A patient presents with Von-Gierk's disease and ketosis was detected on investigation. All of the following would he associated findings except:
(A) There is hypoglycemia (B) Have low blood sugar levels (C) Oxaloacetate is required for gluconeogenesis (D) Low fat mobilization
(D) Ans. d. Low fat mobilization (Ref Harper 's 27/e p/66)Excessive fatty acid mobilization (not the Low fat mobilization) from adipose tissues leads to ketosis in Von-Gierke's disease. Von Gierke's Disease:Type I glycogen storage disorder, inherited as autosomal recessiveQ, due to the deficiency of glucose-6 phosphataseDeficiency of glucose-6 phosphatase leads to low blood sugar and hypoglycemic attacks.Gluconeogenesis is enhanced, that's why Oxaloacetate is required for gluconeogenesis Excessive fatty acid mobilization from adipose tissues leads to ketosis