Alternatively, continuous overnight nasogastric feeding can provide glucose. GSD IV has been treated with liver transplantation. Allopurinol may be needed to prevent gout and renal urate calculi. A high-protein diet is helpful for some patients. Prolonged ingestion of fructose may cause proximal renal tubular acidosis with urinary loss of phosphate and glucose, cirrhosis, and mental deterioration.
Diagnosis is suggested by the onset of symptoms in infancy with recent fructose intake.
IV glucose should be given as soon as the fall in blood glucose has been documented. Diagnosis and identification of heterozygous carriers of the mutated gene can be made by direct DNA analysis. Avoidance of sugar-containing sweets and fruit results in teeth that are usually free of caries. Fructose-induced hypoglycemia is treated with glucose. A genetic defect of fructokinase resulting in benign and asymptomatic excretion of fructose in the urine.