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Table 9 Triggers, clinical signs & symptoms and biochemical signs of acute decompensation in MMA/PA*

From: Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

Triggers Clinical signs and symptoms Biochemical signs
Infection Poor feeding Metabolic acidosis (pH <7.3, anion gap >20 mmol/l, low pCO2 or base excess greater than -5 mmol/l)
Fever Vomiting
Prolonged fasting Lethargy Elevated blood lactate (>3 mmol/l)
Medication (e.g. chemotherapy, high dose glucocorticoids) Hypotonia Hyperammonemia
Prolonged or intense physical exercise, surgery and/or general anesthesia Irritability Ketonuria (greater than trace in infants or greater than + in children)
Acute trauma, significant hemorrhage Respiratory distress Uric acid and/or elevated urinary urea (urea/creatinine > 20) as signs of catabolism
Psychological stress Hypothermia Neutropenia
Excessive protein intake Dehydration and weight loss Thrombocytopenia
  1. *Please note that columns are independent from each other. Thus a given line in a column does not refer to the line in the neighboring column.
  2. Grade of recommendation: D.