The Use of Uncooked Sweet Polvilho to Treat Hepatic Glycogen Storage Diseases

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Brief Title

The Use of Uncooked Sweet Polvilho to Treat Hepatic Glycogen Storage Diseases

Official Title

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

Brief 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
      polvilho, 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 polvilho in the treatment of patients with
      hepatic Glycogen Storage Diseases, using as model the Glycogen Storage Diseases type Ia.

Detailed Description

      A randomize and crossover study will be conducted on two consecutive days, comparing the use
      of classic cornstarch to the use of sweet polvilho 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 polvilho 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 polvilho, 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 polvilho, or the opposite.If
      the patient has hypoglycaemia during the study, it will be discontinued.

Study Type


Primary Outcome

Duration of normoglycemia

Secondary Outcome

 Glucose levels


Glycogen Storage Disease Type I


sweet polvilho

Study Arms / Comparison Groups

Description:  Ingestion of cornstarch, the standard treatment for hepatic GSD.


* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information

Recruitment Status

Dietary Supplement

Estimated Enrollment


Start Date

April 29, 2017

Completion Date

May 31, 2020

Primary Completion Date

August 30, 2019

Eligibility Criteria

        Inclusion Criteria:

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

        Exclusion Criteria:





16 Years - N/A

Accepts Healthy Volunteers



Ida D Schwartz, Dr, , 

Location Countries


Location Countries


Administrative Informations



Organization ID


Responsible Party


Study Sponsor

Hospital de Clinicas de Porto Alegre


 Conselho Nacional de Desenvolvimento Científico e Tecnológico

Study Sponsor

Ida D Schwartz, Dr, Principal Investigator, Hospital de Clínicas de Porto Alegre

Verification Date

February 2020