Fructose 1,6-Diphosphatase Deficiency Follow-up

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

Further Inpatient Care

Challenge with fructose or a fasting study should be performed only under the close supervision of a pediatric endocrinologist or metabolic specialist in an inpatient setting.

Several Asian countries use glycerol solution containing 5% fructose to manage cerebral edema. Such solutions should not be administered to infants and children because most patients with fructose-1,6-diphosphatase (FDPase) deficiency are undiagnosed. These agents can be fatal.

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Deterrence/Prevention

Avoidance of food that contains fructose prevents the metabolic crisis that results from this disorder.

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Complications

Fatal hepatic or renal injury has been reported from the metabolic crisis associated with FDPase deficiency.

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Prognosis

With prompt diagnosis of this disorder, the prognosis is excellent.

Several successful pregnancies in affected mothers have been reported. [9]

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Patient Education

Parents and patients should be counseled by a dietitian regarding the fructose and sorbitol content of various foods.

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