The European Rare Kidney Disease Reference Network


Research Project

Project Title:


Project Type:


Disease group(s):

AD structural kidney disorders

Project Summary:

ADPedKD: An international web-based database for longitudinal
data registry of children with Autosomal Dominant Polycystic Kidney
Disease (ADPKD)

A longitudinal international registry on ADPKD children is built up in this ADPedKD project, to provide an observational evidence base for unified diagnostic, follow-up and treatment approaches regarding modifiable disease factors in order to slow down disease progression such as hypertension and
proteinuria. We also aim to establish clinical and/or bio-markers predicting the risk of early and progressive disease and potentially lay the foundation for clinic trial patient selection.
To sum up, our objectives are the following:
- As data on pediatric ADPKD are scarce, the first aim is to generate data on its incidence and presentation in childhood, and of its comorbidities such as hypertension and left ventricular hypertrophy.

- Secondly, we aim to define a pediatric scoring system, after identification of
progression factors, by which we will be able to stratify patients from an early disease stage on into low to high risk categories.

- Next, we will assess the effect of early treatment of hypertension and
proteinuria on long term renal outcome.

- Last, we aim to provide international guidelines for workup, follow-up and treatment of ADPKD in childhood.

Lead principal investigator(s):

Djalila Mekahli, Leuven


Franz Schaefer, Heidelberg
Max Liebau, Cologne
Stephanie De Rechter, Leuven

Project Period:

07/2017   -   09/2042


Local resources

Project web page:

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