Back
For best output, select "Paper Size" as "A4" and "Margin" as "0" or "None".
To save or print to PDF, please select Print Destination > Save as PDF, enable Background Graphics under "More Settings", then click "Save".
During the congress, E-Posters will be accessible to all participants on the congress website 24/7, as well as in the E-poster stations in the congress center.
Preparing your E-Poster
Please review the E-Poster format requirements carefully when preparing your E-Poster. Should your E-Poster not meet the mentioned requirements, it may not be displayed as described above.
E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
Please follow the instructions below to input your abstract title.
Abstract titles should be brief and reflect the content of the abstract.
Comprehensive epidemiological data on Alport syndrome, particularly from national cohorts, remain limited.
Using the national Alport syndrome registry in Japan (JP-ALPS), established in October 2022, we described baseline characteristics in the 2022–2025 cohort (N = 257) and analyzed longitudinal trends in estimated glomerular filtration rate (eGFR) and urine protein-to-creatinine ratio in the 2022–2024 subcohort (N = 121).
Median age at onset was 3 (IQR, 1-4) years, 137 (53.3%) were male, and 210 (81.7%) underwent genetic testing. Among those with genetic testing, 155 (73.8%) had X-linked, 15 (7.1%) autosomal recessive, and 26 (12.4%) autosomal dominant Alport syndrome. Kidney biopsy was performed in 158 (61.5%) patients. On light microscopy, minor glomerular abnormalities were the most common finding. On electron microscopy, GBM changes were seen in most patients. Alpha five staining was evaluated in 122 patients; patchy staining was most common in females, while negative staining was most common in males. Kidney function was normal at disease onset, with a median eGFR of 112.9 (interquartile range, 99.3–131.1) mL/min/1.73 m2. Although a steep decline during adolescence was observed in some male patients with X-linked Alport syndrome, eGFR decline was relatively slow during childhood and adolescence; the point estimate of eGFR at age 20 was 88.6 mL/min/1.73 m2. Six patients transitioned to end-stage kidney disease during the follow-up period. Eighty-one patients (66.9%) used renin-angiotensin-system (RAS) inhibitors, and the rate of eGFR decline was slower after RAS inhibitor initiation.
Most patients were diagnosed through genetic testing. In our cohort which consisted mainly of patients who did not require kidney replacement therapy in childhood and adolescence, kidney function was preserved throughout this period except for some male patients with X-linked Alport syndrome. RAS inhibitor use may be associated with a reduced rate of eGFR decline.
The results reported in part of this abstract have been published in Clinical and Experimental Nephrology.
