Fructose 1,6-Diphosphatase Deficiency Treatment & Management

Updated: Nov 03, 2015
  • Author: Sunil Kumar Sinha, MD; Chief Editor: Maria Descartes, MD  more...
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Treatment

Medical Care

Complete avoidance of fructose, its cognate sugars (eg, sorbitol) and prolonged fasting prevents hypoglycemia and lactic acidosis. Nevertheless, ingestion of small amounts of fructose and related sugars may be tolerated in most patients with fructose 1,6-diphosphatase (FDPase) deficiency. No other specific medical therapy is required.

Patients may only exhibit hepatomegaly during the metabolic crisis, which resolves promptly with administration of dextrose.

Parenteral administration of fructose or sorbitol to a patient with FDPase deficiency can be fatal.

Sorbitol is a constituent of many basic foodstuffs and some sugarless chewing gums. The oral bioavailability of sorbitol from routine gum use is usually clinically insignificant. However, use and susceptibility to exposure widely vary; thus, sorbitol should be avoided by patients with FDPase deficiency whenever possible.

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Consultations

Consultation with a pediatric endocrinologist or metabolism specialist is recommended.

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Diet

Avoidance of fructose, cognate sugars, and prolonged fasting usually is sufficient to prevent hypoglycemia and lactic acidosis, particularly during febrile illness.

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