Safety and Efficacy of Chronic Extended Release Cornstarch Therapy for Glycogen Storage Disease Type I

Ross, Katalin M; Brown, Laurie M.; Corrado, Michelle M.; Chengsupanimit, Tayoot; Curry, Latravia M.; Ferrecchia, Iris A.; Porras, Laura Y.; Mathew, Justin; Weinstein, David A.

doi:10.1007/8904_2015_488

Cited by 34 publications

(42 citation statements)

References 16 publications

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“…To prevent fasting hypoglycemia in patients with GSD I and metabolic phenotype, frequent (every 3–4 h) oral uncooked cornstarch during the day and at night or a continuous nasogastric infusion of glucose is used [103] . A meta-analysis indicates that overnight intermittent administration of uncooked cornstarch prevents nocturnal hypoglycemia in GSD-Ia children more effectively than continuous nocturnal feeding of dextrose.…”

Section: Glucose Dephosphorylation: Glucose 6-phosphatase Systemmentioning

confidence: 99%

Glycogen metabolism in humans

Adeva‐Andany¹,

et al. 2016

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In the human body, glycogen is a branched polymer of glucose stored mainly in the liver and the skeletal muscle that supplies glucose to the blood stream during fasting periods and to the muscle cells during muscle contraction. Glycogen has been identified in other tissues such as brain, heart, kidney, adipose tissue, and erythrocytes, but glycogen function in these tissues is mostly unknown. Glycogen synthesis requires a series of reactions that include glucose entrance into the cell through transporters, phosphorylation of glucose to glucose 6-phosphate, isomerization to glucose 1-phosphate, and formation of uridine 5ʹ-diphosphate-glucose, which is the direct glucose donor for glycogen synthesis. Glycogenin catalyzes the formation of a short glucose polymer that is extended by the action of glycogen synthase. Glycogen branching enzyme introduces branch points in the glycogen particle at even intervals. Laforin and malin are proteins involved in glycogen assembly but their specific function remains elusive in humans. Glycogen is accumulated in the liver primarily during the postprandial period and in the skeletal muscle predominantly after exercise. In the cytosol, glycogen breakdown or glycogenolysis is carried out by two enzymes, glycogen phosphorylase which releases glucose 1-phosphate from the linear chains of glycogen, and glycogen debranching enzyme which untangles the branch points. In the lysosomes, glycogen degradation is catalyzed by α-glucosidase. The glucose 6-phosphatase system catalyzes the dephosphorylation of glucose 6-phosphate to glucose, a necessary step for free glucose to leave the cell. Mutations in the genes encoding the enzymes involved in glycogen metabolism cause glycogen storage diseases.

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Section: Glucose Dephosphorylation: Glucose 6-phosphatase Systemmentioning

confidence: 99%

Glycogen metabolism in humans

Adeva‐Andany¹,

et al. 2016

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“…Adding glucose is not recommended since it stimulates insulin production and offsets the advantage of the starch. Of note, a new extended release formulation (Glycosade) was recently introduced for night feeds, and it has allowed older children and adults to have a 7-10 hour period of coverage without sacrificing metabolic control [25].…”

Section: Treatmentmentioning

confidence: 99%

Glycogen storage diseases: Diagnosis, treatment and outcome

Chen¹,

Weinstein

2016

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100

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The glycogen storage diseases (GSDs) are a group of inherited metabolic disorders that result from a defect in any one of several enzymes required for either glycogen synthesis or glycogen degradation. The GSDs can be divided into those with hepatic involvement, which present as hypoglycemia, and those which are associated with neuromuscular disease and weakness. The severity of the GSDs range from those that are fatal in infancy if untreated to mild disorders with a normal lifespan. The diagnosis, treatment, and prognosis for the common types of GSDs are reviewed.

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“…The use of Glycosade, an extended release cornstarch, allows some subjects to extend the time between their nighttime doses. 9 The number of doses per day for all subjects is included in Table A1 in Appendix A. Total starch by body weight was not statistically significant, which supports the assertion that cornstarch should not be dosed by body weight, but rather by the carbohydrate requirement and central nervous system demands of each patient.…”

Section: Resultsmentioning

confidence: 54%

Cornstarch requirements of the adult glycogen storage disease Ia population: A retrospective review

Dahlberg

Ferrecchia

Dambska-Williams

et al. 2019

J of Inher Metab Disea

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Cornstarch has been the primary treatment for glycogen storage disease type Ia (GSD Ia) for over 35 years. When cornstarch was first described as a treatment, few people survived beyond early childhood. As the prognosis for this population has improved, the need to ensure appropriate cornstarch dosing for different age groups has become imperative. Records from 115 patients (10‐62 years of age) with GSD Ia evaluated at our center between 2015 and 2017 were reviewed. Data collected included weight, age, genetic mutation, amount and frequency of cornstarch doses, body mass index, gender, 24‐hour glucose and lactate concentrations, and biochemical markers of metabolic control. The data demonstrate that adult treatment needs vary greatly from younger age groups, and the required cornstarch support decreases with age (P < .001). The required number of doses, however, did not change with a mean of six doses (range 4‐8) daily in all age groups. General laboratory findings across time demonstrate that significantly reducing the amount of starch required to maintain euglycemia with aging can be done without sacrificing metabolic control. Carbohydrate requirements decrease with aging, and older patients were found to require less cornstarch. Failure to lower the cornstarch doses contributes to over‐treatment in adults with GSD Ia. Not only does this lead to worsening hepatomegaly and excessive weight gain, but over‐treatment contributes to relative hyperinsulinism and rebound hypoglycemia. This knowledge is essential in designing nutritional therapies for the aging GSD population.

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Safety and Efficacy of Chronic Extended Release Cornstarch Therapy for Glycogen Storage Disease Type I

Cited by 34 publications

References 16 publications

Glycogen metabolism in humans

Glycogen metabolism in humans

Glycogen storage diseases: Diagnosis, treatment and outcome

Cornstarch requirements of the adult glycogen storage disease Ia population: A retrospective review

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