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A 4-month-old previously healthy, exclusively breast-fed male infant presented to the emergency department with severe tachypnoea and respiratory distress requiring intubation for transfer. Arterial blood gas revealed profound metabolic acidosis (pH 6.98) with markedly elevated lactate (18–20 mmol/L), low PaCO2 (16 mm Hg) and concomitant hypoglycaemia (34 mg/dL), suggesting a primary metabolic aetiology.