The Dietary Treatment of Children with Type I Glycogen Storage Disease with Slow Release Carbohydrate
The Dietary Treatment of Children with Type I Glycogen Storage Disease with Slow Release Carbohydrate
复制标题
缓释碳水化合物饮食治疗儿童I型糖原累积病
DOI：
10.1203/00006450-198409000-00015
复制
发表时间：
1984
影响因子：
3.6
通讯作者：
J. Fernandes
中科院分区：
文献类型：
--
作者：
G. Smit;R. Berger;R. Potasnick;S. Moses;J. Fernandes
摘要：研究了8例IA型糖原蓄积症（GSD）患者和1例IB型GSD患者分别摄入生淀粉（2 g/kg体重）、生淀粉（1 g/kg体重）和葡萄糖（2 g/kg体重）的影响。在每次耐量试验中每隔30分钟测定一次血糖浓度；每隔60分钟测定一次血乳酸、血胰岛素和呼气氢。在三个耐量试验期间，葡萄糖水平分别在6.5 ~ 9.0 h、3.5 ~ 6.5 h和2.25 ~ 4.0 h保持在正常范围（> ~ 1.8 mM）。在不同的测试中，每个患者的乳酸水平显著不同，在同一类型的测试中，患者之间的乳酸水平也显著不同。给淀粉后的血胰岛素浓度不超过空腹水平50 mU/l，明显低于给葡萄糖后的血胰岛素浓度（最高280 mU/l）。给予玉米淀粉（< 20 ppm）后，呼气氢排泄量没有增加或仅略有增加。
Summary: The effect of ingestion of uncooked cornstarch (2 g/kg body weight) in water, uncooked starch (1 g/kg) added to a meal, and glucose (2 g/kg) in water, was studied in eight patients with type IA glycogen storage disease (GSD) and one patient with type IB GSD. Blood glucose concentrations were determined at 30-min intervals during each tolerance test; blood lactate, blood insulin, and expiratory hydrogen were determined at 60-min intervals. The glucose levels remained in the normal range (> 1.8 mM) during approximately 6.5-9.0 h, 3.5-6.5 h, and 2.25-4.0 h during the three tolerance tests, respectively. The lactate levels differed markedly for the different tests per patient, and for the same type of test between the patients. Blood insulin concentrations after starch administration did not exceed values of 50 mU/liter above fasting levels and were markedly lower than those after glucose administration (maximum levels of 280 mU/liter). The expiratory hydrogen excretion did not increase or only slightly increased after cornstarch administration (< 20 ppm).