Table 10.

Recommended Surveillance for Individuals with Hereditary Fructose Intolerance

System/ConcernEvaluationFrequency
Diet/Nutrition
  • Dietary assessment by nutritionist as child grows, develops different tastes, & tries different foods
  • Monitor for vitamin & mineral deficiencies.
Every 3-4 mos in 1st yr of life; every 6-12 mos thereafter
Liver dysfunction/
NAFLD
Abdominal ultrasound, liver function tests to assess progression of NAFLDEvery 6-12 mos
Renal dysfunction BUN, creatinine, cystatin C, urine amino acids, plasma electrolytes to assess renal function (in setting of unintentional, ongoing ingestion of fructose)Every 6-12 mos
Hypoglycemia Plasma glucoseAs needed for findings such as lethargy, seizures, jitteriness, diaphoresis
Chronic excess
fructose ingestion
  • N-glycan eval
  • Plasma lysosomal enzyme analysis
  • Dietary intake assessment
Every 6-12 mos

BUN = blood urea nitrogen; NAFLD = non-alcoholic fatty liver disease

From: Hereditary Fructose Intolerance

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