Dear ERKNet members, partners and friends,

We would like to take this occasion to thank you all for the good collaboration and the commitment of each and every one of you. Our network is continuously growing and the activities in the working groups and task forces are deepening and becoming more complex.

In this past year we have focused strongl​y on patient engagement, the further development of the ERKReg Registry and subregistries, the development of guidelines and the establishment of the ERKNet Postgraduate Curriculum in Rare Kidney Disease.

We are about​ to welcome 31 new members to our Network. This profound expansion will further empower us to accomplish our mission of promoting education, research, and the quality of care for rare kidney disorders throughout Europe.

We cautiously hope to finally see you in person again in 2022 for our 6th Annual Meeting. We wish you safe and peaceful holidays with your families and a hopeful outlook for the coming year.

Happy Holidays from the ERKNet team!

SAVE THE DATE !

10-12 May, 2022 | 6th ERKNet Annual Meeting

Depending on the COVID situation we will meet on-site/hybrid or virtual. 

NEW LOGO !

We are pleased to announce that ERKNet has a new official logo . Feel free to include the new logo into your presentations related to ERKNet. You can download the file in the member protected area.

After a two-year evaluation process, the Board of Member States and the European Commission have finally approved ERKNet membership for 31 new specialized centres.

As of 1st January 2022, the network's coverage will increase from 12 to 24 EU countries, with a total of 72 centres taking care of 70,000 patients.

We welcome all new ERKNet members and are looking forward to a fruitful collaboration.

• In October, the ePag chairmanship was handed over to Susana Carvajal Arjona and Uwe Korst. They are replacing Claudia, who has greatly contributed to many projects as the third ePAG chair.
• The first ERKNet-EURORDIS feedback session was launched in August to promote an effective patient-doctor partnership and a sense of shared responsibility in the Network. Among the outcomes of the session were the inclusion of the patient voice in training webinars and the organization of "Ask the Expert" sessions for patients with various diagnoses.
• The ERKNet Patient Website has been undergoing further improvements and is now available in 11 languages. 
• Standardized patient disease information materials are currently under development and will be followed by multilingual translations.
• This year, the ERKNet office designed a new patient brochure that was printed in 22 languages. The brochures will soon be sent to ERKNet members and patient organisations. You can also find the patient brochures on the member-protected website. 
  

POSTGRADUATE CURRICULUM IN RARE KIDNEY DISEASES

On January 1^st we have launched the first class of our new established ERKNet Postgraduate Curriculum. A total of 231 motivated students from 59 ERKNet healthcare providers in 22 EU countries have enrolled to the programme.

The ERKNet Postgraduate Curriculum is a 3-year structured online programme consisting of webinars and case-based eLearning covering the whole spectrum of rare kidney diseases. The webinars are presented twice a month by outstanding pediatric or adult nephrologists, nephropathologists and geneticists.  A total of 24 educational webinars and a virtual workshop on the fundamentals of pediatric dialysis with >4,400 live attendees has been organized in collaboration with ESPN, ERA, and IPNA.  

Additionally, we offer eLearning cases that present scenarios of patients with rare kidney diseases, allowing the students to establish a diagnosis and choose appropriate therapeutic strategies. After three years, participants receive a certificate as "Specialist in Rare Kidney Diseases".​  To date, 21 eLearning cases have been created and review by 26 senior ERKNet experts. 

Registration for the second class is open until December 31st. Learn more here.

CLINICAL EXCHANGE PROGRAMME

This year a new clinical exchange programme has launched aiming to harmonize specific knowledge and reduce gaps in expertise. This concept proposes either the exchange of expert knowledge by senior experts visiting other sites or the on-site training of junior professionals at experienced ERKNet centres. The program is open to all healthcare professionals (including nurses, technicians, etc.) and both junior and senior members are encouraged to participate.

This year 4 junior ERKNet experts had the opportunity to visit another ERKNet host institution.

An additional 6 exchanges were scheduled, but have been postponed due to the growing number of Covids in several countries. Presently, the programme is suspended, but will relaunch by May 2022.

To date, we have used almost half of all packages. If you are interested to participate in this programme, please find more information here.

In 2021, the European Registry for Rare Kidney Diseases (ERKReg) continued to grow steadily, with 150-200 new patients being registered weekly. To date, 13,000 patients have been enrolled in the only European registry. Currently, 27 ERKNet Full Members, 7 Affiliated Partners and 31 external centers are active ERKReg partners.

Our appreciation goes out to all ERKReg collaborators for their efforts and dedication that have made ERKReg the largest of the ERN registries and a unique resource for future clinical research.

The first ERKReg paper describing the design, objectives and initial results has been published in the Orphanet Journal of Rare Diseases. Further publications are in preparation. The second Annual Registry Report has been posted on the ERKReg website.

