Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia
Abstract Background Patients with glycogen storage disease type Ia (GSDIa) are prone to hypoglycemia. Uncooked cornstarch (CS) is the treatment, but maintaining nighttime blood glucose levels is still difficult. Methods The study enrolled patients with GSDIa to investigate the benefits of bedtime ex...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2023-10-01
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| Series: | Molecular Genetics & Genomic Medicine |
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| Online Access: | https://doi.org/10.1002/mgg3.2221 |
| _version_ | 1827795023643541504 |
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| author | Rai‐Hseng Hsu Hui‐An Chen Yin‐Hsiu Chien Wuh‐Liang Hwu Ju‐Li Lin Hui‐Ling Weng Yi‐Ting Lin Yu‐Ching Lin Ni‐Chung Lee |
| author_facet | Rai‐Hseng Hsu Hui‐An Chen Yin‐Hsiu Chien Wuh‐Liang Hwu Ju‐Li Lin Hui‐Ling Weng Yi‐Ting Lin Yu‐Ching Lin Ni‐Chung Lee |
| author_sort | Rai‐Hseng Hsu |
| collection | DOAJ |
| description | Abstract Background Patients with glycogen storage disease type Ia (GSDIa) are prone to hypoglycemia. Uncooked cornstarch (CS) is the treatment, but maintaining nighttime blood glucose levels is still difficult. Methods The study enrolled patients with GSDIa to investigate the benefits of bedtime extended release CS (ER‐CS, Glycosade®) versus regular CS. The daytime CS schedule was not altered. A 7‐day continuous glucose monitoring (CGM) was performed at the baseline and 12 weeks after using ER‐CS. Biochemical profile, sleep quality (Pittsburgh Sleep Quality Index, PSQI), and quality of life (SF‐36 questionnaire) were measured at the baseline and 24 weeks after using ER‐CS. Results Nine patients (9 to 33 years of age) were enrolled. Compared with the baseline (80.0 ± 6.33 mg/dL), the 12‐week evaluations revealed higher mean morning glucose levels (86.5 ± 8.26 mg/dL, p = 0.015). Twenty‐four weeks after the use of bedtime ER‐CS, alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels both decreased (from 69.3 ± 77.8 to 41.1 ± 40.4 U/L and from 78.8 ± 99.6 to 37.8 ± 28.81 U/L, respectively, p = 0.013 for both analyses), and sleep and fasting time both elongated (from 7.8 ± 0.87 to 8.6 ± 1.02 h and from 6.5 ± 1.22 to 7.6 ± 1.02 h, respectively, p = 0.011 for both analyses). The mean PSQI score in the five adult patients decreased significantly (from 5.8 ± 1.29 to 3.0 ± 1.71, p = 0.042). Conclusion This study provides evidence of clinically meaningful improvements by shifting only bedtime regular CS to ER‐CS in patients with GSDIa. As ER‐CS is considerably more expensive than regular CS, this approach presents a cost‐effective alternative. |
| first_indexed | 2024-03-11T18:42:18Z |
| format | Article |
| id | doaj.art-01c4fe3350914ee0a57502dc1abfcda5 |
| institution | Directory Open Access Journal |
| issn | 2324-9269 |
| language | English |
| last_indexed | 2024-03-11T18:42:18Z |
| publishDate | 2023-10-01 |
| publisher | Wiley |
| record_format | Article |
| series | Molecular Genetics & Genomic Medicine |
| spelling | doaj.art-01c4fe3350914ee0a57502dc1abfcda52023-10-12T08:38:42ZengWileyMolecular Genetics & Genomic Medicine2324-92692023-10-011110n/an/a10.1002/mgg3.2221Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type IaRai‐Hseng Hsu0Hui‐An Chen1Yin‐Hsiu Chien2Wuh‐Liang Hwu3Ju‐Li Lin4Hui‐Ling Weng5Yi‐Ting Lin6Yu‐Ching Lin7Ni‐Chung Lee8Department of Pediatrics National Taiwan University Hospital Taipei TaiwanDepartment of Pediatrics National Taiwan University Hospital Taipei TaiwanDepartment of Pediatrics National Taiwan University Hospital Taipei TaiwanDepartment of Pediatrics National Taiwan University Hospital Taipei TaiwanDivision of Genetics and Endocrinology, Department of Pediatrics Linkou Chang Gung Memorial Hospital Taoyuan TaiwanDepartment of Dietetics National Taiwan University Cancer Center Taipei TaiwanDepartment of Medical Genetics National Taiwan University Hospital Taipei TaiwanDepartment of Medical Genetics National Taiwan University Hospital Taipei TaiwanDepartment of Pediatrics National Taiwan University Hospital Taipei TaiwanAbstract Background Patients with glycogen storage disease type Ia (GSDIa) are prone to hypoglycemia. Uncooked cornstarch (CS) is the treatment, but maintaining nighttime blood glucose levels is still difficult. Methods The study enrolled patients with GSDIa to investigate the benefits of bedtime extended release CS (ER‐CS, Glycosade®) versus regular CS. The daytime CS schedule was not altered. A 7‐day continuous glucose monitoring (CGM) was performed at the baseline and 12 weeks after using ER‐CS. Biochemical profile, sleep quality (Pittsburgh Sleep Quality Index, PSQI), and quality of life (SF‐36 questionnaire) were measured at the baseline and 24 weeks after using ER‐CS. Results Nine patients (9 to 33 years of age) were enrolled. Compared with the baseline (80.0 ± 6.33 mg/dL), the 12‐week evaluations revealed higher mean morning glucose levels (86.5 ± 8.26 mg/dL, p = 0.015). Twenty‐four weeks after the use of bedtime ER‐CS, alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels both decreased (from 69.3 ± 77.8 to 41.1 ± 40.4 U/L and from 78.8 ± 99.6 to 37.8 ± 28.81 U/L, respectively, p = 0.013 for both analyses), and sleep and fasting time both elongated (from 7.8 ± 0.87 to 8.6 ± 1.02 h and from 6.5 ± 1.22 to 7.6 ± 1.02 h, respectively, p = 0.011 for both analyses). The mean PSQI score in the five adult patients decreased significantly (from 5.8 ± 1.29 to 3.0 ± 1.71, p = 0.042). Conclusion This study provides evidence of clinically meaningful improvements by shifting only bedtime regular CS to ER‐CS in patients with GSDIa. As ER‐CS is considerably more expensive than regular CS, this approach presents a cost‐effective alternative.https://doi.org/10.1002/mgg3.2221continuous glucose monitoring (CGM)extended release cornstarch (ER‐CS)glycogen storage disease type Ia (GSDIa)Pittsburgh sleep quality index (PSQI) |
| spellingShingle | Rai‐Hseng Hsu Hui‐An Chen Yin‐Hsiu Chien Wuh‐Liang Hwu Ju‐Li Lin Hui‐Ling Weng Yi‐Ting Lin Yu‐Ching Lin Ni‐Chung Lee Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia Molecular Genetics & Genomic Medicine continuous glucose monitoring (CGM) extended release cornstarch (ER‐CS) glycogen storage disease type Ia (GSDIa) Pittsburgh sleep quality index (PSQI) |
| title | Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia |
| title_full | Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia |
| title_fullStr | Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia |
| title_full_unstemmed | Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia |
| title_short | Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia |
| title_sort | bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type ia |
| topic | continuous glucose monitoring (CGM) extended release cornstarch (ER‐CS) glycogen storage disease type Ia (GSDIa) Pittsburgh sleep quality index (PSQI) |
| url | https://doi.org/10.1002/mgg3.2221 |
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