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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.