Table 5.

Acute In-Patient Treatment in Individuals with Fructose-1,6-Bisphosphatase Deficiency

Manifestation/
Concern		TreatmentConsiderations
Hypoglycemia
1.

IV glucose bolus (2 mL/kg of 10% dextrose) followed by continuous infusion of glucose at high rates (10% dextrose infusion)

2.

Transition to oral/enteral feeds as clinically tolerated

The symptoms of acute illness typically subside soon after administration of IV dextrose & child should recover quickly (w/in hrs), usually w/no residual damage.
Metabolic acidosis
1.

IV glucose bolus as above

2.

If pH remains <7.1 or worsens, administer NaHCO3 as 1/2 the calculated dose over a 30-min period.

3.

Restrict fructose, glycerol, sucrose, & sorbitol.

  • Acidosis usually corrects quickly w/out NaHCO3 infusion.
  • No consensus exists re restriction of dietary fructose & sucrose.
Hepatomegaly & ↑ transaminases NoneTransient findings that resolve spontaneously

IV = intravenous; NaHCO3 = sodium bicarbonate

From: Fructose-1,6-Bisphosphatase Deficiency

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