Background Hypoglycaemia between meals is one of the main problems in hereditary fructose intolerance (HFI). This is a recessive disorder caused by deficiency of fructose 1-phosphate aldolase, isozyme b, which catalyses cleavage of fructose-1-phosphate to form dihydroxyacetone phosphate and D-glyceraldehyde. The hypoglycaemia can follow fructose ingestion, as a result of accumulation of fructose l-phosphate, which inhibits the activation of hepatic phosphorylase and gluconeogenesis, or appears between meals, as a result of liver impairment.
Therapy involves elimination of fructose from the diet, so there are not many options to correct hypoglycaemia besides oral administration of glucose.
Purpose To ascertain that oral manose is an effective and save alternative to oral glucose in the rapid management of hypoglycaemia.
Material and methods Description of three cases: two infants diagnosed with HFI and another in whom it was suspected. Regarding refusal of patients to treatment with oral glucose, there is no published alternative treatment. Despite the fact that there is no experience with the use of oral mannose, we studied several sugar routes, including mannose, glucose and fructose, seeking common points between them, and we found that oral mannose could be an option. Treatment was started with 2 g three times daily. Glycaemia was measured on an outpatient basis between visits to the paediatrician, with a frequency of 3–4 times daily, and glycosylated haemoglobin was measured before every visit to the hospital.
Results Both glycaemic controls (all glycaemia values measured were between 78 and 100 mg/dL) and serum determinations (Hb1ac 5.1–5.7%) demonstrated correct glycaemic control during the observational period. Clinical improvement was shown in the children’s status.
Conclusion Despite the limited number of patients and that a conclusion would require a well designed study, it seems that mannose could be an effective and safe alternative, as an option to avoid oral glucose, in the management of HFI glycaemic abnormalities. This is the first information about this problem to our knowledge.
References and/or Acknowledgements
Haymond MW. Hypoglycemia in infants and children. Endocrinol Metab Clin North Am 1989;18:211
Ali M, Rellos P, Cox TM. Hereditary fructose intolerance. J Med Genet 1998;35:353
References and/or AcknowledgementsNo conflict of interest.
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