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    Subjects/Biochemistry/Amino Acidurias — PKU, Alkaptonuria, Maple Syrup
    Amino Acidurias — PKU, Alkaptonuria, Maple Syrup
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    flask-conical Biochemistry

    A 6-year-old boy from rural India presents with progressive joint pain, stiffness in the knees and ankles, and dark urine that turns black on standing. His urine reduces Fehling's reagent but is negative on glucose oxidase. Physical examination reveals blue-black discolouration of the sclera and cartilage of the ears. Which investigation is most specific for confirming the diagnosis of alkaptonuria?

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