Amino Acid Deficiency Secondary to Continuous Venovenous Hemofiltration in Acute Decompensation of Organic Acidemias: An Anabolic Dead End?

Chloé Grosyeux, Noémie Cammiciotto, Elise Jeannesson, Eva Feigerlova, Valentine Villada, Camille Wicker, Michael Levy, Manuel Schiff, Apolline Imbard, David Coelho, François Feillet, Arnaud Wiedemann

Journal: Critical care explorations 2026;8(8):e1413

PMID: 42538737

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Abstract

OBJECTIVE

To evaluate the impact of continuous renal replacement therapy (CRRT) on plasma amino acid (AA) concentrations in patients with acute metabolic decompensation of organic acidemias (OAs), and to explore whether AA supplementation during CRRT may mitigate AA depletion.

DESIGN

Multicenter retrospective observational study.

SETTING

PICUs managing acute metabolic decompensations of OAs.

PATIENTS OR SUBJECTS

Patients with confirmed OAs who underwent CRRT for severe acute metabolic decompensation.

INTERVENTIONS

CRRT was initiated according to current guidelines in cases of severe decompensation. Standard metabolic management included high caloric intake through carbohydrates and lipids with temporary protein withdrawal (24-48 hr). In one patient, AA supplementation (2 g/kg) during CRRT combined with thiamin and citrate (anaplerotic therapy) was administered.

MEASUREMENTS AND MAIN RESULTS

Plasma AA concentrations were measured before and after CRRT in nine patients with a median age of 21 days (interquartile range [IQR], 3-570 d). Quantitative variables are expressed as medians (IQR, 25th-75th). Before CRRT, 31% (95% CI, 24-39) of plasma AAs were below the normal range compared with 69% (95% CI, 59-77) after CRRT (p < 0.0001). A significant increase in lactatemia was observed following CRRT, without evidence of organ failure: median 2.2 mmol/L (IQR, 1.3-2.3) before CRRT vs. 5.3 mmol/L (IQR, 3.2-7.1) after CRRT; Hodges-Lehmann median difference +3.2 (95% CI, 0.8-5.6; p = 0.0065). In the patient receiving AA supplementation with anaplerotic therapy, plasma AA status improved markedly, with 65% of AAs below normal before CRRT vs. 15% after CRRT.

CONCLUSIONS

In acute decompensated OAs, CRRT performed without protein supplementation, as currently recommended, significantly reduces total plasma AA concentrations and may impair restoration of anabolism. AA infusion during CRRT could help preserve or restore protein anabolism. Prospective studies are needed to assess the safety and efficacy of AA supplementation during CRRT, with or without anaplerotic therapy, in this setting.

Address: Pediatric Nephrology Department, University Hospital of Nancy, Nancy, France.; Pediatric Department, Reference Center of Inborn Errors of Metabolism (ORPHA67872), University Regional Hospital Center of Nancy, INSERM UMRS 1256 NGERE-Nutrition, Genetics, and Environmental Risk Exposure, Nancy, France.; Pediatric Department, Reference Center of Inborn Errors of Metabolism (ORPHA67872), University Regional Hospital Center of Nancy, INSERM UMRS 1256 NGERE-Nutrition, Genetics, and Environmental Risk Exposure, Nancy, France.; Division of Biochemistry, Department of Molecular Medicine, University Hospital of Nancy, Nancy, France.; Pediatric Inherited Metabolic Diseases Department, University Hospital of Strasbourg-Filière G2M, Strasbourg, France.; Pediatric Intensive Care Unit, University Hospital of Strasbourg, Strasbourg, France.; Pediatric Department, Reference Center for Inborn Error of Metabolism, Hôpital Necker-Enfants Malades, APHP and Université Paris Cité, Paris, France.; Département Médicaments et Technologies pour La Santé (DMTS), Paris-Saclay University, CEA, Gif-sur-Yvette, France.; Inserm UMRS_1163, Institut Imagine, Paris, France.; Pediatric Department, Reference Center for Inborn Error of Metabolism, Hôpital Necker-Enfants Malades, APHP and Université Paris Cité, Paris, France.; Département Médicaments et Technologies pour La Santé (DMTS), Paris-Saclay University, CEA, Gif-sur-Yvette, France.; Pediatric Department, Reference Center of Inborn Errors of Metabolism (ORPHA67872), University Regional Hospital Center of Nancy, INSERM UMRS 1256 NGERE-Nutrition, Genetics, and Environmental Risk Exposure, Nancy, France.; Pediatric Intensive Care Unit, University Regional Hospital Center of Nancy, Nancy, France.; Research Center, Sainte-Justine Hospital, University of Montreal, Montreal, QC, Canada.

DOI: 10.1097/CCE.0000000000001413

MeSH Terms: Humans, Amino Acids, Continuous Renal Replacement Therapy, Retrospective Studies, Male, Female, Middle Aged