Effect of Uncooked Cornstarch Treatment on Metabolic Control in Patients With Hepatic Glycogen Storage Diseases

Büşra Akyol Yılmaz; Elvan Yılmaz Akyüz; Tanyel Zübarioğlu

Makale detayları

Effect of Uncooked Cornstarch Treatment on Metabolic Control in Patients With Hepatic Glycogen Storage Diseases

Yazarlar:

Büşra Akyol Yılmaz | Elvan Yılmaz Akyüz | Tanyel Zübarioğlu

Yayıncı:

Dokuz Eylul University

Ana Başlıklar:

Clinical Sciences (Other)

Dergi:

Journal of Basic and Clinical Health Sciences Vol.9 Issue 3, pp.494-503

Belge Tipi:

Makale

Yayın Tarihi:

9/2025

Belge Dili:

İngilizce

Document ID (Org No.):

2001359216

pISSN:

2564-7288

Anahtar kelimeler:

Özet:

 Background and Purpose: Glycogen storage diseases GSD are a group of rare inherited metabolic disorders. Hypoglycemia, the most severe clinical manifestation of hepatic GSD, can be averted particularly with the supplementation of uncooked cornstarch. This study aims to elucidate the nutritional condition of individuals with hepatic GSD and to examine the impact of dietary therapy using uncooked cornstarch on metabolic regulation. Methods: This study was a single-center cross-sectional prospective design and included 8 follow-up patients with hepatic GSD patients aged 0-18 years. Demographic characteristics, anthropometric measurements, biochemical markers, energy expenditure, and macronutrient intake of patients were assessed over a period of six months. Results: The mean age of the patients was 8.0  5.9 years. 25 of the patients were female, while 75 were male. A significant difference was observed in plasma fasting glucose and alanine aminotransferase levels among patients with uncooked cornstarch intake p0.024; p0.044, respectively and extended overnight fasting durations. The patients weight, height, and abdominal circumference exhibited significant changes during the 6-month follow-up period p0.038; p0.001; p0.008, respectively. Conclusions: An uncooked cornstarch-based diet in individuals with hepatic glycogen storage diseases effectively maintains normoglycemia and mitigates possible metabolic consequences. Its appropriate use facilitates adequate growth and development in patients and plays a vital function in metabolic regulation.

 Background and Purpose: Glycogen storage diseases GSD are a group of rare inherited metabolic disorders. Hypoglycemia, the most severe clinical manifestation of hepatic GSD, can be averted particularly with the supplementation of uncooked cornstarch. This study aims to elucidate the nutritional condition of individuals with hepatic GSD and to examine the impact of dietary therapy using uncooked cornstarch on metabolic regulation. Methods: This study was a single-center cross-sectional prospective design and included 8 follow-up patients with hepatic GSD patients aged 0-18 years. Demographic characteristics, anthropometric measurements, biochemical markers, energy expenditure, and macronutrient intake of patients were assessed over a period of six months. Results: The mean age of the patients was 8.0  5.9 years. 25 of the patients were female, while 75 were male. A significant difference was observed in plasma fasting glucose and alanine aminotransferase levels among patients with uncooked cornstarch intake p0.024; p0.044, respectively and extended overnight fasting durations. The patients weight, height, and abdominal circumference exhibited significant changes during the 6-month follow-up period p0.038; p0.001; p0.008, respectively. Conclusions: An uncooked cornstarch-based diet in individuals with hepatic glycogen storage diseases effectively maintains normoglycemia and mitigates possible metabolic consequences. Its appropriate use facilitates adequate growth and development in patients and plays a vital function in metabolic regulation.

