NIH R01 · 2024
Imaging Assessments of ARPKD Kidney Disease Progression
PROJECT SUMMARY / ABSTRACT Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a potentially lethal inherited disorder that affects approximately 1/20,000 children and is genetically and clinically distinct from the more common Autosomal Dominant PKD (ADPKD). ARPKD is characterized by diffuse kidney microcysts resulting from fusiform dilatations of the collecting tubules. As these kidneys cysts accumulate, the kidneys do not show progressive enlargement, but instead accumulate more cysts and eventually become progressively more fibrotic, resulting in end-stage kidney disease (ESKD). Approximately 40-50% of ARPKD children who survive the neonatal period progress to ESKD by age 18,…
From the public funding record at NIH RePORTER. Describes the funded project, not the reviews below.