Department of Neurological Sciences, University of Bologna - IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
Sleep. 2013 Feb 1;36(2):175-81. doi: 10.5665/sleep.2366.
We analyzed the potential predictive factors for precocious puberty, observed in some cases of childhood narcolepsy with cataplexy (NC) and for obesity, a much more common feature of NC, through a systematic assessment of pubertal staging, body mass index (BMI), and metabolic/endocrine biochemical analyses.
Cross-sectional on consecutive recruitment.
Hospital sleep center and pediatric unit.
Forty-three children and adolescents with NC versus 52 age-matched obese children as controls.
N/A.
Patients underwent clinical interview, polysomnographic recordings, cerebrospinal fluid hypocretin-1 measurement, and human leukocyte antigen typing. Height, weight, arterial blood pressure, and Tanner pubertal stage were evaluated. Plasma lipid and glucose profiles were analyzed. When an altered pubertal development was clinically suspected, plasma concentrations of hypothalamic-pituitary-gonadal axis hormones were determined. Children with NC showed a high prevalence of overweight/obesity (74%) and a higher occurrence of precocious puberty (17%) than obese controls (1.9%). Isolated signs of accelerated pubertal development (thelarche, pubic hair, advanced bone age) were also present (41%). Precocious puberty was significantly predicted by a younger age at first NC symptom onset but not by overweight/obesity or other factors. In addition, overweight/obesity was predicted by younger age at diagnosis; additional predictors were found for overweight/obesity (short disease duration, younger age at weight gain and lower high-density lipoprotein cholesterol), which did not include precocious puberty. NC symptoms, pubertal signs appearance, and body weight gain developed in close temporal sequence.
NC occurring during prepubertal age is frequently accompanied by precocious puberty and overweight/obesity, suggesting an extended hypothalamic dysfunction. The severity of these comorbidities and the potential related risks require a multidiagnostic approach and a tailored therapeutic management.
通过对青春期分期、体重指数（BMI）和代谢/内分泌生化分析的系统评估，我们分析了一些儿童发作性睡病伴猝倒（NC）病例中早熟和肥胖的潜在预测因素，肥胖是 NC 更为常见的特征。
连续招募的横断面研究。
医院睡眠中心和儿科病房。
43 名 NC 患儿和青少年患者与 52 名年龄匹配的肥胖儿童对照组。
无。
患者接受临床访谈、多导睡眠图记录、脑脊液下丘脑泌素-1 测量和人类白细胞抗原分型。评估身高、体重、动脉血压和 Tanner 青春期分期。分析血浆脂质和葡萄糖谱。当临床怀疑青春期发育改变时，测定下丘脑-垂体-性腺轴激素的血浆浓度。NC 患儿超重/肥胖（74%）和性早熟（17%）的发生率高于肥胖对照组（1.9%）。孤立的加速性青春期发育（乳房早发育、阴毛、骨龄提前）也存在（41%）。性早熟显著预测首发 NC 症状的年龄较小，但与超重/肥胖或其他因素无关。此外，超重/肥胖预测年龄较小，肥胖/超重的其他预测因素是诊断年龄较小、体重增加年龄较小和高密度脂蛋白胆固醇水平较低，不包括性早熟。NC 症状、青春期体征出现和体重增加呈紧密的时间顺序。
发生在青春期前的 NC 常伴有性早熟和超重/肥胖，提示下丘脑功能障碍广泛。这些合并症的严重程度和潜在的相关风险需要多诊断方法和针对性的治疗管理。
