Chronic Kidney Disease
Management in Patients with Fabry Disease.
IMPORTANCE OF MONITORING AND MANAGING CKD IN FABRY DISEASE
• CKD progression is associated with increased all-cause and cardiovascular mortality.1,2
• In young patients with Fabry disease, progressive podocyte injury caused by the accumulation of GL3 correlates with an increased UACR.3
• Proteinuria, specifically albuminuria, is a significant risk factor and an important clinical marker for Fabry nephropathy.4
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Association of albuminuria and GFR with All-Cause Mortality1
Translating CKD Risk Classes into CVD Risk Classes2
Fabry nephropathy is a progressive proteinuric CKD of metabolic origin.5,6
SCHEDULE FOR RENAL MONITORING IN FABRY DISEASE AS RECOMMENDED BY EXPERTS7
IMPORTANCE OF MONITORING LYSO-GL3 LEVELS7,9-11
In Fabry disease, monitoring lyso-GL3 levels is crucial for assessing renal involvement and guiding treatment, as early detection of elevated lyso-GL3 can lead to timely interventions, preventing or delaying CKD and improving patient outcomes.
The accumulation of lyso-GL3 causes significant kidney damage by inducing inflammation, oxidative stress, and fibrosis in renal cells.
eGFR AND RESPONSE TO TREATMENT IN ADULT PATIENTS WITH FABRY DISEASE12
NEPHROPROTECTIVE STRATEGY IN FABRY DISEASE7,13
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References
1. KDIGO 2012 Clinical Practice Guideline for CKD. Kidney Int Suppl.2013;3:1–150.
2. Ortiz A, et al. Eur J Prev Cardiol. 2022;29:2211–2215.
3. Najafian B, et al. Kidney Int. 2011;79(6):663-670.
4. Wanner C, et al. Clin J Am Soc Nephrol. 2010;5(12):2220-8.
5. Ortiz A, et al. Nephrol Dial Transplant. 2010;25(3):769–775.
6. Ortiz A, et al. Nephrol Dial Transplant. 2008;23:1600–1607.
7. Ortiz A, et al. Mol Genet Metab. 2018;123:416-427.
8. Wanner C, et al. Mol Genet Metab. 2018;124:189–203
9. Ortiz A, Sanchez-Niño MD. Port J Nephrol Hypert. 2017;31:200-6.
10. Sanchez-Niño MD, et al. Nephrol Dial Transplant. 2011;26:1797-802.
11. Sanchez-Niño MD, et al. Hum Mol Genet. 2015;24:5720-32.
12. Pisani A, et al. Nephrol Dial Transplant. 2024;39:18–25.
13. KDIGO 2024 Clinical Practice Guideline for CKD. Kidney Int. 2024;105(4S):S117–S314.
A Heart for Fabry - Podcast
A podcast series designed for cardiologists and healthcare professionals, focusing on the critical role of cardiologists in diagnosing and managing Fabry disease.
Building Care webinar series
The Building Care webinar series is designed to deepen your understanding of the critical role a patient-centered, multidisciplinary approach plays in managing Fabry disease.
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