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A novel starch for the treatment of glycogen storage diseases

  • Original Article
  • Published:
Journal of Inherited Metabolic Disease

Summary

Objective:

To determine whether a new starch offers better short-term metabolic control than uncooked cornstarch in patients with glycogen storage diseases (GSDs).

Study design:

A short-term double-blind cross-over pilot study comparing uncooked physically modified cornstarch (WMHM20) with uncooked cornstarch in patients with GSD types Ia, Ib and III. Twenty-one patients (ages 3–47, 9 female) were given 2 g/kg cornstarch or WMHM20 mixed in water. Blood glucose, lactate and insulin, and breath hydrogen and 13CO2 enrichment were measured, at baseline and after each load. The hourly biochemical evaluations terminated when blood glucose was ≤3.0 mmol/L, when the study period had lasted 10 h or when the patient wished to end the test. The alternative starch was administered under similar trial conditions a median of 10 days later.

Results:

The median starch load duration was 9 h for WMHM20 versus 7 h for cornstarch. Glucose decreased more slowly (p=0.05) and lactate was suppressed faster (p=0.17) for WMHM20 compared with cornstarch. Peak hydrogen excretion was increased (p=0.05) when cornstarch was taken.

Conclusion:

These data indicate longer duration of euglycaemia and better short-term metabolic control in the majority of GSD patients with WMHM20 compared to cornstarch.

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Abbreviations

CNPF:

continuous nocturnal pump feed

GSD:

glycogen storage disease

IQ:

interquartile range

PDB:

Pee Dee Belemnite

UCCS:

uncooked cornstarch

WMHM20:

Waxy Maize (Heat Modified) 20

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Author information

Authors and Affiliations

  1. Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK

    K. Bhattacharya, H. Mundy & P. J. Lee

  2. Metabolic Unit, Great Ormond Street Hospital, London, UK

    K. Bhattacharya, R. C. Orton, H. Mundy & P. J. Lee

  3. Department of Medicine, University College London, London, UK

    K. Bhattacharya, H. Mundy & P. J. Lee

  4. Glycologic Ltd, Glasgow, UK

    X. Qi & R. F. Tester

  5. Environmental Change Research Centre, University College London, London, UK

    D. W. Morley

  6. Department of Paediatric Metabolic Medicine, Evelina Children’s Hospital, London, UK

    M. P. Champion

  7. Department of Biochemistry, Endocrinology and Metabolism, Institute of Child Health, University College London, London, UK

    S. Eaton

Authors
  1. K. Bhattacharya
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  2. R. C. Orton
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  3. X. Qi
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  4. H. Mundy
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  5. D. W. Morley
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  6. M. P. Champion
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  7. S. Eaton
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  8. R. F. Tester
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  9. P. J. Lee
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Corresponding author

Correspondence to K. Bhattacharya.

Additional information

Communicating editor: René Santer

Competing interests: None declared

References to electronic databases: Glycogen storage disease I, OMIM 232200. Glycogen storage disease III, OMIM 232400. Glucose-6-phosphatase, EC 3.1.3.9. Amylo-1,6-glucosidase, EC 3.2.1.33. Oligo-1,4-1,4-glucanotransferase, EC 2.4.1.25

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Bhattacharya, K., Orton, R.C., Qi, X. et al. A novel starch for the treatment of glycogen storage diseases. J Inherit Metab Dis 30, 350–357 (2007). https://doi.org/10.1007/s10545-007-0479-0

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  • DOI: https://doi.org/10.1007/s10545-007-0479-0

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