Chronic kidney disease in propionic acidemia.

ABSTRACT:

Purpose

Propionic acidemia (PA) is a severe metabolic disorder characterized by multiorgan pathology, including renal disease. The prevalence of chronic kidney disease (CKD) in PA patients and factors associated with CKD in PA are not known.

Methods

Thirty-one subjects diagnosed with PA underwent laboratory and clinical evaluations through a dedicated natural history study at the National Institutes of Health (ClinicalTrials.gov identifier: NCT02890342).

Results

Cross-sectional analysis of the creatinine-based estimated glomerular filtration rate (eGFR) in subjects with native kidneys revealed an age-dependent decline in renal function (P < 0.002). Among adults with PA, 4/8 (50%) had eGFR <60 mL/min/1.73 m². There was a significant discrepancy between eGFRs

SUBMITTER: Shchelochkov OA 

PROVIDER: S-EPMC7045176 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature