Abstract | RATIONALE & OBJECTIVE: STUDY DESIGN: Retrospective observational study. SETTING & PARTICIPANTS: Patients with PH1 enrolled in the Rare Kidney Stone Consortium (RKSC) registry who did not have kidney failure at diagnosis and who had at least 2 eGFR values recorded from within 1 month of diagnosis until their last contact date or incident kidney failure event. PREDICTORS: CKD GFR category, baseline patient and laboratory characteristics. OUTCOME: Annualized rate of eGFR decline. ANALYTICAL APPROACH: Generalized estimating equations and linear regression were used to evaluate the associations between CKD GFR category, baseline patient and laboratory characteristics, and annual change in eGFR during follow-up. RESULTS: Compared with the slope in CKD G2 (-2.3 mL/min/1.73 m2 per year), the mean annual eGFR decline was nominally steeper in CKD G3a (-5.3 mL/min/1.73 m2 per year) and statistically significantly more rapid in CKD G3b and G4 (-14.7 and -16.6 mL/min/1.73 m2 per year, respectively). In CKD G2, older age was associated with a more rapid rate of eGFR decline (P = 0.01). A common PH1-causing variant of alanine glyoxylate aminotransferase, a glycine to arginine substitution at amino acid 170 (G170R), appeared to be associated with less severe annual decline in eGFR. LIMITATIONS: Data at regular time points were not available for all patients due to reliance on voluntary reporting in a retrospective rare disease registry. CONCLUSIONS: The eGFR decline was not uniform across CKD GFR categories in this PH1 population, with a higher rate of eGFR decline in CKD G3b and G4. Thus, CKD GFR category needs to be accounted for when analyzing eGFR change in the setting of PH1.
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Authors | Prince Singh, Lisa E Vaughan, Phillip J Schulte, David J Sas, Dawn S Milliner, John C Lieske |
Journal | American journal of kidney diseases : the official journal of the National Kidney Foundation
(Am J Kidney Dis)
Vol. 80
Issue 3
Pg. 373-382
(09 2022)
ISSN: 1523-6838 [Electronic] United States |
PMID | 35306035
(Publication Type: Journal Article, Observational Study, Research Support, Non-U.S. Gov't, Research Support, N.I.H., Extramural)
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Copyright | Copyright © 2022 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved. |
Topics |
- Disease Progression
- Glomerular Filtration Rate
- Humans
- Hyperoxaluria, Primary
- Kidney Calculi
- Renal Insufficiency, Chronic
(epidemiology)
- Retrospective Studies
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