背景:患有假性甲状旁腺功能减退症1a型(PHP-1a)的儿童发展为早发性肥胖。这些儿童的静息能量消耗减少,但尚不清楚饮食过多是否会导致他们的肥胖。
方法:我们使用饮食亢进问卷(HQ)和儿童饮食行为问卷(CEBQ)对2至12岁的PHP-1a患者和匹配的对照进行了调查评估。还将PHP-1a组的结果与肥胖对照组和正常体重同胞组进行了比较。
结果:我们招募了10名PHP-1a患者和9名匹配的对照。PHP-1a组和匹配对照组的总HQ评分没有显着差异(p=0.72),行为(p=0.91),驱动(p=0.48)或严重性(p=0.73)子集得分。PHP-1a组和CEBQ上的匹配对照之间也没有差异。在次要分析中,将PHP-1a组与肥胖对照组(n=30)和正常体重兄弟姐妹(n=6)进行比较.看护者报告说,10名PHP-1a患者中有6名(60%)在2岁之前对食物的兴趣增加,30名肥胖对照中有9名(30%),无兄弟姐妹(p=0.04)。兄弟姐妹组的积极饮食行为得分明显低于PHP-1a组(2.6[2.4,2.9]与3.5[3.1,4.0],p<0.01)和肥胖对照组(2.6[2.4,2.9]vs.3.4[2.6、3.8]、p=0.04),但PHP-1a和肥胖对照组之间没有显着差异(p=0.35)。兄弟姐妹组的饮酒欲望得分低于PHP-1a组(1.8[1.6,2.7]与4.3[3.3,5.0],p<0.01)和肥胖对照组(1.8[1.6,2.7]vs.3.3[3.0,4.0],p<0.01),但PHP-1a和肥胖对照的饮酒欲望评分之间没有显着差异(p=0.11)。
结论:PHP-1a患者表现出与匹配的肥胖对照组相似的过度症状。
BACKGROUND: Children with
pseudohypoparathyroidism type 1a (PHP-1a) develop early-onset obesity. These children have decreased resting energy expenditure but it is unknown if hyperphagia contributes to their obesity.
METHODS: We conducted a survey assessment of patients 2 to 12 years old with PHP-1a and matched controls using the Hyperphagia Questionnaire (HQ) and Children\'s Eating Behavior Questionnaire (CEBQ). Results of the PHP-1a group were also compared with an obese control group and normal weight sibling group.
RESULTS: We enrolled 10 patients with PHP-1a and 9 matched controls. There was not a significant difference between the PHP-1a group and matched controls for total HQ score (p = 0.72), Behavior (p = 0.91), Drive (p = 0.48) or Severity (p = 0.73) subset scores. There was also no difference between the PHP-1a group and matched controls on the CEBQ. In a secondary analysis, the PHP-1a group was compared with obese controls (n = 30) and normal weight siblings (n = 6). Caregivers reported an increased interest in food before age 2 years in 6 of 10 PHP-1a patients (60%), 9 of 30 obese controls (30%) and none of the siblings (p = 0.04). The sibling group had a significantly lower Positive Eating Behavior score than the PHP-1a group (2.6 [2.4, 2.9] vs. 3.5 [3.1, 4.0], p < 0.01) and obese controls (2.6 [2.4, 2.9] vs. 3.4 [2.6, 3.8], p = 0.04), but there was not a significant difference between the PHP-1a and obese controls (p = 0.35). The sibling group had a lower Desire to Drink score than both the PHP-1a group (1.8 [1.6, 2.7] vs. 4.3 [3.3, 5.0], p < 0.01) and obese controls (1.8 [1.6, 2.7] vs. 3.3 [3.0, 4.0], p < 0.01) but there was not a significant difference between the PHP-1a and obese control Desire to Drink scores (p = 0.11).
CONCLUSIONS: Patients with PHP-1a demonstrate hyperphagic symptoms similar to matched obese controls.