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A hypoglycaemic adolescent with an enlarged liver and gout

Objectives

By the end of this exercise you should be able to:

Distinguish between different possible ways in which enzyme or hormone receptor defects may lead to excessive accumulation of glycogen in the liver, and fasting hypoglycaemia.

Explain how lack of glucose 6-phosphatase leads to excessive accumulation of glycogen in the liver and profound hypoglycaemia on fasting.

Explain how lack of glucose 6-phosphatase leads to lactic acidosis in the fasting state, and how this may lead to the development of gout.