ReviewPediatric nephrology (Berlin, Germany)2020
Congenital nephrotic syndrome: is early aggressive treatment needed?-No.
Review in Pediatric nephrology (Berlin, Germany), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
5 citing papers in PubMed, 11 citations in OpenAlex.
- Native nephrectomy in advanced pediatric kidney disease: indications, timing, and surgical approaches.Pediatric nephrology (Berlin, Germany) · 2024Review
- Case report: Unveiling a less severe congenital nephrotic syndrome in a Rapa Nui patient with aFrontiers in nephrology · 2024Article
- Unilateral nephrectomy for young infants with congenital nephrotic syndrome of the Finnish type.Clinical and experimental nephrology · 2022Article
- Congenital nephrotic syndrome.Journal of perinatology : official journal of the California Perinatal Association · 2021Review
- Case Report: CMV-Associated Congenital Nephrotic Syndrome.Frontiers in pediatrics · 2020Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.