Body composition in patients with hepatic glycogen storage diseases

Bruna B Dos Santos,Karina Colonetti,Tatiéle Nalin,Bibiana M de Oliveira,Carolina F M de Souza,Poli Mara Spritzer,Ida V D Schwartz

Nutrition（2022）

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摘要

•Elevated fat mass was frequent in a sample of Brazilian patients with hepatic glycogen storage disorders (GSDs)•Uncooked cornstarch therapy was associated with excess weight in hepatic GSDs•Uncooked cornstarch therapy can entail a decrease in lean mass in hepatic GSDs•A strict nutritional evaluation is necessary for patients with hepatic GSDs•Further studies are needed to evaluate the use of dual energy x-ray absorptiometry in patients with hepatic GSDs

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关键词

Inborn errors of metabolism,Hepatic glycogen storage disease,Body composition,absorptiometry,Dual x-ray,Uncooked cornstarch,treatment

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