The management of infants with congenital nephrotic syndrome (CNS) is very challenging as they are prone to severe complications such as hemodynamic disturbances, infections, thromboses, and impaired growth, and most will develop end-stage kidney disease (ESKD) within a few years. Since the seventies, an "aggressive" approach, including daily albumin infusions, early nephrectomies, dialysis, and transplantation, has dramatically improved survival and morbidity. More recent case-note reviews have reported successful conservative treatment (using optimized nutrition, complication prophylaxis, and delayed renal replacement therapy), which led to similarly good outcomes and low complication rates. This questions the indications for early preemptive bilateral nephrectomy and dialysis given the mortality and morbidity rates in dialysis in infants and their life-long management with possible repeated transplantations. Two large series provide the most recent evidences supporting the conservative management: firstly, at least 55% children with CNS are not spontaneously in ESKD at the age of 2 years; secondly, albumin tapering/discontinuation and hospital discharge are possible before nephrectomy; and lastly, CNS complication rates are similar in case of preemptive nephrectomies or conservative care. Until now, no clear genotype-phenotype correlation has been identified to guide clinical management. Taken together, these data support the safety of conservative care until ESKD in a subset of patients with CNS.

中文翻译：

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先天性肾病综合征：是否需要早期积极治疗？-不需要。

先天性肾病综合征 (CNS) 婴儿的管理非常具有挑战性，因为他们容易出现严重的并发症，如血流动力学紊乱、感染、血栓形成和生长受损，并且大多数会在几年内发展为终末期肾病 (ESKD) . 自 70 年代以来，一种“积极的”方法，包括每日输注白蛋白、早期肾切除术、透析和移植，显着提高了生存率和发病率。最近的病例报告审查报告了成功的保守治疗（使用优化营养、并发症预防和延迟肾脏替代治疗），这导致了同样好的结果和低并发症发生率。鉴于婴儿透析的死亡率和发病率以及可能重复移植的终生管理，这对早期先发制人双侧肾切除术和透析的指征提出了质疑。两个大型系列提供了支持保守治疗的最新证据：第一，至少 55% 的 CNS 儿童在 2 岁时不是自发性 ESKD；其次，在肾切除术之前，白蛋白逐渐减少/停止和出院是可能的；最后，在先行性肾切除术或保守治疗的情况下，CNS 并发症发生率相似。到目前为止，尚未确定明确的基因型-表型相关性来指导临床管理。总之，这些数据支持在一部分 CNS 患者中进行保守治疗直至 ESKD 的安全性。