One of the tasks of ERKReg is to monitor and improve the quality and outcomes of patients in ERKNet Expert Centers. In August the first semi-annual reports on Key Peformance Indicators (KPI) have been sent out to the centers. We hope that these will help optimizing the quality of care throughout the Network.

In the past year, we have successfully launched two disease-specific sub-registries on the ERKReg platform:

• ERKNet/ESPN subregistry for Pediatric Lupus Nephritis
• Eurocys subregistry for Cystinuria

Together with the well established subregistry for distal renal tubular acidosis, these databases will allow valuable, more detailed analyses of treatment and outcomes. Several additional disease-specific sub-registries are currently being prepared and will be launched in 2022.

This year was another successful one for the members of ERKNet in developing clinical practice recommendations for rare kidney diseases. As a result of collaborations with the professional renal societies (ESPN, ERA, IPNA) and other ERNs, five clinical practice recommendations were published in high-ranking journals.

During the workgroup meetings, gap analyses disclosed the need for further clinical guidance documents. Thus, another seven projects are now being developed, and many more are in planning

The European Commission has launched the "ERN Guideline Programme" to assist the ERNs in developing, approving, and implementing high-quality clinical practice guidelines. ERKNet has been selected as one of the first ERNs to collaborate with OSTEBA, a Spanish Health Technology Assessment Agency, that carried out the literature search for the guideline project on tuberous sclerosis complex.

Published documents:

• Distal renal tubular acidosis (WG tubulopathies w/ESPN, lead: D. Bockenhauer. London)
• Diagnosis and Management of Bartter syndrome (WG tubulopathies, lead: M.Konrad, Münster)
• Congenital nephrotic syndrome – clininal aspects (WG inherited glomerulopathies w/ESPN, lead: O. Boyer, Paris)
• Nephrogenetic diagnostics (TF Molecular diagnostics w/ERA, lead: N. Knoers, Groningen)
• Consensus statement on standards and guidelines for the molecular diagnostics of Alport syndrome: refining the ACMG criteria (Alport working group w/ members of WG inherited glomerulopathies, leads: Judy Savige, Melbourne/B. Lipska, Gdansk)

Submitted:

• Renal dysplasia (WG CAKUT, lead: M. Liebau, Cologne)
• Posterior urethral valves (WG CAKUT w/eUROGEN, lead: G. Montini, Milano)

Under preparation:

• Bardet-Biedl-Syndrome (ERN-Eye w/members of WG renal malformaitons)
• Tuberous Sclerosis Complex (WG AD structural disorders, lead: D. Mekahli, Leuven)
• Dent disease (WG tubulopathies, lead: A. Bökenkamp, Amsterdam)
• Nephrogenic diabetes insipidus (WG tubulopathies, leads: N. Knoers, Groningen/E. Levtchenko, Leuven)
• Methylmalonic acidemia (WG metabolic nephropathies w/MetabERN, lead: Aude Servais)
• Monoclonal gammopathies of renal significance (WG immune glomerulopathies w/EuroBloodNet, lead: J. Wetzels, Nijmegen)
• Lipid Apheresis (WG Pediatric CKD/Dialysis, lead: C.P. Schmitt, Heidelberg)

On the ERKNet website under “guidelines&pathways” you can find all guidance documents that were developed by our Network, as well as those developed by our partner medical societies and endorsed by the Network Board.

The 5th ERKNet Annual Meeting took place virtually a second time due to the pandemic. Prior to the annual meeting, nine working group meetings were organized to reduce work load.

More than 260 participants from 68 centers attended the 3-day-long meeting via the Zoom platform.

The post-meeting survey revealed that 100% of meeting participants were satisfied with the quality of the meeting. Importantly, more than 85% of the respondents to our survey rated the Network progress as very good or excellent.

The top 3 most important activities of the Network were indicated by the survey participants as:

• Research initiatives
• Guideline development
• Educational activities

A number of online surveys on various aspects of different rare kidney disorders have been circulated among Network members in 2021 with excellent participation rates. Surveys on pregnancy outcomes of women with Alport syndrome (ALPART study), Dent's disease, HNF1B, pregnancy in Cystinosis patients, and Fanconi-Bickel syndrome are now being analyzed.

Thanks to all who contributed to the surveys!

Ongoing surveys – consider your contribution !

• Cinacalcet in infants
• NSAIDs in NDI and Bartter 
• PRDM15-related Galloway Mowat Syndrome

Final results of the 2020 study on COVID-19 in children treated with immunosuppressive medication for kidney diseases Archives of Disease in Childhood were published in Archives of Disease in Childhood.

Results of the survey on treatment and long-term outcome in primary nephrogenic diabetes insipidus were published in Nephrology Dialysis Transplantation,

The manuscript describing the results of the ERKNet/ESPN/IPNA study on Paediatric ANCA associated glomerulonephritis is in final stage and close to submission.