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Calcium kinetics in glycogen storage disease type 1a

R E Goans1, G H Weiss, N E Vieira

  • 1Laboratory of Theoretical and Physical Biology, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA.

Calcified Tissue International
|December 1, 1996
PubMed
Summary

Glycogen storage disease type 1a (GSD 1a) patients show altered calcium kinetics in bone due to disturbed calcium deposition. This study reveals a significantly decreased calcium ion half-life on long-term bone sites in GSD 1a adults compared to controls.

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Area of Science:

  • Biochemistry
  • Bone Metabolism
  • Pediatric Endocrinology

Background:

  • Glycogen storage disease type 1a (GSD 1a), also known as Von Gierke's disease, is a common metabolic disorder.
  • Patients with GSD 1a frequently develop severe idiopathic osteopenia, even at a young age.
  • Calcium tracer studies are sensitive to bone microenvironment and calcium deposition dynamics.

Purpose of the Study:

  • To investigate calcium plasma dilution kinetics in patients with GSD 1a.
  • To compare calcium dynamics between GSD 1a patients and age-matched controls using a novel binding site model.
  • To analyze potential disturbances in calcium deposition and kinetics at the plasma-bone interface in GSD 1a.

Main Methods:

  • Utilized the stable isotope 42Ca for plasma dilution kinetic studies.

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  • Applied a new binding site model to describe calcium dynamics, including reversible binding to short-term and long-term sites.
  • Analyzed kinetic parameters such as the retention probability density function psi (t).

    • Main Results:

    • Demonstrated significant differences in plasma dilution kinetics between GSD 1a patients and controls.
    • Adult GSD subjects showed a statistically significant decrease (P = 0.023) in the apparent half-life of calcium ions on longer-term bone sites compared to controls.
    • The findings suggest impaired calcium homeostasis at the plasma-bone interface in GSD 1a.

    Conclusions:

    • Calcium kinetics are demonstrably altered in patients with Glycogen storage disease type 1a.
    • The study highlights a reduced calcium retention on long-term bone sites, contributing to osteopenia in GSD 1a.
    • The findings support the need for further investigation into calcium metabolism and bone health in GSD 1a patients.