nephrology  2023 ;
19 : 194–211

 

J.W.Groothoff et al.

Metabolic and Stone Disorders

Oxaleurope

An update on the use of tolvaptan for autosomal dominant polycystic kidney disease: consensus statement on behalf of the ERA Working Group on Inherited Kidney Disorders, the European Rare Kidney Disease Reference Network and Polycystic Kidney Disease InternationalNephrol Dial Transplant (2022) 37: 825–839R. Müller et al

AD structural kidney disorders

ERA

Definition, diagnosis and management of fetal lower urinary tract obstructionNat Rev Urol. 2022 Feb 8V. Capone et al.CAKUT and obstructive nephropathies

Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations

 

Nephrol Dial Transplant 2022: 0: 1–12

 

S.Kohl et al.CAKUT and obstructive nephropathies

Genetic testing in the diagnosis of chronic kidney disease: recommendations for clinical practice

 

Nephrol Dial Transplant. 2022;
37: 239–254.
N.Knoers et al.TF Molecular Diagnostics
Cystinuria : clinical practice recommendation

Kidney International 2021; 99: 48–58

 

A.Servais et al.Metabolic and Stone Disorders

Distal renal tubular acidosis: ERKNet/ESPN clinical practice points

 

Nephrol Dial Transplant 2021; 36: 1585–1596

 

F. Trepiccione et al.

Tubulopathy

ESPN

Clinical practice recommendations for recurrence of focal and segmental glomerulosclerosis/steroid-resistant nephrotic syndrome

 

Pediatr Transplant

. 2021 May;25(3):e13955.

L. Weber et al.

Pediatric Tx

CERTAIN

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

 

Nature Reviews Nephrology 2021; 17

 

O.Boyer et al.

Hereditary Glomerulopathies

ESPN

Genetic aspects of congenital nephrotic syndrome: a consensus statement from the ERKNet–ESPN inherited glomerulopathy working groupEuropean Journal of Human Genetics 2020B. Lipska-Zietkiewicz et al.Hereditary Glomerulopathies
ESPN
TopicWorking GroupCollaborationStatus
 Cardiovascular evaluation and management in ped CKDPedCKD&DialysisESPNPaper in final preparation
 Attenuation of CKD progressionPedCKD&DialysisESPNPaper in preparation
ARPKDCAKUT and CiliopathiesESPNDelphi Round
 Monoclonal gammopathies of renal significanceImmune glomerulopathiesEuroBloodNetPaper in preparation
 Lowe Syndrome Metabolic nephropathies Writing draft recommendations
William Beuren SyndromeCAKUT and CiliopathiesERN-ITHACAWriting draft recommendation
cTTPTMA Writing draft recommendation
 HypomagnesemiaTubulopathiesERALiterature screening
 Megaureter CAKUT and CiliopathieseUROGENLiterature screening
NephrocalcinosisTubulopathies Literature screening
KRTPediatric CKD/Dialysis Litrature screenining
Postnatal PUVCAKUT/CiliopathieseUROGENIn planning
VURCAKUT/Ciliopathies In planning for 2025

update 20.11.2014

Topic

Working Group

Collaboration

Status

Postnatal PUV

CAKUT/Ciliopathies

eUROGEN

In planning

VUR

CAKUT/Ciliopathies

 

In planning