Table 5.

Recommended Surveillance for Individuals with GSDVI

System/ConcernEvaluationFrequency
Ketosis Blood ketone level upon wakening using portable blood ketone meter.At least several times per month 1; goal is to maintain blood beta-OH-butyrate concentrations <0.3 mmol/L.
Hypoglycemia Self-glucose monitoringAt least several times per month & as needed 1
Gastrointestinal Liver ultrasoundAnnually beginning at age 5 yrs
Skeletal Bone densityWhen puberty is complete & as clinically indicated
Renal 24-hr urine to screen for microalbuminuriaAnnually
1.

Glucose and ketone levels should also be measured during times of stress including illness, intense activity, periods of rapid growth, or any time at which intake of food is reduced and before and after changes are made to the amount of cornstarch or protein intake. Monitoring recommendations should be tailored to individual needs.

From: Glycogen Storage Disease Type VI

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