 Background and Purpose: Glycogen storage diseases GSD are a group of rare inherited metabolic disorders. Hypoglycemia, the most severe clinical manifestation of hepatic GSD, can be averted particularly with the supplementation of uncooked cornstarch. This study aims to elucidate the nutritional condition of individuals with hepatic GSD and to examine the impact of dietary therapy using uncooked cornstarch on metabolic regulation. Methods: This study was a single-center cross-sectional prospective design and included 8 follow-up patients with hepatic GSD patients aged 0-18 years. Demographic characteristics, anthropometric measurements, biochemical markers, energy expenditure, and macronutrient intake of patients were assessed over a period of six months. Results: The mean age of the patients was 8.0  5.9 years. 25 of the patients were female, while 75 were male. A significant difference was observed in plasma fasting glucose and alanine aminotransferase levels among patients with uncooked cornstarch intake p0.024; p0.044, respectively and extended overnight fasting durations. The patients weight, height, and abdominal circumference exhibited significant changes during the 6-month follow-up period p0.038; p0.001; p0.008, respectively. Conclusions: An uncooked cornstarch-based diet in individuals with hepatic glycogen storage diseases effectively maintains normoglycemia and mitigates possible metabolic consequences. Its appropriate use facilitates adequate growth and development in patients and plays a vital function in metabolic regulation.

 Background and Purpose: Glycogen storage diseases GSD are a group of rare inherited metabolic disorders. Hypoglycemia, the most severe clinical manifestation of hepatic GSD, can be averted particularly with the supplementation of uncooked cornstarch. This study aims to elucidate the nutritional condition of individuals with hepatic GSD and to examine the impact of dietary therapy using uncooked cornstarch on metabolic regulation. Methods: This study was a single-center cross-sectional prospective design and included 8 follow-up patients with hepatic GSD patients aged 0-18 years. Demographic characteristics, anthropometric measurements, biochemical markers, energy expenditure, and macronutrient intake of patients were assessed over a period of six months. Results: The mean age of the patients was 8.0  5.9 years. 25 of the patients were female, while 75 were male. A significant difference was observed in plasma fasting glucose and alanine aminotransferase levels among patients with uncooked cornstarch intake p0.024; p0.044, respectively and extended overnight fasting durations. The patients weight, height, and abdominal circumference exhibited significant changes during the 6-month follow-up period p0.038; p0.001; p0.008, respectively. Conclusions: An uncooked cornstarch-based diet in individuals with hepatic glycogen storage diseases effectively maintains normoglycemia and mitigates possible metabolic consequences. Its appropriate use facilitates adequate growth and development in patients and plays a vital function in metabolic regulation.

Makale detayları

Effect of Uncooked Cornstarch Treatment on Metabolic Control in Patients With Hepatic Glycogen Storage Diseases

Yazarlar:

Büşra Akyol Yılmaz | Elvan Yılmaz Akyüz | Tanyel Zübarioğlu

Yayıncı:

Dokuz Eylul University

Ana Başlıklar:

Clinical Sciences (Other)

Dergi:

Journal of Basic and Clinical Health Sciences Vol.9 Issue 3, pp.494-503

Belge Tipi:

Makale

Yayın Tarihi:

9/2025

Belge Dili:

İngilizce

Document ID (Org No.):

2001359216

pISSN:

2564-7288

Diet

Glycogen storage diseases

Metabolic control

Nutritional status

Uncooked cornstarch

Sayı

Makale detayları

Effect of Uncooked Cornstarch Treatment on Metabolic Control in Patients With Hepatic Glycogen Storage Diseases

Yazarlar: Büşra Akyol Yılmaz | Elvan Yılmaz Akyüz | Tanyel Zübarioğlu

Yayıncı: Dokuz Eylul University

Ana Başlıklar: Clinical Sciences (Other)

Dergi: Journal of Basic and Clinical Health Sciences Vol.9 Issue 3, pp.494-503

Belge Tipi: Makale

Yayın Tarihi: 9/2025

Belge Dili: İngilizce

Document ID (Org No.): 2001359216

pISSN: 2564-7288

Diet

Glycogen storage diseases

Metabolic control

Nutritional status

Uncooked cornstarch

Sayı

Yazarlar:

Büşra Akyol Yılmaz | Elvan Yılmaz Akyüz | Tanyel Zübarioğlu

Yayıncı:

Dokuz Eylul University

Ana Başlıklar:

Clinical Sciences (Other)

Dergi:

Journal of Basic and Clinical Health Sciences Vol.9 Issue 3, pp.494-503

Belge Tipi:

Makale

Yayın Tarihi:

9/2025

Belge Dili:

İngilizce

Document ID (Org No.):

2001359216

pISSN:

2564-7288