Continuous Renal Replacement Therapy for Acute Decompensation in Inborn Errors of Metabolism: Single-Center, Pediatric Cohort, 2014–2025

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Continuous renal replacement therapy achieves substantial reductions in toxic metabolites in critically ill pediatric patients with inborn errors of metabolism, with no significant difference in mortality between hemodialysis and hemodiafiltration modalities. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.SUPPORTS_CONSENSUS.

Merve Güneş; Engi̇n Köse; Eda Eyduran; Doğan Kaymaz; Leyla Kaya; Merve Havan; Emel Okulu; Tanil Kendirli; Fatma Tuba Eminoğlu

doi:10.1097/pcc.0000000000004043

September 11, 2026Pediatric Critical Care Medicine

Continuous Renal Replacement Therapy for Acute Decompensation in Inborn Errors of Metabolism: Single-Center, Pediatric Cohort, 2014–2025

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Key Points

To evaluate the efficacy, complications, and mortality associated with continuous renal replacement therapy (CRRT) during acute metabolic crises in pediatric inborn errors of metabolism.
Retrospective single-center cohort study conducted in a pediatric and neonatal ICU at a tertiary center in Türkiye from August 2014 to June 2025.
Analyzed N=38 children undergoing 49 CRRT sessions (continuous venovenous hemodiafiltration [CVVHDF] vs. hemodialysis) for urea cycle disorders, organic acidemias, or maple syrup urine disease.
CRRT achieved substantial clearance of toxic metabolites, resulting in a mean reduction of 87.6% for ammonia and 74.8% for leucine.
Overall mortality was 28.9% (11 of 38; 95% CI, 17–44.8%), with non-surviving episodes exhibiting a significantly higher frequency of hypophosphatemia compared to surviving episodes (6 of 11 vs. 8 of 38; percentage difference 33.4%, 95% CI, 2.8–59.6%, p=0.03).
Mortality did not differ significantly by CRRT modality when comparing CVVHDF against hemodialysis (6 of 21 vs. 5 of 28; mean difference 10.7%, 95% CI, -12.4 to 34.3%, p=0.38).

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Implication

Retrospective cohort study reveals continuous renal replacement therapy effectively clears toxic metabolites in infants with metabolic disorders, indicating its vital role in crisis management.

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Cite This Study

Güneş et al. (2026) studied this question.

synapsesocial.com/papers/6aa7b2b20926e14a848b12d9 https://doi.org/10.1097/pcc.0000000000004043

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Continuous Renal Replacement Therapy for Acute Decompensation in Inborn Errors of Metabolism: Single-Center, Pediatric Cohort, 2014–2025

Key Points

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Implication

Discussion

Cite This Study

Continuous Renal Replacement Therapy for Acute Decompensation in Inborn Errors of Metabolism: Single-Center, Pediatric Cohort, 2014–2025

Key Points

No abstract yet

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Implication

Discussion

Cite This Study

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