ArticlePediatric nephrology (Berlin, Germany)2003
Management of congenital nephrotic syndrome.
Article in Pediatric nephrology (Berlin, Germany), 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 78 citations in OpenAlex.
- Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.Nature reviews. Nephrology · 2021Guideline
- Nephrectomy for congenital nephrotic syndrome: unanswered questions.Pediatric nephrology (Berlin, Germany) · 2025Article
- The clinical characteristics of patients with congenital nephrotic syndrome secondary to NPHS1 mutation: Is nephrectomy still a therapeutic option for selected cases?Pediatric nephrology (Berlin, Germany) · 2025Article
- A novel NPHS1 variant in a Chinese infant with congenital nephrotic syndrome: a case report and literature review.Frontiers in pediatrics · 2025Article
- Native nephrectomy in advanced pediatric kidney disease: indications, timing, and surgical approaches.Pediatric nephrology (Berlin, Germany) · 2024Review
- Nephrotic Syndrome: A Review.Cureus · 2024Review
- Unilateral nephrectomy for young infants with congenital nephrotic syndrome of the Finnish type.Clinical and experimental nephrology · 2022Article
- Clinical presentation and management of nephrotic syndrome in the first year of life: A report from the Pediatric Nephrology Research Consortium.Frontiers in pediatrics · 2022Article
- Risk factors for post-nephrectomy hypotension in pediatric patients.Pediatric nephrology (Berlin, Germany) · 2021Observational
- Thromboprophylaxis in congenital nephrotic syndrome: 15-year experience from a national cohort.Pediatric nephrology (Berlin, Germany) · 2021Article
- Congenital nephrotic syndrome: is early aggressive treatment needed? Yes.Pediatric nephrology (Berlin, Germany) · 2020Review
- Article
- A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan.BMC nephrology · 2020Observational
- Home Albumin Infusion Therapy, Another Alternative Treatment in Patients With Congenital Nephrotic Syndrome of the Finnish Type.Frontiers in pediatrics · 2020Article
- Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases.Pediatric nephrology (Berlin, Germany) · 2019Article
- Diagnostic and Management Challenges in Congenital Nephrotic Syndrome.Pediatric health, medicine and therapeutics · 2019Review
- Long-term outcome of congenital nephrotic syndrome after kidney transplantation in Japan.Clinical and experimental nephrology · 2018Article
- Treatment of Genetic Forms of Nephrotic Syndrome.Frontiers in pediatrics · 2018Review
- Variation in estimated glomerular filtration rate at dialysis initiation in children.Pediatric nephrology (Berlin, Germany) · 2017Observational
- Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry.Pediatric nephrology (Berlin, Germany) · 2016Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We reviewed the medical records of seven children with congenital nephrotic syndrome (CNS) treated by unilateral nephrectomy, captopril, and indomethacin since 1990. Clinical response to the treatment was analyzed using the Students' t-test. After a median period of 54 months (range 36-88 months) follow-up, five patients were alive at a median age of 74 (range 43-88) months. Median (range) plasma albumin rose from 11 (6-17) g/l at the start of treatment to 18 (15-22) g/l and 21 (18-25) g/l after 6 and 12 months treatment, respectively ( P=0.001 and P=0.0006). Albumin infusions per patient per month decreased from 7 (0-18) to 0 (0-30) in the 6 months post treatment ( P=0.017). The median (range) height standard deviation scores at 12 months and 30 months from onset of treatment were -1.56 (-2.96 to 0.41) and -1.43 (-2.40 to 0.90), respectively. In conclusion, management of CNS with captopril and indomethacin therapy in combination with unilateral nephrectomy achieves significant improvements in plasma albumin and reduces the need for albumin infusions and time in hospital, while growth is maintained. Second nephrectomy, dialysis, and transplantation can be delayed until the 3rd year of life or longer.
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