Revista chilena de nutrición
versión On-line ISSN 0717-7518
The disorders of fructose metabolism are produced by the following 3 enzyme defects. 1.- Fructokinase deficiency does not produce any symptom and treatment is not indicated. 2.- The deficiency of aldolase B produces hereditary fructose intolerance. This defect inhibits the transformation of fructose-1-phosphate in fructose 1,6 diphosphate. Symptoms appear when sugar is introduced in the diet, presenting nausea, vomits, pallor, sweating, trembling, lethargy, convulsions, hypoglycemia, hepatic failure, jaundice, edema and ascites. Treatment consists in eliminating fructose, sucrose, sorbitol in the diet. In the majority of cases the early treatment has an excellent result, vomiting disappears and renal function normalizes. 3.- The deficiency of fructose 1-6-biphosphatase impairs the formation of glucose from all neoglucogenic precursors including lactate, glycerol, alanine and dietary fructose. Frequently severe lactic acidosis, hypoglycemia, dyspnea, tachycardia, apneic spells, irritability, lethargy, coma, convulsions are observed. The treatment consists in preventing hypoglycemia and neoglucogenesis, avoiding prolonged fasting with frequent feeding.
Palabras llave : fructosemia; hypoglycemia; fructose.