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Growth and endocrine changes in the hepatic glycogenoses

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Abstract

The biochemical and endocrine responses of 13 patients with hepatic glycogen storage disease (HGSD) (type I-six patients, type Ib-two, type III-three, type IX-two patients) to an oral glucose load have been investigated. Longitudinal growth data was available in all patients. The height velocity standard deviation score (HVSDS) was positively correlated with the plasma somatomedin and inversely correlated with the glucose-insulin ratio, plasma cortisol and plasma growth hormone concentrations. There was no correlation between plasma glucagon and HVSDS. Free fatty acid and lactate concentrations were highest in the older untreated patients who were growing slowly.

In four patients improvement in the HVSDS with treatment was accompanied by a rise in plasma somatomedin and a fall in growth hormone and cortisol. In two patients the glucose-insulin ratio decreased.

Growth retardation in HGSD can be explained as part of the adaptation to the inability to maintain normal glucose homeostasis.

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Authors and Affiliations

  1. The Department of Child Health, Institute of Child Health, University of London, 30 Guilford Street, WC1N 1EH, London, England

    D. B. Dunger & J. V. Leonard

  2. The Department of Growth and Development, Institute of Child Health, University of London, 30 Guilford Street, WC1N 1EH, London, England

    A. T. Holder, J. Okae & M. A. Preece

Authors
  1. D. B. Dunger
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  2. A. T. Holder
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  3. J. V. Leonard
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  4. J. Okae
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  5. M. A. Preece
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Dunger, D.B., Holder, A.T., Leonard, J.V. et al. Growth and endocrine changes in the hepatic glycogenoses. Eur J Pediatr 138, 226–230 (1982). https://doi.org/10.1007/BF00441207

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  • DOI: https://doi.org/10.1007/BF00441207

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