Mandy Rickard, Armando J Lorenzo, Cal Robinson, Samer Maher, Jin Kyu Kim, Adree Khondker, Mirriam Mikhail, Beverly Miranda, Rodrigo Romao, Joao Pippi Salle, Michael Chua, Aseel Al-Dmour, Nithiakishna Selvathesan, Joana Dos Santos
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引用次数: 0
Abstract
Background: Posterior urethral valves (PUV) are a rare congenital condition leading to chronic kidney disease (CKD) and bladder dysfunction. Traditional management is often reactive and non-standardized. In 2019, we implemented a dedicated multidisciplinary PUV clinic with a standardized, proactive management pathway to optimize kidney and bladder outcomes. This study evaluates its impact over 5 years.
Methods: We conducted a retrospective review of our prospectively maintained PUV database, including patients diagnosed before 24 months and managed exclusively at our institution. Outcomes were compared between patients managed in the PUV clinic (aPUV) to patients before implementation (bPUV). Primary outcomes included kidney function (serum creatinine, eGFR, CKD progression, and kidney replacement therapy). Secondary outcomes assessed bladder function, including medications, catheterization, and urinary tract infections.
Results: A total of 196 patients were analyzed (bPUV: 133; aPUV: 63). The aPUV cohort had earlier interventions and a higher rate of primary urinary diversion (54% vs. 11%; p < 0.01). The aPUV group had lower nadir serum creatinine (21 vs. 29 μmol/l; p < 0.01), reduced CKD progression (12% vs. 27%; p = 0.02), and fewer patients requiring kidney replacement therapy (3% vs. 20%; p < 0.01). Earlier bladder therapy initiation led to reduced hydronephrosis and timelier catheterization.
Conclusion: A standardized PUV clinic and proactive management approach significantly improved kidney and bladder outcomes at 5 years. If sustained, this strategy may delay CKD progression and reduce the need for kidney replacement therapy. Longer follow-up is needed to assess long-term impact.
背景:后尿道瓣膜(PUV)是一种罕见的先天性疾病,可导致慢性肾脏疾病(CKD)和膀胱功能障碍。传统的管理往往是被动的、不规范的。2019年,我们实施了一个专门的多学科PUV诊所,采用标准化、主动的管理途径来优化肾脏和膀胱的预后。本研究评估其在5年内的影响。方法:我们对前瞻性维护的PUV数据库进行了回顾性审查,包括24个月前诊断并在我们机构专门管理的患者。将在PUV诊所(aPUV)治疗的患者与实施前(bPUV)患者的结果进行比较。主要结局包括肾功能(血清肌酐、eGFR、CKD进展和肾脏替代治疗)。次要结局评估膀胱功能,包括药物、导尿和尿路感染。结果:共分析196例患者(bPUV: 133;aPUV: 63)。aPUV组干预较早,原发性尿分流率较高(54% vs. 11%;结论:标准化的PUV临床和积极的治疗方法显著改善了5年患者的肾脏和膀胱预后。如果持续下去,这种策略可以延缓CKD的进展,减少对肾脏替代治疗的需求。需要更长的随访来评估长期影响。
期刊介绍:
International Pediatric Nephrology Association
Pediatric Nephrology publishes original clinical research related to acute and chronic diseases that affect renal function, blood pressure, and fluid and electrolyte disorders in children. Studies may involve medical, surgical, nutritional, physiologic, biochemical, genetic, pathologic or immunologic aspects of disease, imaging techniques or consequences of acute or chronic kidney disease. There are 12 issues per year that contain Editorial Commentaries, Reviews, Educational Reviews, Original Articles, Brief Reports, Rapid Communications, Clinical Quizzes, and Letters to the Editors.