Glucose-galactose malabsorption: pathogenesis, diagnosis, diet therapy

Q3 Medicine
E. Roslavtseva, M. Varichkina, T. Bushueva, К.А. Kazakova, V. Skvortsova, T. Borovik, A. Fisenko
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引用次数: 0

Abstract

Congenital glucose-galactose malabsorption is an orphan autosomal recessive life-threatening disease resulting from a selective defect in the intestinal glucose and galactose/sodium co-transport system in the brush border membrane of the enterocyte. In glucose-galactose malabsorption, glucose, galactose, as well as di- and polysaccharides: starch, dextrins, maltose, sucrose, lactose cannot be absorbed. The disease manifests itself in the neonatal period in the form of severe watery osmotic diarrhea, leading to rapid dehydration and metabolic acidosis. The only treatment is diet therapy with the exclusion of all carbohydrates except fructose. In infants with glucose-galactose malabsorption, diet therapy using specialized formulas with fructose as a carbohydrate component is vital. Subsequently, such infants need the introduction of low-carbohydrate complementary foods with primarily fructose content. The article presents a clinical example of the disease course and its successful dietary correction in an infant using a specialized formula and a specific diet. Key words: congenital diarrheal disorders, glucose-galactose malabsorption, infants, diet therapy, specialized milk formula based on fructose
葡萄糖-半乳糖吸收不良:发病机制、诊断、饮食治疗
先天性葡萄糖-半乳糖吸收不良是一种罕见的常染色体隐性遗传病,由肠细胞刷状边缘膜中肠道葡萄糖和半乳糖/钠共转运系统的选择性缺陷引起。在葡萄糖-半乳糖吸收不良时,葡萄糖、半乳糖以及二糖和多糖:淀粉、糊精、麦芽糖、蔗糖、乳糖不能被吸收。该病在新生儿期表现为严重的水样渗透性腹泻,导致迅速脱水和代谢性酸中毒。唯一的治疗方法是饮食疗法,除果糖外,不含所有碳水化合物。对于患有葡萄糖-半乳糖吸收不良的婴儿,使用含有果糖作为碳水化合物成分的专门配方的饮食疗法至关重要。随后,这些婴儿需要引入主要含果糖的低碳水化合物辅食。本文介绍了一个临床例子,疾病的过程和其成功的饮食纠正在一个婴儿使用专门的配方和特定的饮食。关键词:先天性腹泻,葡萄糖-半乳糖吸收不良,婴儿,食疗,果糖配方奶粉
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来源期刊
Voprosy Detskoi Dietologii
Voprosy Detskoi Dietologii Medicine-Pediatrics, Perinatology and Child Health
CiteScore
1.20
自引率
0.00%
发文量
17
期刊介绍: The scientific journal Voprosy Detskoi Dietologii is included in the Scopus database. Publisher country is RU. The main subject areas of published articles are Food Science, Pediatrics, Perinatology, and Child Health, Nutrition and Dietetics, Клиническая медицина.
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