1.       The goal of the pediatric ADPKD registry “ADPedKD”

The ADPKD registry ADPedKD aims to collect data on the clinical courses of ADPKD patients diagnosed and followed-up from childhood. We believe that identifying determinants of disease progression in childhood, will improve the management of ADPKD patients.

In ADPedKD, medical staff of (Pediatric) Nephrology centers, mainly in Europe, will enter pseudonomyzed patient data. A detailed "basic data" form and "initial visit" questionnaire will be followed by yearly "follow-up visits".

2.       ADPedKD inclusion and exclusion criteria

Inclusion criteria for ADPedKD are:

- ADPKD diagnosed by positive family history and renal imaging (the presence of  at least one cyst, preferably >1 cm in diameter) and/or molecular assessment

- ADPKD diagnosed at the age of 19 years or younger

- Informed consent of patient and his/her parents/legal representatives

- TSC2/PKD1 contiguous syndromes will be also included and analyzed separtely

Exclusion criteria are:

- Renal cysts due to another disorder such as ARPKD, TSC, ect….

- Genetic proof of another cystic kidney disorder  

3.       How to use the ADPedKD webpage

If you would like to include your patients data in ADPedKD, please register (www.adpedkd.org/index.php). We will send you a username and a password as soon as possible.

Once you have registered, please make sure that an approval of the corresponding ethics committee has been obtained and that your patient and his/her parents have received, understood and signed the patient and parent/legal representative information and informed consent form.

Within the password-restricted area, you will be able to create new patients for your center. Please make sure that you have the informed consent of your patient at hand. You will have to indicate the date of informed consent.  You will always be able to review the data of all patients from your own center, but you will not have access to data from other centers.

Once you have created a novel patient, please fill in the “basic data” form first. After filling in the mandatory data fields, the system will create a unique patient-ID for your patient. You will only have to fill in the “basic data” form once.

Please fill in the “initial visit” form, once you have completed the "basic data" form.  The "initial visit" form deals with the clinical presentation at an indicated date. It is intended to follow every patient with at least one yearly follow-up visit. ADPedKD will calculate the time to the next scheduled visit and will indicate it in the “patients registry” for your center. You can however add a follow-up visit at any time. Please provide us with as much and as detailed information as possible.

You can also download a small ADPedKD handbook (link here)