<prompt> Baby has hypoglycaemia, specially early morning hypoglycaemia. Glucagon given. It raises blood glucose if given after meals But does not raises blood glucose if given during fasting. Liver biopsy shows increased glycogen deposits. Enzyme defect is ?Muscle phosphorylaseGlucose-6-phosphataseBranching enzymeDebranching enzyme  <response>: This patient has Debranching enzyme deficiency, also known as limit dextrinosis or Coris Disease. Normally this enzyme releases just 10 glucose. So the patient has only early morning hypoglycemia in its deficiency i.e. mild hypoglycemia. No hypoglycemia during the daytime, when meals are taken. Limit dextrins a branched oligosaccharide having lots of alpha 1-- 6 bonds are accumulated in this patients liver during fasting so if glucagon given during fasting, phosphorylase rate-limiting enzyme of glycogenolysis, which is activated by Glucagon will be activated which cannot break limit dextrins. Phosphorylase enzyme can only break alpha 1-- 4 bonds. So this will not raise blood glucose. In fed state glycogen is formed. So if glucagon is given during fed state, phosphorylase will be activated which can break glycogen has both alpha 1-- 4 alpha 1--6 glycosidic bonds can raise blood glucose. In von Gierkes disease, if glucagon is given, blood glucose will not be increased and in this disease, the patient has severe hypoglycemia as both glycogenolysis and gluconeogenesis cannot occur due to deficiency of Glucose-6-phosphatase - So, Option B is not the answer. 