What is the Registry?

ERKReg is the European Rare Kidney Disease Registry.

This Registry records anonymised information on diagnosis, treatment, therapeutic outcome of all patients with rare and complex kidney diseases within the Network.

ERKReg also serves to monitor how well the patients are treated at the individual ERKNet centers by following indicators of patient wellbeing ensuring the excellence of care in all ERKNet center members.

Moreover the Registry allows to track the number of patients with individual rare kidney diseases treated at the ERKNet centers, and to identify and inform patients whenever new drugs and other treatment options become available.

What data is collected in ERKReg?

General information
Registration identification number Number = pseudonym
Gender  
Date of birth Month + year (no day)
Ethnicity For example: Caucasian/ Asian / African, etc

General information regarding the kidney disease
Date of first symptoms Day / Month / Year
Kidney function on that first day Laboratory results: serum creatinine
Other persons within the family with a kidney disease? Yes / No
Clinical diagnosis A number from orphanet will be registered (orphanet is a database of all possible diagnoses)
Website orphanet: https://orpha.net/..

Specific information regarding the kidney disease (kidney tissue and/or skin tissue)
Has a kidney biopsy been performed? Yes / No
If yes: Date + diagnosis (conclusion of kidney biopsy report)
Immunohistochemical results (this is a specific staining of the biopsy)
Has a skin biopsy been performed? Yes / No
If yes: Date + diagnosis (conclusion of kidney biopsy report)
Immunohistochemical results (this is a specific staining of the biopsy)

Specific information regarding the kidney disease (imaging)
Has imaging been performed? Yes / No
If yes: Type of imaging (ultrasound/ MRI/ X-ray)

Specific information regarding the kidney disease (genetics)
If the orphacode (see above) concerns a disease which can be inherited, ERKreg will ask if genetic analysis has been performed.
Has a genetic test been performed? Yes / No
Date of genetic testing Day / Month / Year
Type of genetic test Sanger / gene panel / WES/ WGS (this concerns the technical test which has been performed)
Date of test result Day / Month / Year
Is the test result confirming the clinical diagnosis? Yes / No
If yes: Which gene and which variant has been found? Gene and variant

Specific information regarding the clinical course of the kidney disease (renal replacement therapy)
Has there been a need for renal replacement therapy? Yes / No
If yes: Date of start of renal replacement therapy Day / Month / Year
Type of renal replacement therapy Haemodialysis / peritoneal dialysis / kidney transplantation
If there has been a change in type of renal replacement therapy, it can be registered in ERKreg as well. In that case again the date and type of renal replacement therapy will be registered. Furthermore, the reason of the change will be registered (failure of current therapy or wish of patient/family).

Specific information regarding the clinical follow up (yearly update)
A yearly update about clinical course will take place. Data that will be collected are the following:
Data regarding physical examination:
  • Blood pressure
  • Length / weight
Laboratory measurements:
  • Serum creatinine (kidney function)
  • Urinary protein to creatinine ratio (indication of amount of loss of proteins into urine)
  • Dependant on type of kidney disease results of other laboratory measurements.

At closure of ERKreg participation
When a patient is no longer followed up in the ERKreg registry, a follow-up termination date will be added. Also, the reason of termination of registering data of this particular patient shall be added. Reasons can include: patient withdrawn, patient deceased, or patient cannot be reached due to relocation.

Disease specific information (it will be dependent on type of kidney disease which additional data can be collected)
Data regarding physical examination: For example:
  • Hearing loss?
  • Visual problems?
Laboratory measurements: Extensive laboratory measurements, for example:
  • Immunological tests (e.g. ANA/ANCA/anti-PLA2R)
  • Haemoglobin / leukocytes / platelets
  • Infection parameters
  • Liver function
“Events” For example:
  • Thrombosis
  • Admission to hospital
  • Infections (for instance as a complication when a patient uses immunosuppressive therapy)
  • Death
  • Cancer (which can be a complication of certain treatments of kidney diseases)
Data regarding treatment For example:
  • Immunosuppressive therapy
  • Antihypertensive treatment
  • Anticoagulation
Data regarding the results of treatment This will be data as described above