Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD.

ABSTRACT: Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational study we analyzed the longitudinal development of sonographic kidney measurements in a cohort of 456 ARPKD patients from the international registry study ARegPKD. We furthermore evaluated correlations of sonomorphometric findings and functional kidney disease with the aim to describe the natural disease course and to identify potential prognostic markers. Kidney pole-to-pole (PTP) length and estimated total kidney volume (eTKV) increase with growth t

SUBMITTER: Burgmaier K 

PROVIDER: S-EPMC8568977 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature