Dear colleagues,
we are excited to announce the release of the new ERKNet/ESPN/ERA guideline on the “Diagnosis, management and treatment of the Alport syndrome”.
The document is a joint initiative of ERKNet, ERA Genes&Kidney and ESPN WG Hereditary Kidney Disorders along with patient advocacy groups. A team of experts including adult and paediatric nephrologists, kidney geneticists, audiologists, ophthalmologists and a kidney pathologist describe the current state-of-art in the diagnosis, management and treatment of the disorders resulting from the genetic defects in COL4A3/4/5 genes including the classical Alport syndrome.
Key recommendations:
- Genetic testing for COL4A3/4/5 should be part of the initial workup for individuals with persistent hematuria, proteinuria, unexplained kidney failure, or FSGS of unknown oirigin and possibly cystic kidney disease.
- Early renin-angiotensin system (RAS) blockade is the standard treatment.
- SGLT2 inhibitors may be added for adults with proteinuria and chronic kidney disease.
- Heterozygous carriers should be considered for living kidney donation as the last possible resource, but may be acceptable under certain circumstances.
Watch the video abstract presented b Dr. Roser Torra here
Congratulations to all co-authors and collaborators!
Best wishes,
Your ERKNet Team
