ArticleKidney medicine2026
Multifactorial Predictors of Renal Outcomes in Alport Syndrome: Integrating Genetic, Clinical, and Cystic Phenotypes.
Article in Kidney medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Rationale & Objective: Alport syndrome is an inherited kidney disease with significant clinical heterogeneity. This study aims to explore risk factors affecting the prognosis and investigates the relationship between kidney cysts and clinical manifestations in patients with Alport syndrome. Study Design: A single-center retrospective cohort study. Setting & Participants: A total of 179 patients diagnosed with Alport syndrome in Peking University First Hospital. Predictors: Clinical characteristics (renal manifestations and extrarenal features), genetic patterns, and the presence of kidney cysts. Outcomes: Kidney failure with replacement therapy or chronic kidney disease stage 3b. Analytical Approach: Kaplan-Meier survival analysis, Cox proportional hazards models, logistic regression models, and linear mixed models. Results: In this Chinese adult Alport syndrome cohort, we identified several prognostic risk factors, including baseline serum creatinine (HR, 3.15; 95% CI, 1.95-5.08), proteinuria (HR, 1.29; 95% CI, 1.01-1.65), earlier diagnostic age (HR, 1.30; 95% CI, 1.16-1.45), male sex (HR, 3.00; 95% CI, 1.05-8.54), eye and ear impairment (HR, 19.49; 95% CI, 2.52-150.67), and inheritance mode (X-linked or autosomal recessive) (HR, 5.46; 95% CI, 1.47-20.30). Notably, 33.5% of patients with Alport syndrome presented with kidney cysts, compared with only 12.8% in the matched IgA nephropathy cohort ( Limitations: Selection bias, absence of time-varying cyst observations, and limitations in generalizability. Conclusions: Our study reveals that kidney cysts are associated with severe clinical manifestations in Alport syndrome, particularly in younger patients. This finding highlights the importance of monitoring kidney cysts in patients with Alport syndrome and suggests their potential role as a prognostic indicator.
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