Long-term outcome among females with Alport syndrome from a single pediatric center
Pediatric Nephrology Mar 03, 2021
Goka S, et al. - Researchers report the clinical presentation as well as the course of females suffering from Alport syndrome (AS, a multisystem condition which can lead to progressive kidney disease, hearing loss, and ocular alterations), in this single-center retrospective study. All young females with AS (n = 36) between January 1, 1987, and May 20, 2019 were analyzed. The mean age at presentation was 5.58 ± 3.0 years. At end of the observation span, 15 patients (42%) had gross hematuria, 20 (56%) had proteinuria, and 2 (6.7%) exhibited an estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73m 2 with one patient suffering from stage 5 chronic kidney disease. In this cohort, the most common causes for the initial presentation were known family histories of AS or gross hematuria. Not exceptionally, patients were found to have proteinuria, eGFR < 90 ml/min/1.73m 2 , and abnormal audiology exam, and therefore, there is need for close monitoring of young females into adulthood.
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