ReviewJournal of inherited metabolic disease2015
Treatment options for lactic acidosis and metabolic crisis in children with mitochondrial disease.
Review in Journal of inherited metabolic disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed.
- Type B Lactic Acidosis: Diagnosis, Etiology and Treatment - An Educational Review.Cardiology research · 2026Review
- Reversible Metabolic and Liver Disease in Complex III Deficiency: Novel Variants Expand the ReportedCells · 2026Article
- Pediatric bronchial ulcers induced by ibuprofen overdose: a case report combined with network toxicology to decipher potential mechanisms.Frontiers in pharmacology · 2026Article
- Asymmetric Synthesis, Structure Determination, and Biologic Evaluation of Isomers of TLAM as PFK1 Inhibitors.ACS medicinal chemistry letters · 2025Article
- A coordinated multiorgan metabolic response contributes to human mitochondrial myopathy.EMBO molecular medicine · 2023Article
- Defining the ATPome reveals cross-optimization of metabolic pathways.Nature communications · 2020Article
- VARS2-linked mitochondrial encephalopathy: two case reports enlarging the clinical phenotype.BMC medical genetics · 2019Article
- A fatal case ofJIMD reports · 2019Article
- Recent topics: the diagnosis, molecular genesis, and treatment of mitochondrial diseases.Journal of human genetics · 2019Review
- A high-throughput screen of real-time ATP levels in individual cells reveals mechanisms of energy failure.PLoS biology · 2018Article
- Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations.Cell metabolism · 2018Article
- Neonatal encephalocardiomyopathy caused by mutations in VARS2.Metabolic brain disease · 2017Article
- Mitochondrial Dysfunction Plus High-Sugar Diet Provokes a Metabolic Crisis That Inhibits Growth.PloS one · 2016Article
- Asparagine Synthetase Deficiency causes reduced proliferation of cells under conditions of limited asparagine.Molecular genetics and metabolism · 2015Article
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Authors and funding
9 authors.
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Abstract
The mitochondrial pyruvate oxidation route is a tightly regulated process, which is essential for aerobic cellular energy production. Disruption of this pathway may lead to severe neurometabolic disorders with onset in early childhood. A frequent finding in these patients is acute and chronic lactic acidemia, which is caused by increased conversion of pyruvate via the enzyme lactate dehydrogenase. Under stable clinical conditions, this process may remain well compensated and does not require specific therapy. However, especially in situations with altered energy demands, such as febrile infections or longer periods of fasting, children with mitochondrial disorders have a high risk of metabolic decompensation with exacerbation of hyperlactatemia and severe metabolic acidosis. Unfortunately, no controlled studies regarding therapy of this critical condition are available and clinical outcome is often unfavorable. Therefore, the aim of this review was to formulate expert-based suggestions for treatment of these patients, including dietary recommendations, buffering strategies and specific drug therapy. However, it is important to keep in mind that a specific therapy for the underlying metabolic cause in children with mitochondrial diseases is usually not available and symptomatic therapy especially of severe lactic acidosis has its ethical limitations.
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