Calcium kinetics in glycogen storage disease type 1a.

Abstract:

:Glycogen storage disease type 1a (Von Gierke's disease) is one of the more common glycogen storage diseases (GSD). GSD 1a patients can have severe idiopathic osteopenia, often beginning at a young age. Since calcium tracer studies offer a sensitive probe of the bone microenvironment and of calcium deposition, kinetics might be disturbed in patients with GSD 1a. Plasma dilution kinetics obtained using the stable isotope 42Ca are shown in this paper to be quite different between GSD 1a patients and age-matched controls. Comparison of kinetic parameters in these two populations is made using a new binding site model for describing calcium dynamics at the plasma-bone interface. This model describes reversible binding of calcium ions to postulated short-term and long-term sites by a retention probability density function psi (t). Using this analysis, adult GSD subjects exhibited a significant decrease (P = 0.023) in the apparent half-life of a calcium ion on the longer-term site compared with controls. The general theory of calcium tracer dilution kinetics is then discussed in terms of a new model of short-term calcium homeostasis recently proposed by Bronner and Stein [5].

journal_name

Calcif Tissue Int

authors

Goans RE,Weiss GH,Vieira NE,Sidbury JB,Abrams SA,Yergey AL

doi

10.1007/BF00369209

subject

Has Abstract

pub_date

1996-12-01 00:00:00

pages

449-53

issue

6

eissn

0171-967X

issn

1432-0827

journal_volume

59

pub_type

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