II. Pathophysiology

  1. Bongkrekic Acid
    1. Bongkrekic Acid is a branched tricarboxylic acid (heat stable, odorless, tasteless)
    2. Produced by the bacteria Burkholderia Gladioli pathovar cocovenenans (previously Pseudomonas cocovenenans)
    3. Mitochondrial toxin
      1. Triggers Neutrophil autophagy (internal PMN cleanup)
      2. Inhibitor of mitochondrial ADP and ATP translocases/transporters
        1. Blocks adenine Nucleotide efflux from mitochondria (via membrane binding enhancement)
      3. Inhibits mitochondrial Potassium ATP-sensitive channel (mitoKATP)
        1. MitoKATP is found on inner mitochondrial membrane
        2. MitoKATP regulates Potassium influx (and organelle volume, and cellular stress responses
  2. Burkholderia Gladioli pathovar cocovenenans (BGPC)
    1. Burkholderia Gladioli is an aerobic Gram-negative rod-shaped bacterium found worldwide in soil and water
      1. Opportunistic human and plant pathogen
      2. Burkholderia Gladioli is a pathogen found on the gladiolus plant where it causes vegetation to decay
    2. BGPC generates Bongkrekic Acid only under specific conditions (often within fermented food products)
      1. Temperature 22 to 30 C (71 to 86 F)
      2. pH 6.5 to 8
      3. Lipid substrate (oleic acid, lauric acid, palmitic acid)

III. Risk Factors

  1. Foodborne Illness primarily in Asia
    1. China
    2. Taiwan
    3. Bhutan
    4. Indonesia
    5. Mozambique
    6. Rare cases in the United States
  2. Fermented food ingestion
    1. Fermented coconut, soybean oil, corn or rice flour
    2. Auriculana auricula

IV. Findings

  1. Severity
    1. Mild Poisonings result in mild, self-limited Gastroenteritis
    2. Severe Poisonings result in Acute Liver Failure, encephalopathy and hemodynamic compromise
      1. Mortality rates in outbreaks range from 18 to >50%
  2. Timing
    1. Onset of symptoms by 6 hours after ingestion
  3. Gastrointestinal
    1. Nausea
    2. Vomiting
    3. Abdominal Pain
    4. Diarrhea
  4. Cardiovascular
    1. Acute myocardial injury
    2. Dilated Cardiomyopathy
  5. Liver
    1. Acute liver injury (hepatic necrosis)
    2. Coagulopathy
    3. Hyperbilirubinemia
    4. Hypoglycemia
  6. Neurologic
    1. Headache
    2. Dizziness
    3. Seizures
    4. Cerebral Edema
    5. Coma
  7. Renal
    1. Hematuria
    2. Oliguria
    3. Acute Kidney Injury

V. Labs

  1. See Unknown Ingestion
  2. Complete Blood Count
  3. Serum Troponin
  4. Comprehensive metabolic panel
    1. Metabolic acid with Anion Gap
  5. Lactic Acid
    1. Hyperlactemia
  6. Venous Blood Gas
    1. Metabolic Acidosis
  7. Bongkrekic Acid Levels (Whole blood or serum level, stool level)
    1. Not readily available in U.S.

VI. Imaging

VII. Diagnostics

VIII. Management

  1. Supportive care
  2. Gastrointestinal Decontamination
    1. May consider if presenting soon after large known ingestion
    2. However, Bongkrekic Acid absorption is rapid and Decontamination has a low likelihood of benefit
  3. Enhanced Elimination
    1. Multi-dose Activated Charcoal
    2. Plasma Exchange
  4. Hospital admission criteria
    1. Abnormal transaminases (AST, ALT)
    2. Acute Kidney Injury (increased Serum Creatinine, decreased Urine Output)
    3. Altered Mental Status
    4. Lactic Acidosis
    5. Refractory gastrointestinal symptoms (Nausea, Vomiting or Diarrhea)
  5. Emergency Department Discharge Criteria
    1. Controlled mild gastrointestinal symptoms at >6 hours after ingestion
    2. Normal laboratory tests
    3. Normal Vital Signs
    4. Normal mentation

IX. Prognosis: Poor Prognostic Findings

  1. Bongkrekic Acid Whole Blood Level
    1. Day 2: >100 ng/ml
    2. Day 10: >10 ng/ml
  2. Rapid onset within 6 hours
    1. Severe gastrointestinal symptoms
    2. Metabolic Acidosis
    3. Anuria

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