巴德-比德尔综合征患者的食欲亢进。
Hyperphagia among patients with Bardet-Biedl syndrome.
作者信息
Sherafat-Kazemzadeh R, Ivey L, Kahn S R, Sapp J C, Hicks M D, Kim R C, Krause A J, Shomaker L B, Biesecker L G, Han J C, Yanovski J A
机构信息
Section on Growth and Obesity (SGO), Program in Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), DHHS, Bethesda, MD, USA.
出版信息
Pediatr Obes. 2013 Oct;8(5):e64-7. doi: 10.1111/j.2047-6310.2013.00182.x. Epub 2013 Jun 18.
BACKGROUND
The importance of hyperphagia as a cause for energy imbalance in humans with Bardet-Biedl syndrome (BBS) has not been established. We therefore compared hyperphagic symptoms in patients with BBS vs. controls.
METHODS
We studied 13 patients with BBS and 23 non-syndromic controls with similar age, sex and body mass index (BMI) z-score. A 13-item hyperphagia questionnaire was completed by patients' parents/guardians.
RESULTS
Total hyperphagia questionnaire score was higher in BBS than controls (27.6 ± 9.0 vs. 19.1 ± 7.9, P = 0.005). Behaviour and drive subscales were higher for BBS than controls (12.5 ± 4.1 vs. 7.8 ± 3.2, P = 0.001, and 11.2 ± 4.1 vs. 8.3 ± 3.8, P = 0.04, respectively); severity was not significantly different between groups (3.8 ± 1.5 vs. 3.0 ± 1.3, P = 0.072). After adjustment for demographic variables and BMI z-score, total and behaviour subscale scores remained significantly different between groups, suggesting food-seeking activity, rather than preoccupation with food may be the main hyperphagic feature among patients with BBS.
CONCLUSION
Appetite dysregulation may contribute to obesity in BBS.
背景
食欲亢进作为巴德-比德尔综合征（BBS）患者能量失衡原因的重要性尚未明确。因此，我们比较了BBS患者与对照组的食欲亢进症状。
方法
我们研究了13例BBS患者和23例年龄、性别及体重指数（BMI）z评分相似的非综合征对照组。患者的父母/监护人完成了一份包含13个条目的食欲亢进问卷。
结果
BBS患者的食欲亢进问卷总分高于对照组（27.6±9.0对19.1±7.9，P = 0.005）。BBS患者的行为和驱力子量表得分高于对照组（分别为12.5±4.1对7.8±3.2，P = 0.001；11.2±4.1对8.3±3.8，P = 0.04）；两组间严重程度无显著差异（3.8±1.5对3.0±1.3，P = 0.072）。在对人口统计学变量和BMI z评分进行调整后，两组间的总分和行为子量表得分仍存在显著差异，这表明觅食活动而非对食物的过度关注可能是BBS患者食欲亢进的主要特征。
结论
食欲调节异常可能导致BBS患者肥胖。
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