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    Subjects/Biochemistry/TCA Cycle
    TCA Cycle
    hard
    flask-conical Biochemistry

    A 3-year-old child presents with recurrent episodes of hypoketotic hypoglycemia, developmental delay, and hepatomegaly. Plasma lactate is markedly elevated (8 mmol/L, normal <2). Urine organic acids show elevated dicarboxylic acids. Which investigation is most appropriate to confirm a defect in the TCA cycle?

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