دورية أكاديمية
أعرض في EDS

Long-Term Dietary Changes in Subjects with Glucose Galactose Malabsorption Secondary to Biallelic Mutations of SLC5A1.

التفاصيل البيبلوغرافية
العنوان: Long-Term Dietary Changes in Subjects with Glucose Galactose Malabsorption Secondary to Biallelic Mutations of SLC5A1.
المؤلفون: Chan AP; Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, 90095, USA. alvinchan@mednet.ucla.edu., Namjoshi SS; Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Lucile Packard Children's Hospital and Stanford University School of Medicine, Stanford University, Palo Alto, CA, 94304, USA., Jardack PM; Clinical Translational Research Center, Clinical and Translational Science Institute, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, 90095, USA., Maloney L; Food Science Solutions, San Diego, CA, 92101, USA., Ardjmand A; Department of Medicine Statistics Core, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, 90095, USA., Jackson NN; Department of Medicine Statistics Core, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, 90095, USA., Martin MG; Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, 90095, USA.
المصدر: Digestive diseases and sciences [Dig Dis Sci] 2021 Dec; Vol. 66 (12), pp. 4414-4422. Date of Electronic Publication: 2021 Jan 12.
نوع المنشور: Case Reports; Journal Article; Observational Study; Research Support, N.I.H., Extramural
اللغة: English
بيانات الدورية: Publisher: Springer Science + Business Media Country of Publication: United States NLM ID: 7902782 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1573-2568 (Electronic) Linking ISSN: 01632116 NLM ISO Abbreviation: Dig Dis Sci Subsets: MEDLINE
أسماء مطبوعة: Publication: 2005- : New York, NY : Springer Science + Business Media
Original Publication: New York, Plenum Pub. Corp.
مواضيع طبية MeSH: Carbohydrate Metabolism, Inborn Errors/*psychology , Diet/*statistics & numerical data , Malabsorption Syndromes/*psychology, Adult ; Carbohydrate Metabolism, Inborn Errors/genetics ; Child ; Child, Preschool ; Cohort Studies ; Cross-Sectional Studies ; Female ; Humans ; Infant ; Malabsorption Syndromes/genetics ; Male ; Sodium-Glucose Transporter 1/genetics
مستخلص: Background: Glucose galactose malabsorption (GGM) is a congenital diarrheal disorder of intestinal Na+/glucose cotransport (SGLT1/SLC5A1). The required glucose and galactose-restricted diet has been well described in infancy, but long-term nutrition follow-up is limited.
Aim: To perform a comprehensive nutritional assessment on a cohort of patients with GGM to gain insights into the consumption patterns within the population.
Methods: A cross-sectional study examining dietary intake of a GGM cohort using prospective food records. The calories and nutrients of all foods, beverages, and condiments were analyzed with descriptive statistics and compared to intake patterns of age- and sex-matched NHANES groups.
Results: The six patients were 0.7-26 years old. Whole foods and vegetable fats were major parts of the diet, while dairy and added sweeteners were restricted. Compared to typical US intakes, mean macronutrient distribution was 88th percentile from fat, 18th percentile from carbohydrates, and 78th percentile from protein. Fructose consumption, as a proportion of total sugar intake, decreased with age, from 86.1 to 50.4%. Meanwhile, glucose consumption increased with age, from 13.8 to 48.6% of sugar intake. However, the actual amount of glucose consumed remained low, equivalent to 4th percentile of US consumption level. Galactose intake was marginal throughout life.
Conclusions: A GGM diet is a high-fat and high-protein/low-carbohydrate diet that is rich in fruits and vegetables but limited in dairy and added sugar. Relatively less fructose but more glucose is incorporated into the diet with age. Future studies should investigate the effects of the GGM diet on gut microbiome and long-term health.
(© 2021. The Author(s).)
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معلومات مُعتمدة: R21 DK075009 United States DK NIDDK NIH HHS; RC2 DK118640 United States DK NIDDK NIH HHS; UL1 TR001881 United States TR NCATS NIH HHS; R01 DK083762 United States DK NIDDK NIH HHS; T32 DK007180 United States DK NIDDK NIH HHS
فهرسة مساهمة: Keywords: Diet; Glucose galactose malabsorption; High fat; Low carbohydrate; Nutrition; SLC5A1; Sodium/glucose cotransporter
المشرفين على المادة: 0 (SLC5A1 protein, human)
0 (Sodium-Glucose Transporter 1)
SCR Disease Name: Glucose-Galactose Malabsorption
تواريخ الأحداث: Date Created: 20210112 Date Completed: 20211209 Latest Revision: 20220218
رمز التحديث: 20221213
مُعرف محوري في PubMed: PMC8273183
DOI: 10.1007/s10620-020-06792-4
PMID: 33433815
قاعدة البيانات: MEDLINE
الوصف
تدمد:1573-2568
DOI:10.1007/s10620-020-06792-4