A Prediction Model of Disease Progression in X-Linked Alport syndrome Based on Clinical Characteristics and Genetic Variants
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Le résumé fourni par la source
Introduction: Alport syndrome (AS) is an inherited kidney disease with significant clinical heterogeneity. Prognosis prediction and risk assessment are important to assist patient care. However, a predictive tool of disease progression is still lacking. Methods: The prediction model was developed in 363 patients (124 kidney failure events) with X-linked AS (XLAS) from a single-center retrospective cohort study and validated in 2 external cohorts, including 193 (27 events) and 125 patients (33 events) with XLAS from 6 centers and the literature database, respectively. Cox proportional hazards regression analysis with stepwise selection was used to select the important variables related to the progression to kidney failure, by using the baseline demographic, clinical, and genetic data. The performance of the prediction model was evaluated and compared using receiver-operating characteristic (ROC) curve and calibration plot. Results: Based on the model risk stratification, the median age at kidney failure was 23, 30, and 61 years in the low-, intermediate-, and high-risk groups, respectively. This model shows the best discrimination in predicting the progression to kidney failure before the age of 30 years, with areas under the curve (AUCs) > 0.80 in both development and external cohorts. Conclusion: A prediction model of progression to kidney failure based on clinical characteristics and genetic variants was developed and validated in patients with XLAS.
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- A Prediction Model of Disease Progression in X-Linked Alport syndrome Based on Clinical Characteristics and Genetic Variants
- Date Crossref
- 01/06/2025
- Éditeur
- Elsevier BV
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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Nanjing General Hospital of Nanjing Military Command pays non établi dans la noticeÉtablissement de santé
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Nanjing University National Clinical Research Center of Kidney Diseases pays non établi dans la noticeUniversité ou école supérieure
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Peking University Department of Pediatrics pays non établi dans la noticeUniversité ou école supérieure
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Peking University First Hospital pays non établi dans la noticeÉtablissement de santé
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Children's Hospital of Fudan University Department of Nephrology pays non établi dans la noticeÉtablissement de santé
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Ruijin Hospital Department of Nephrology pays non établi dans la noticeÉtablissement de santé
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Children's Hospital of Zhejiang University pays non établi dans la noticeÉtablissement de santé
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Second Affiliated Hospital of Nanjing Medical University pays non établi dans la noticeÉtablissement de santé
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Zhejiang University School of Medicine National Clinical Research Center for Child Health pays non établi dans la noticeUniversité ou école supérieure
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Children's Hospital of Nanjing Medical University Department of Nephrology pays non établi dans la noticeUniversité ou école supérieure
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Sichuan Provincial People's Hospital Department of Nephrology pays non établi dans la noticeÉtablissement de santé
Nanjing General Hospital of Nanjing Military Command, National Clinical Research Center of Kidney Diseases — Nanjing University et Department of Pediatrics — Peking University, avec 8 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.