Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I
Ориентир для пациента и семьи
Простыми словами
Автоматическая сводка по структурированным данным реестра. Она помогает сориентироваться, но не заменяет официальный протокол или оценку врача.
- Что изучают
- В протоколе указаны: Fructose, Fructose and galactose.
- Кому может быть актуально
- Состояния в реестре: Glycogen Storage Disease Type I. Базовые параметры: от 18 лет · Все.
- Что важно проверить
- Возраст, диагноз и пол — только базовые ориентиры. Предыдущее лечение, анализы и другие обязательные условия указаны ниже в критериях участия.
- Где проводится
- Швейцария
- Следующий шаг
- Сохраните исследование, покажите его лечащему врачу и уточните актуальный статус у исследовательского центра. Расходы, документы и поездка →
Не всё понятно в терминах? Прочитайте наш гид для пациентов →
Официальное название
Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I (GSD-DIET)
Обзор
The present project will specifically assess metabolic effects of dietary interventions with controlled intake of fructose and fructose/galactose in GSDI, with the aim to provide evidence whether relaxed dietary restrictions of fructose and galactose may be justified in treatment recommendations at least for adults, which would considerably enlarge food choice in everyday life of the patients with an expected positive impact on the quality of life of patients with this rare disorder.
Подробное описание
To assess relaxed restriction of fructose and fructose/galactose intake on secondary metabolic alterations in GSDI, (i) by looking at the traditional parameters for assessing metabolic control in clinical chemistry (lactate, triglycerides, uric acid), and (ii) by using a broad analytical approach relying on targeted metabolomics/lipidomics.
It is hypothesized that relaxed restrictions on the intake of fructose and/or galactose as part of the diet in everyday life may lead to an increase in blood lactate levels (=primary outcome), triglycerides, and uric acid to a certain degree compared to baseline. However, this increase is expected to remain within a range that is not clinically relevant for adult patients, especially when fructose/galactose intake is not excessive and stays within the usual daily allowances for healthy individuals, as planned in this study.
Вмешательства
- Пищевая добавка Fructose
40g fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g) - Пищевая добавка Fructose and galactose
10g galactose (mostly from lactose) plus 40g of fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g)
Первичные конечные точки
- Change in lactate during the dietary intervention compared to baseline under the usual diet [Срок оценки: 4 Weeks]
Вторичные конечные точки (4)
- Change in plasma triglycerides during the dietary intervention compared to baseline (as parameters traditionally measured together with lactate to estimate overall metabolic control) [Срок оценки: 4 Weeks]
- Change in plasma uric acid during the dietary intervention compared to baseline (as parameters traditionally measured together with lactate to estimate overall metabolic control) [Срок оценки: 4 Weeks]
- Plasma metabolite changes in targeted metabolomics during the dietary intervention compared to baseline measurements. [Срок оценки: 4 Weeks]
- Plasma metabolite changes in targeted lipidomics during the dietary intervention compared to baseline measurements. [Срок оценки: 4 Weeks]
Критерии участия
Критерии включения
- Genetically and/or enzymatically confirmed diagnosis of GSDI (GSDIa or GSDIb)
- Male or female ≥ 18y
- Restriction of fructose intake in usual dietary treatment
- Written informed consent
Критерии исключения
- Non-compliance with routine dietary treatment
- Pregnancy or lactation
- Liver transplant
- Recurrent hospitalisations due to metabolic decompensation within the last 12 months
- Severe chronic kidney disease with glomerular filtration rate (GFR) < 30 ml/min
- For GSDIb: Severe, uncontrolled symptomatic inflammatory bowel disease
Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.
Здоровые добровольцы: Нет
Дизайн исследования
- Распределение
- Рандомизированное
- Модель
- Перекрёстный дизайн
- Маскирование
- Открытое
- Основная цель
- Лечение
Центры проведения
Швейцария · 2 центра
- Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, B — Bern
- Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zuric — Zurich
Идентификаторы
NCT: NCT06852612 · 2024-02154