Klaus Richard, Lange-Sperandio Bärbel
Division of Pediatric Nephrology, Department of Pediatrics, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Lindwurmstrasse 4, 80337 Munich, Germany.
Biomedicines. 2022 Aug 5;10(8):1894. doi: 10.3390/biomedicines10081894.
Posterior urethral valves (PUV) are the most common form of lower urinary tract obstructions (LUTO). The valves can be surgically corrected postnatally; however, the impairment of kidney and bladder development is irreversible and has lifelong implications. Chronic kidney disease (CKD) and bladder dysfunction are frequent problems. Approximately 20% of PUV patients will reach end-stage kidney disease (ESKD). The subvesical obstruction in PUV leads to muscular hypertrophy and fibrotic remodelling in the bladder, which both impair its function. Kidney development is disturbed and results in dysplasia, hypoplasia, inflammation and renal fibrosis, which are hallmarks of CKD. The prognoses of PUV patients are based on prenatal and postnatal parameters. Prenatal parameters include signs of renal hypodysplasia in the analysis of fetal urine. Postnatally, the most robust predictor of PUV is the nadir serum creatinine after valve ablation. A value that is below 0.4 mg/dl implies a very low risk for ESKD, whereas a value above 0.85 mg/dl indicates a high risk for ESKD. In addition, bladder dysfunction and renal dysplasia point towards an unbeneficial kidney outcome. Experimental urinary markers such as MCP-1 and TGF-β, as well as microalbuminuria, indicate progression to CKD. Until now, prenatal intervention may improve survival but yields no renal benefit. The management of PUV patients includes control of bladder dysfunction and CKD treatment to slow down progression by controlling hypertension, proteinuria and infections. In kidney transplantation, aggressive bladder management is essential to ensure optimal graft survival.
后尿道瓣膜（PUV）是下尿路梗阻（LUTO）最常见的形式。这些瓣膜可在出生后通过手术矫正；然而，肾脏和膀胱发育的损害是不可逆的，并且会产生终身影响。慢性肾脏病（CKD）和膀胱功能障碍是常见问题。大约20%的PUV患者会发展至终末期肾病（ESKD）。PUV中的膀胱下梗阻会导致膀胱肌肉肥大和纤维化重塑，这两者都会损害其功能。肾脏发育受到干扰，导致发育异常、发育不全、炎症和肾纤维化，这些都是CKD的特征。PUV患者的预后基于产前和产后参数。产前参数包括胎儿尿液分析中肾脏发育不良的迹象。出生后，PUV最有力预测指标是瓣膜切除术后血清肌酐最低点。低于0.4mg/dl的值意味着ESKD风险极低，而高于0.85mg/dl的值则表明ESKD风险高。此外，膀胱功能障碍和肾脏发育异常提示肾脏预后不佳。实验性尿液标志物如MCP-1和TGF-β以及微量白蛋白尿表明进展为CKD。到目前为止，产前干预可能会提高生存率，但对肾脏没有益处。PUV患者的管理包括控制膀胱功能障碍和CKD治疗，通过控制高血压、蛋白尿和感染来减缓疾病进展。在肾移植中，积极的膀胱管理对于确保最佳的移植物存活至关重要。
