From the Guidelines

Elevated organic acids (OAs) have severe and potentially life-threatening effects on the body, particularly the nervous system and metabolism.

Toxic encephalopathy: associated with vomiting, poor feeding, and neurologic symptoms such as seizures, abnormal tone, and lethargy that progresses to coma 1
Metabolic crisis: prolonged fasting can produce a catabolic state and precipitate a metabolic crisis, which can be managed with proper intravenous glucose support and metabolic monitoring 1
Neurological deterioration: patients with organic acidemia, such as methylmalonic acidemia (MMA), remain at risk for neurological deterioration and/or progressive renal insufficiency following liver transplantation (LT) 1
Impaired brain development: classic variant maple syrup urine disease (MSUD) is associated with impaired brain development or unpredictable risk of neurologic crisis, despite strict dietary management 1

Diet: total parenteral nutrition, gelatin, medium-chain triglyceride oil supplementation, infant formula, and the ketogenic diet can contribute to abnormal organic acid profiles 1
Medications: valproate, levetiracetam, aspirin, benzoic acid, ibuprofen, acetaminophen, and many others can be the source of a wide variety of exogenous peaks 1
Clinical state: sample contamination, intestinal bacteria metabolism, and nutritional status can also affect organic acid levels 1

Autosomal recessive traits: most organic acidemias are inherited as autosomal recessive traits, with important implications for other family members 1
Urine organic acid analysis: a valuable tool for diagnosing organic acidemias and other inborn errors of metabolism, with diverse clinical indications including neonatal or late-onset acute illness, neurologic abnormalities, and unexplained metabolic acidosis 1

From the Research

Elevated OAs can contribute to acid-base imbalance and affect nitrogen balance and recycling 2
High anion gap metabolic acidosis with hyperammonemia is a characteristic biochemical finding of OA disorders 2
In plants, OAs play a role in stress tolerance, improving soil mineral acquisition and toxic metal tolerance 3
OAs can also act as chelating agents, binding metals, and as carbon sources for microbes 3
In animal nutrition, protected OAs can improve growth performance, nutrient digestibility, and decrease gas emission in broilers 4
OAs have been shown to have antimicrobial effects against "big six" Escherichia coli serotypes on fresh produce 5
The effectiveness of OAs in inhibiting pathogens can vary depending on the type of OA and the specific strain of the pathogen 5

OAs can accumulate in body fluids due to enzyme defects in amino acid catabolism pathways 2
Gas chromatography-mass spectrometry techniques (GC/MS) can be used to identify OAs in body fluids 2
OAs can be extracted from fermentation broths using solvents such as alcohols 6
The recovery of OAs from aqueous media and fermentation broths can be improved by successive extractions 6

OAs have potential applications in the production of ester-based new generation biofuels 6
OAs can be used as a treatment for OA disorders, with optimal metabolic control and regular monitoring being key to long-term management 2
OAs can be used to improve soil carbon sequestration and combat climate change 3
OAs can be used as a sanitizing agent for fresh produce, reducing the risk of foodborne illness 5