Unit of Clinical Nutrition, Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Unit of Clinical Nutrition, Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Obes Res Clin Pract. 2009 Mar;3(1):1-52. doi: 10.1016/j.orcp.2008.11.001.
To study the metabolic parameters which may affect the excessive weight of treated and untreated patients with Laron Syndrome.
Body composition, daily caloric intake and resting energy expenditure (REE), when possible, were measured for each patient. Caloric intake was calculated based on 7-day food records, REE was measured by indirect calorimetry and body composition was determined by dual energy X-ray absorptiometry (DEXA).
Nine untreated adult subjects with Laron Syndrome (6 female subjects, 3 male subjects) aged 28-53 years and 4 girls with Laron Syndrome treated by insulin-like growth factor-I (IGF-I) 120-150 μg/kg/d were included in the study.
Patients with Laron Syndrome have an abnormally high body fat (BF) mass (54 ± 10% of body weight) and a relatively low lean body mass (LBM) compared to a healthy normal population. Energy intake varied but in most of the patients was not significantly higher than the measured REE. The REE corrected for LBM was higher than expected, based on our norms for healthy adults. The mean distribution of energy sources in the food was 47% carbohydrates, 17% protein and 36% fat.
The severe obesity of patients with Laron Syndrome is not due to hyperphagia or hypometabolism.
研究可能影响治疗和未治疗的拉隆综合征患者体重过重的代谢参数。
对每位患者均测量身体成分、每日热量摄入和静息能量消耗（REE），在可能的情况下进行测量。热量摄入根据 7 天的食物记录进行计算，REE 通过间接量热法测量，身体成分通过双能 X 射线吸收法（DEXA）确定。
9 名未治疗的成年拉隆综合征患者（6 名女性，3 名男性），年龄 28-53 岁，4 名接受胰岛素样生长因子-I（IGF-I）治疗的拉隆综合征女孩，剂量为 120-150μg/kg/d。
拉隆综合征患者的体脂（BF）质量异常高（占体重的 54±10%），与健康正常人群相比，瘦体重（LBM）相对较低。能量摄入存在差异，但在大多数患者中，并未明显高于测量的 REE。校正 LBM 后的 REE 高于我们健康成年人正常值的预期。食物中能量来源的平均分布为 47%碳水化合物、17%蛋白质和 36%脂肪。
拉隆综合征患者的严重肥胖并非由于多食或代谢低下所致。
