ESPN 51th Annual Meeting

ESPN 2018


 
ADPedKD: an international web-based database for longitudinal data registry of children with Autosomal Dominant Polycystic Kidney Disease (ADPKD)
STéPHANIE DE RECHTER 1 SALIM ÇALIŞKAN 2 PARIPOVIC DUSAN 3 JAAP GROOTHOFF 4 ADRIAN LUNGU 5 LAURA MASSELLA 6 GIOVANNI MONTINI 7 LARISA PRIKHODINA 8 BRUNO RANCHIN 9 CHAFAI RONIT 10 TOMAS SEEMAN 11 MANISH SINHA 12 ANA TEIXEIRA 13 MARCIN TKACZYK 14 ROSER TORRA 15 BERT BAMMENS 1 FRANZ SCHAEFER 16 MAX CHRISTOPH LIEBAU 17 DJALILA MEKAHLI 1

1- UNIVERSITY HOSPITALS LEUVEN, BELGIUM
2- ISTANBUL CERRAHPASA FACULTY OF MEDICINE, ISTANBUL, TURKEY
3- UNIVERSITY CHILDRENS HOSPITAL BELGRADE, SERBIA
4- 4EMMA CHILDRENS HOSPITAL AMC, AMSTERDAM, THE NETHERLANDS
5- FUNDENI CLINICAL INSTITUTE, BUCHAREST, ROMANIA
6- BAMBINO GESU CHILDRENS HOSPITAL, ROME, ITALY
7- FONDAZIONE IRCCS CA GRANDA, MILANO, ITALY
8- RESEARCH & CLINICAL INSTITUTE FOR PEDIATRICS, PIROGOV RUSSIAN NAT. RES. MED. UNI, MOSCOW, RUSSIAN FEDERATION
9- HôPITAL FEMME MèRE ENFANT, LYON, FRANCE
10- CLINIQUE PéDIATRIQUE DU CENTRE HOSPITALIER DE LUXEMBOURG, LUXEMBOURG
11- DPT. OF PEDIATRICS OF THE UNIVERSITY HOSPITAL MOTOL, PRAGUE, CZECH REPUBLIC
12- EVELINA LONDON CHILDRENS HOSPITAL, LONDON, UK
13- CENTRO MATERNO INFANTIL DO NORTE, CENTRO HOSPITALAR DO PORTO, PORTUGAL
14- POLISH MOTHERS MEMORIAL HOSPITAL RESEARCH INSTITUTE, LODZ, POLAND
15- FUNDACIO PUIGVERT, BARCELONA, SPAIN
16- HEIDELBERG UNIVERSITY CENTER, GERMANY
17- UNIVERSITY HOSPITAL OF COLOGNE, GERMANY
 
Introduction:

Although literature on Autosomal Dominant Polycystic Kidney Disease (ADPKD) is merging over the last decades, data on paediatric ADPKD are still scarce. Moreover, unified diagnostic, follow-up and treatment approaches regarding modifiable disease factors in order to slow down disease progression are currently lacking for ADPKD children. We therefore aim to build the largest international multicentre cohort, named ADPedKD, from which we will generate data on the incidence and presentation of ADPKD in childhood. Furthermore, we intent to define a paediatric patient risk stratification score, after identification of progression factors.

Material and methods:

Patients diagnosed with ADPKD, based on genetic analysis and/ or a positive familial history and with renal cysts on imaging, before the age of 19 years are eligible for inclusion in this observational register study. Ethical committee approval is organized per country as regulations differ internationally. After written informed consent, clinical patient data are pseudonymously introduced in our secured web-based database (www.adpedkd.org), both retrospectively and prospectively, from all participating centres throughout the world. Our main focus consists of the initial presentation, pre- and perinatal history, genetic analysis, renal function and longitudinal follow-up.

Results:

We currently have 34 centres from 15 different countries registered in ADPedKD: Belgium, Czech Republic, France, Germany, Italy, Luxembourg, Poland, Portugal, Romania, Russian Federation, Serbia, Spain, the Netherlands, Turkey and United Kingdom. Twenty-two centres are in the patient recruitment phase; while the others are already actively entering patient data, resulting in 128 registered paediatric ADPKD patients.

Conclusions:

The ADPedKD initiative is the first and the largest international paediatric ADPKD registry that will provide evidence for a paediatric patient risk stratification scoring system from an early disease stage.