The Use of Uncooked Sweet Manioc Starch to Treat Hepatic Glycogen Storage Diseases

Glycogen Storage Disease Type I Clinical Trial

Official title:

A Novel Approach to Treatment of Hepatic Glycogen Storage Diseases: a Study Based on the Use of Uncooked Sweet Manioc Starch

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT03871673
• Other study ID #: 160056
• Status: Completed
• Phase: N/A
• Start date: April 29, 2017
• Est. completion date: May 31, 2021

Study information

• Verified date: September 2023
• Source: Hospital de Clinicas de Porto Alegre
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

Hepatic Glycogen Storage Diseases are a group of 10 serious genetic diseases that present in childhood and are characterized more frequently by the occurrence of repetitive hypoglycemia and dyslipidemia. Regarding treatment, the most commonly used strategy is the frequent administration of uncooked cornstarch, in average, every 4 hours. Although this treatment is successful, the use of large amounts of cornstarch can lead to overweight and, especially, to the decrease in the quality of life of patients and caregivers, due to the need to use the starch during the night. The search for a treatment that is widely available and that can lead to the prolongation of the fasting time, can collaborate to improve the care of these patients. The main scientific question to be answered by this research is: does sweet manioc starch, a Brazilian product, safely prolong the fasting time (with normoglycemia) of the patients as already suggested in experimental models? Main objective: To evaluate the efficacy and safety of the use of uncooked Sweet Manioc Starch in the treatment of patients with hepatic Glycogen Storage Diseases, using as model the Glycogen Storage Diseases type Ia.

Description:

A randomize and crossover study will be conducted on two consecutive days, comparing the use of classic cornstarch to the use of sweet manioc starch in patients with Glycogen Storage Disease type Ia. Ten patients diagnosed with Glycogen Storage Disease type Ia, confirmed by genetic analysis, will be included in the study. Sweet manioc starch and cornstarch, both of Brazilian origin, were analyzed in a previous study of the team in the artificial gastrointestinal model (TIM-1). They will be supplied uncooked, in the same way that cornstarch is currently used in the treatment of hepatic Glycogen Storage Diseases. Doses administered to patients will be 100 g of starch diluted in 200 ml of water at room temperature. To carry out the study, the patients will remain two consecutive nights in the hospital. They will remain in their normal diet until the administration of the starches at 22 hours. To perform the test, at 10 pm blood collection will be performed and after that the patient will ingest the starch (cornstarch or sweet manioc starch, determined by previous randomization). In sequence, blood collection will be performed every hour until the patient has a serum glucose value below 70mg/dL or after the patient stays 10h in a fast. Afterwards, the patient will return to his usual diet until the next test at 22 hours. The same procedures will be performed on the second night, the only change will be the starch used, and if the patient ingested uncooked cornstarch the first night, the same night will ingest the sweet manioc starch, or the opposite.If the patient has hypoglycaemia during the study, it will be discontinued.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 11
• Est. completion date: May 31, 2021
• Est. primary completion date: August 30, 2019
• Accepts healthy volunteers: No
• Gender: All
• Age group: 16 Years and older

Eligibility

Inclusion Criteria:

• patients with a confirmed molecular diagnosis of glycogen storage disease type Ia who are already on uncooked cornstarch therapy Exclusion Criteria: -

Related Conditions & MeSH terms

• Glycogen Storage Disease
• Glycogen Storage Disease Type I
• Metabolic Diseases

Intervention

Dietary Supplement:
• Sweet manioc starch
100 g of starch diluted in 200 ml of water at room temperature In the subsequent night the patient is crossed over to the other starch.
• Cornstarch
100 g of starch diluted in 200 ml of water at room temperature In the subsequent night the patient is crossed over to the other starch.

Locations

Country	Name	City	State
Brazil	Ida Vanessa D Schwartz	Porto Alegre	RS

Sponsors (3)

Lead Sponsor	Collaborator
Hospital de Clinicas de Porto Alegre	Conselho Nacional de Desenvolvimento Científico e Tecnológico, Fundação Médica do Rio Grande do Sul

Country where clinical trial is conducted

Brazil, 

References & Publications (1)

Monteiro VCL, de Oliveira BM, Dos Santos BB, Sperb-Ludwig F, Refosco LF, Nalin T, Derks TGJ, Moura de Souza CF, Schwartz IVD. A triple-blinded crossover study to evaluate the short-term safety of sweet manioc starch for the treatment of glycogen storage d — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Duration of normoglycemia	To verify the normoglycemia will be accessed glucose level. The glucose dosage will be performed by hexokinase enzyme assay. Peripheral blood samples will be collected to determine the baseline value and every hour.	up to 10 hours
Secondary	Glucose levels	The glucose dosage will be performed by hexokinase enzyme assay. Peripheral blood samples will be collected to determine the baseline value and every hour.	up to 10 hours
Secondary	Lactate levels	The lactase dosage will be performed by colorimetric (Lactate ? Pyruvate). Peripheral blood samples will be collected to determine the baseline value and every hour.	up to 10 hours
Secondary	Insulin levels	The insulin dosage will be performed by microparticle chemiluminescent immunoassay. Peripheral blood samples will be collected to determine the baseline value and every hour.	up to 10 hours

External Links

•   A triple-blinded crossover study to evaluate the short-term safety of sweet manioc starch for the treatment of glycogen storage disease type Ia