Journal of Inherited Metabolic Disease

, Volume 38, Issue 3, pp 529–536 | Cite as

In vitro digestion of starches in a dynamic gastrointestinal model: an innovative study to optimize dietary management of patients with hepatic glycogen storage diseases

  • Tatiéle Nalin
  • Koen Venema
  • David A. Weinstein
  • Carolina F. M de Souza
  • Ingrid D. S. Perry
  • Mario T. R. van Wandelen
  • Margreet van Rijn
  • G. Peter A. Smit
  • Ida V. D. Schwartz
  • Terry G. J. Derks


Uncooked cornstarch (UCCS) is a widely used treatment strategy for patients with hepatic glycogen storage disease (GSD). It has been observed that GSD-patients display different metabolic responses to different cornstarches. The objective was to characterize starch fractions and analyze the digestion of different starches in a dynamic gastrointestinal in vitro model. The following brands of UCCS were studied: Argo® and Great Value® from the United States of America; Brazilian Maizena Duryea® and Yoki® from Brazil; Dutch Maizena Duryea® from the Netherlands. Glycosade®, a modified starch, and sweet polvilho, a Brazilian starch extracted from cassava, were also studied. The starch fractions were analyzed by glycemic TNO index method and digestion analyses were determined by the TIM-1 system, a dynamic, computer-controlled, in vitro gastrointestinal model, which simulates the stomach and small intestine. The final digested amounts were between 84 and 86 % for the UCCS and Glycosade®, but was 75.5 % for sweet povilho. At 180 min of the experiment, an important time-point for GSD patients, the digested amount of the starches corresponded to 67.9–71.5 for the UCCS and Glycosade®, while it was 55.5 % for sweet povilho. In an experiment with a mixture of sweet polvilho and Brazilian Maizena Duryea®, a final digested amount of 78.4 % was found, while the value at 180 min was 61.7 %. Sweet polvilho seems to have a slower and extended release of glucose and looks like an interesting product to be further studied as it might lead to extended normoglycemia in GSD-patients.


Starch Cornstarch Resistant Starch Glycogen Storage Disease Endogenous Glucose Production 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



Continuous nocturnal gastric drip feeding

GTI method

Glycemic TNO index method


Glycogen storage disease


Rapidly available glucose


Resistant starch


Slowly available glucose


TNO intestinal model-1


Uncooked cornstarch



The authors thank Carlota Bussolo de Souza (TNO) for analytical help during the experiments and Marion G. Priebe (UMCG) for fruitful discussions. They also thank the CAPES Foundation/Ministry of Education of Brazil, CNPq Foundation/Ministry of Science, Technology and Innovation of Brazil,  CNPq Foundation - Chamada 31/2013 – Doenças Metabólicas e Endócrinas, and FAPERGS Foundation - PPSUS/2013, and Vitaflo for their financial support.

Conflict of interest


Details of funding

CAPES Foundation/Ministry of Education of Brazil, CNPq Foundation/Ministry of Science, Technology and Innovation of Brazil, CNPq - Chamada N° 31/2013 – Doenças Metabólicas e Endócrinas, and FAPERGS Foundation - PPSUS/2013 provided grants for training and travel expenses of Tatiéle Nalin. Vitaflo provided a grant for bench fee for the laboratory studies and travel expenses of Tatiéle Nalin.


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Copyright information

© SSIEM 2014

Authors and Affiliations

  • Tatiéle Nalin
    • 1
    • 2
  • Koen Venema
    • 3
    • 9
  • David A. Weinstein
    • 4
  • Carolina F. M de Souza
    • 2
  • Ingrid D. S. Perry
    • 5
    • 6
  • Mario T. R. van Wandelen
    • 3
  • Margreet van Rijn
    • 7
  • G. Peter A. Smit
    • 7
  • Ida V. D. Schwartz
    • 1
    • 2
    • 8
  • Terry G. J. Derks
    • 7
  1. 1.Post-Graduation Program in Genetics and Molecular BiologyUniversidade Federal do Rio Grande do SulPorto AlegreBrazil
  2. 2.Medical Genetics Service, Hospital de Clínicas de Porto AlegrePorto AlegreBrazil
  3. 3.The Netherlands Organization for Applied Scientific Research (TNO)DelftThe Netherlands
  4. 4.Glycogen Storage Disease Program, Division of Pediatric Endocrinology, Department of PediatricsUniversity of FloridaGainesvilleUSA
  5. 5.Food and Nutrition Research Centre, Hospital de Clínicas de Porto AlegrePorto AlegreBrazil
  6. 6.Health UnitUniversidade do Extremo Sul CriciúmaCriciúmaBrazil
  7. 7.Section of Metabolic Diseases, Beatrix Children’s Hospital, University Medical Center of GroningenUniversity of GroningenGroningenThe Netherlands
  8. 8.Department of GeneticsUniversidade Federal do Porto AlegreRio Grande do SulBrazil
  9. 9.Beneficial Microbes ConsultancyWageningenThe Netherlands

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