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BCHM2072/2972

BCHM2072/2972. 40 Minute Famine?. Presentation and history.

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BCHM2072/2972

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  1. BCHM2072/2972 40 Minute Famine?

  2. Presentation and history A 3-year old boy, Mark, is brought in suffering from stupor and very rapid breathing (hyperpnoea). His mother reports that he is very prone to suffer from sweats, trembling and occasionally convulsions after more than a few hours without food. Mark has an enlarged abdomen and, on examination, is found to have hepatomegaly (an enlarged liver). Mark also appears to have several xanthomas (nodules of lipid) around his body. Blood and urine samples were taken from Mark on his arrival and sent for biochemical analysis. An 'instant' blood glucose reading showed that Mark's glycemia was only 2 mM. After intravenous infusion of glucose, Mark's condition stabilised but he was kept in hospital was subjected to further tests.

  3. Initial Blood Assays Metabolite/EnzymeControl (range)Mark Glucose (mM) 4.5 - 5.5 1.9 Triglycerides (mM) 0.6 - 3.2 8.1 Fatty acids (mM) 0.2 - 0.8 4.0 Lactate (mM) 0.6 - 1.8 10.5 Uric Acid (mM) 0.2 - 0.4 2.9 Alanine aminotransferase (mU ml-1) 10 – 40 170 Bilirubin (uM) 3 - 15 70 pH 7.35 - 7.45 7.18 Ketone bodies (mM) <0.5 0.6 Insulin (pM) 20 – 100 2 Glucagon (pM) 20 – 50 250

  4. Galactose tolerance testThis involved infusing Mark with 200 mg/kg galactose via one of his veins.

  5. Glucagon tolerance testThis involved infusing Mark with intravenous glucagon.

  6. Dual-labelled glucose infusionThis involved infusing Mark with intravenous glucose which is labelled with 14C on every carbon atom but with 3H (tritium) on C2. To help interpret this data, have a look at the reactions that occur at the 'top' (hexose stages) of glycolysis/gluconeogenesis.Focus on the interconversion of G, G6-P, F6-P & F1,6-BP) and see if the ratio of 14C to 3H would be expected to change if glucose went down glycolysis and back again.

  7. Liver biopsyEnzyme was measured under Vmax conditions. Enzyme/metabolic fluxControl (range) Mark Glucose 6-phosphatase (µmol/min/(g tissue)) in extracts of frozen tissue 8 – 12 10 using fresh tissue 8 – 12 0.1 Glycogen (mg/(g wet weight tissue)) 6 – 9 22.2

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