Table 8. [Treatment of Manifestations in Individuals with Hereditary Fructose Intolerance]. - GeneReviews®

Manifestation/ Concern	Treatment	Considerations/Other
Acute presentation	Symptomatic in hospital setting	Incl: Intravenous glucose (dextrose) Supportive treatment of hepatic insufficiency (incl fresh frozen plasma or exchange transfusion) Treatment of metabolic acidosis, if present
Acute episodes of intoxication	In hospital setting	Immediate & complete elimination of fructose (by substitution of fructose w/other carbohydrate sources incl glucose, maltose, & cornstarch) to rapidly reverse symptoms & normalize related metabolic disturbances
Dietary restriction of fructose, sucrose, & sorbitol	By nutritionist w/specific experience in treating HFI & other inherited metabolic diseases	See Prevention of Primary Manifestations. Dietary assessment should be ongoing as the child grows, develops different tastes, & tries different foods.
Incl ongoing education of children & adults re need for dietary restriction
Other	Medically approved alert bracelet/necklace worn at all times
Hepatomegaly	Remove sources of fructose. Treat manifestations of hepatic insufficiency (e.g., hypoalbuminemia, coagulopathy).	Abdominal ultrasound & assessment of liver enzymes
Renal involvement	Remove sources of fructose.	Monitor renal function until normalized. If necessary, implement renal replacement therapy.

Manifestation/
Concern

Treatment

Considerations/Other

Acute
presentation

Symptomatic in hospital setting

Incl:

Intravenous glucose (dextrose)
Supportive treatment of hepatic insufficiency (incl fresh frozen plasma or exchange transfusion)
Treatment of metabolic acidosis, if present

Acute episodes
of intoxication

In hospital setting

Immediate & complete elimination of fructose (by substitution of fructose w/other carbohydrate sources incl glucose, maltose, & cornstarch) to rapidly reverse symptoms & normalize related metabolic disturbances

Dietary
restriction
of fructose,
sucrose, &
sorbitol

By nutritionist w/specific experience in treating HFI & other inherited metabolic diseases

See Prevention of Primary Manifestations.
Dietary assessment should be ongoing as the child grows, develops different tastes, & tries different foods.

Incl ongoing education of children & adults re need for dietary restriction

Other

Medically approved alert bracelet/necklace worn at all times

Hepatomegaly

Remove sources of fructose.
Treat manifestations of hepatic insufficiency (e.g., hypoalbuminemia, coagulopathy).

Abdominal ultrasound & assessment of liver enzymes

Renal
involvement

Remove sources of fructose.

Monitor renal function until normalized.
If necessary, implement renal replacement therapy.

From: Hereditary Fructose Intolerance

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

Seattle (WA): University of Washington, Seattle; 1993-2024.

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