ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: SA-PO0585

Characteristics of Genetically Tested Participants in the ADPKD Registry

Session Information

Category: Genetic Diseases of the Kidneys

  • 1201 Genetic Diseases of the Kidneys: Monogenic Kidney Diseases

Authors

  • Westerfield, Vanessa, PKD Foundation, Kansas City, Missouri, United States
  • Chen, Christopher, PKD Foundation, Kansas City, Missouri, United States
  • Moore, Savanna, PKD Foundation, Kansas City, Missouri, United States
Background

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetically inherited kidney disease; it affects approximately 500,000 in the United States and 12.4 million worldwide. The disease causes fluid filled cysts to grow on the kidneys leading to loss of kidney function and kidney enlargement. Additionally, those with ADPKD may experience extrarenal manifestations such as hypertension, frequent urinary tract infections, and an increased risk of intracranial aneurysm. ADPKD is caused by a genetic mutation, primarily in the PKD1 gene (78%) and the PKD2 gene (15%). The PKD1 genetic mutation presents a more severe and progressive form of polycystic kidney disease than PKD2.

Methods

The ADPKD Registry is an IRB approved, online study that is a database of current data from over 3,300 individuals with ADPKD. Registry participants provide patient-reported outcomes data and have the option to link their electronic health records to accelerate research towards a cure. The Registry inquires on topics such as family history, diet and lifestyle, social determinants of health, genetic testing, etc. The Registry data are deidentified to protect participant privacy.

Results

The ADPKD Registry aims to assess the prevalence of genetic testing for ADPKD patients and to utilize this information for clinical trial recruitment. 216 Registry participants indicated that they have received genetic testing. Of those, 48% cited the PKD1 gene mutation, 16% PKD2, and 36% were not sure, didn’t know, or had a different mutation. The study sample has an average age of 49, is primarily female (78%), and is pre-transplant (82%). There is a fair distribution of chronic kidney disease (CKD) stages represented, Stage 1 (15%), Stage 2 (31%), Stage 3a (19%), Stage 3b (18%), Stage 4 (13%), Stage 5 (4%). Nearly half (43%) of participants identified receiving care at an institution that is a part of the Center of Excellence (COE) Program.

Conclusion

The ADPKD Registry provides a unique opportunity for researchers and industry partners alike to understand the population that has received genetic testing. Current and future clinical trial opportunities rely on identifying the genetic makeup of an ADPKD patient with the goal of targeted genetic therapy.

Digital Object Identifier (DOI)