医学
内科学
体质指数
双重能量
内分泌学
瘦体质量
动物科学
作文(语言)
体重
生物
骨矿物
骨质疏松症
语言学
哲学
作者
Bruna Bento dos Santos,Karina Colonetti,Tatiéle Nalin,Bibiana Mello de Oliveira,Carolina Fischinger Moura de Souza,Poli Mara Spritzer,Ida Vanessa Döederlein Schwartz
出处
期刊:Nutrition
[Elsevier]
日期:2022-11-01
卷期号:103-104: 111763-111763
被引量:1
标识
DOI:10.1016/j.nut.2022.111763
摘要
The present study aimed to evaluate the body composition of hepatic glycogen storage disorders (GSDs) through dual energy x-ray absorptiometry.This was an exploratory, observational, cross-sectional study. Twenty-four patients with GSD (type Ia: n = 13, Ib: n = 5, III: n = 2, and IX-α/β/γ: n = 4; female sex: n = 13; age <8 y: n = 3, 8-19 y: n = 14, and >19 y: n = 7) were included. Three-day dietary records were collected in the week preceding dual energy x-ray absorptiometry. Body composition findings were correlated with clinical parameters, uncooked cornstarch (UCCS) regimen, dietary intake, and markers of treatment adherence.An elevated fat mass (FM) index was found in 16 of 21 patients (age 8-19 y: n = 10 and >19 y: n = 6; GSD type Ia: n = 12, Ib: n = 2, III: n = 1, and IX-γ: n = 1). A lean mass (LM) index evaluation showed no LM deficits in relation to corresponding reference populations. Relative skeletal muscle index values were decreased in 2 of 7 adult patients (type Ib: n = 1 and IX-α: n = 1). UCCS (g/d) correlated positively with the FM index (rs = 0.7; P ≤ 0.01). In contrast, relative UCCS intake (g/kg body weight) was negatively associated with LM/kg (rs = -0.8; P ≤ 0.01).These findings suggest a high frequency of elevated FM in patients with hepatic GSDs. We also suggest that treatment with UCCS is associated with excess weight in these patients. Additionally, the treatment strategy can impair protein intake, and lead to a decrease in LM.
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