ArticlePediatric nephrology (Berlin, Germany)2022
Characteristics of continuous venovenous hemodiafiltration in the acute treatment of inherited metabolic disorders.
Article in Pediatric nephrology (Berlin, Germany), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers, 1 of them a synthesis that pooled it.
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Who cites it
10 citing papers in PubMed, 1 synthesis or guideline pooled it, 14 citations in OpenAlex.
- Inborn errors of metabolism in neonates and pediatrics on varying dialysis modalities: a systematic review and meta-analysis.Pediatric nephrology (Berlin, Germany) · 2025Pooled it
- A multicenter descriptive study of neonatal-onset urea cycle disorder patients hospitalized in level IV NICUs.Journal of perinatology : official journal of the California Perinatal Association · 2026Article
- Enhancing outcomes in pediatric populations with continuous renal replacement therapy: Current multidisciplinary strategies and innovations.Journal of intensive medicine · 2025Review
- Evaluation of anticoagulant methods in newborns undergoing continuous renal replacement therapy: what is the optimum method?European journal of pediatrics · 2025Article
- Extracorporeal pediatric renal replacement therapy: diversifying application beyond kidney failure.Pediatric nephrology (Berlin, Germany) · 2025Review
- 3D Printing of Dietary Products for the Management of Inborn Errors of Intermediary Metabolism in Pediatric Populations.Nutrients · 2023Review
- Evaluation of the efficacy and associated complications of regional citrate anticoagulation in neonates: experience from a fourth level neonatal intensive care unit.European journal of pediatrics · 2023Article
- Clinical characteristics and outcomes of continuous renal replacement therapy performed on younger children weighing up to 10 kg.Turkish journal of medical sciences · 2023Observational
- Efficacy of hemodialysis combined with hemofiltration in the treatment of uremia complicated with intractable hypertension.American journal of translational research · 2023Article
- Intravenous branched-chain amino-acid-free solution for the treatment of metabolic decompensation episodes in Spanish pediatric patients with maple syrup urine disease.Frontiers in pediatrics · 2022Article
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7 authors at 1 institution in 1 country.
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Abstract
backgroundContinuous kidney replacement therapies (CKRT) have been reported to be an effective approach to removing toxic metabolites in inborn errors of metabolism (IEM). The present study evaluates efficiency and complications of CKRT in children with IEM.
methodsPatients diagnosed with IEM who underwent CKRT in pediatric and neonatal intensive care units were analyzed. CKRT were initiated in patients with persistently high blood ammonia levels (≥ 500 μmol/L), blood ammonia levels > 250 μmol/L in the presence of moderate encephalopathy, high blood leucine levels (≥ 1500 μmol/L), and blood leucine levels < 1500 μmol/L in the presence of deteriorating neurological status or persistent metabolic acidosis.
resultsOf 22 patients enrolled, nine (40.9%) Maple syrup urine disease (MSUD), eight (36.4%) urea cycle disorders (UCD), and five (22.7%) organic acidemias (OA). Median age was 72.3 [9.9-1040.8] days. In total, 28 dialysis sessions were analyzed [16 (57.1%) continuous venovenous hemodialysis, and 12 (42.9%) continuous venovenous hemodiafiltration]. A significant decrease was noted in leucine levels (from 1608.4 ± 885.3 to 314.6 ± 109.9 µmol/L) of patients with MSUD, while ammonia levels were significantly decreased in patients with UCD and OA (from 1279.9 ± 612.1 to 85.1 ± 21.6 µmol/L). The most frequent complications of CKRT were thrombocytopenia (60.7%), hypotension (53.6%), and hypocalcemia (42.9%). Median age of patients with hypotension treated with vasoactive medications was significantly lower than median age of those with normal blood pressure.
conclusionCKRT is a reliable approach for effective and rapid removal of toxic metabolites in children with IEM, and CKRT modalities can be safely used and are well-tolerated in infants.
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