The cause of hyperuricemia and gout in glucose-6phosphatase deficiency is :: (A) More formation of pentose, (B) Decreased availability of glucose to tissues, (C) Increased accumulation of sorbitol, (D) Impaired degradation of free radicals
Answer is A. A i.e More formation of pentose Von Gierke's (type Ia glycogen storage) disease d/t deficiency of glucose 6 phosphatase enzymeQ is autosomal recessive in inheritance. It is characterized by - Cells (of liver, renal tubular epithelium & intestinal mucosa) containing normal structured metabolically unavailable glycogen, hepato-renomegalyQ, doll like face, hypoglycemia, hyper-lipoemia, hyper cholesterolemia, fatty infiltration of liver, ketosisQ, acidemia, T lactic acid, t uric acid and stunted growth.