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Congenital nephrotic syndrome: is early aggressive treatment needed?-No

Olivia Boyer1, Sandra Bérody2

  • 1Néphrologie Pédiatrique, Centre de Référence MARHEA, Centre de Référence du Syndrome Néphrotique Idiopathique de l'enfant et l'adulte, Hôpital Necker - Enfants Malades, APHP, Inserm U1163, Institut Imagine, Université de Paris, Paris, France. olivia.boyer@aphp.fr.

Pediatric Nephrology (Berlin, Germany)
|May 29, 2020
PubMed

Insights

Congenital nephrotic syndrome (CNS) management is evolving. Conservative care, focusing on nutrition and delayed treatment, shows promising outcomes comparable to aggressive approaches, questioning early nephrectomy and dialysis in infants.

Area of Science:

  • Pediatric Nephrology
  • Genetics and Rare Diseases
  • Neonatal Intensive Care

Background:

  • Infants with congenital nephrotic syndrome (CNS) face significant challenges, including hemodynamic instability, infections, thrombosis, impaired growth, and high rates of end-stage kidney disease (ESKD).
  • Historically, aggressive management involving daily albumin, early nephrectomies, dialysis, and transplantation improved survival but carried substantial risks.
  • Recent evidence suggests conservative management may offer comparable outcomes with reduced complications.

Purpose of the Study:

  • To evaluate the safety and efficacy of conservative management strategies for infants with congenital nephrotic syndrome (CNS).
  • To question the necessity of early preemptive bilateral nephrectomy and dialysis in infants with CNS.
  • To analyze recent evidence supporting conservative care until the development of end-stage kidney disease (ESKD).

Main Methods:

  • Review of recent case-note series and large datasets on congenital nephrotic syndrome (CNS) management.
  • Comparison of outcomes between aggressive (early nephrectomy, dialysis) and conservative (optimized nutrition, delayed renal replacement therapy) treatment approaches.
  • Analysis of complication rates, albumin requirements, and progression to end-stage kidney disease (ESKD) in different management groups.

Main Results:

  • Conservative management, including optimized nutrition and delayed renal replacement therapy, demonstrates good outcomes and low complication rates.
  • Approximately 55% of children with CNS do not spontaneously develop ESKD by age 2 years.
  • Complication rates are similar between preemptive nephrectomy and conservative care groups; albumin tapering and hospital discharge are feasible before nephrectomy.

Conclusions:

  • Conservative management of congenital nephrotic syndrome (CNS) until the development of end-stage kidney disease (ESKD) appears safe for a subset of patients.
  • The findings challenge the routine indication for early preemptive bilateral nephrectomy and dialysis due to associated infant mortality and morbidity.
  • Further research is needed to identify genotype-phenotype correlations to guide individualized clinical management strategies for CNS.

Keywords:
AlbuminCongenital nephrotic syndromeGeneticsNephrectomy

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