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Oral cornstarch therapy: is persorption harmless?

R Gitzelmann1, M A Spycher

  • 1Department of Pediatrics, University of Zürich, Kinderspital, Switzerland.

European Journal of Pediatrics
|July 1, 1993
PubMed
Summary
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Patients with glycogen storage disease Ia on cornstarch therapy showed starch granules in urine, indicating persorption. Amyluria was higher in these patients, suggesting potential long-term risks despite successful treatment.

Area of Science:

  • Biochemistry
  • Gastroenterology
  • Pediatrics

Background:

  • Glycogenosis Ia (von Gierke disease) is a metabolic disorder.
  • Leucine-sensitive hypoglycemia is a related condition.
  • Oral cornstarch therapy is a common treatment for glycogen storage diseases.

Purpose of the Study:

  • To investigate the presence and implications of starch persorption in patients with glycogenosis Ia undergoing cornstarch therapy.
  • To compare amyluria levels in treated patients versus untreated controls.

Main Methods:

  • Urine sediment analysis from four patients with glycogenosis Ia on cornstarch therapy.
  • Comparison of starch granule presence and amyluria levels with untreated controls.

Main Results:

Related Experiment Videos

  • Starch granules were detected in the urine sediments of patients on cornstarch therapy.
  • Evidence of persorption (uptake of undissolved starch particles) was observed.
  • Amyluria was significantly more pronounced in treated patients compared to controls.

Conclusions:

  • Cornstarch therapy for glycogenosis Ia leads to starch persorption.
  • While effective and generally safe, the potential for late adverse reactions due to persorbed starch warrants consideration.
  • Further monitoring for long-term effects of starch persorption is recommended.