Yang Hongbo, Zhang Mengqi, Song Hongmei, Zhu Huijuan, Pan Hui
Key Laboratory of Endocrinology of the Ministry of Health, Department of Endocrinology, Peking Union Medical College Hospital, Beijing, China.
Department of Pediatrics, Peking Union Medical College Hospital, Beijing, China.
Congenit Anom (Kyoto). 2015 Nov;55(4):173-7. doi: 10.1111/cga.12111.
The aim of the present study was to investigate the spontaneous development of growth and weight gain of patients with Prader-Willi syndrome (PWS) in Mainland China. We retrospectively analyzed 120 cases of PWS diagnosed from 1994 to 2014 in Mainland China. Scatter diagram of the growth data was compared to standardized growth curve. The length at birth was similar to the normal population and the mean birthweight is under the 50th centile of normal population. Heights in 43% (27/62) of patients had dropped off below the 3(rd) centile of their peers after 5 years of age. Weights in 65.9% (58/88) of patients had exceeded the 97(th) centile of their peers after 3 years of age. Early obesity is obvious in 92.9% (66/71) of patients with body mass index (BMI) up the 97(th) centile of their peers from 2-3 years of age. Final mean height is 149.3 ± 11.2 cm for females (n = 7) and 146.2 ± 9.8 cm for males (n = 6), showing 11.3 cm and 26.6 cm below the average height for normal Chinese girls and boys. The growth pattern of PWS in Mainland China is comparable to those in Caucasians. Early intervention with recombinant human growth hormone is warranted considering the early onset of growth retardation and obesity.
本研究旨在调查中国大陆普拉德-威利综合征（PWS）患者生长发育和体重增加的自然发展情况。我们回顾性分析了1994年至2014年在中国大陆诊断的120例PWS患者。将生长数据的散点图与标准化生长曲线进行比较。出生时的身长与正常人群相似，平均出生体重低于正常人群的第50百分位数。43%（27/62）的患者在5岁后身高降至同龄人第3百分位数以下。65.9%（58/88）的患者在3岁后体重超过同龄人第97百分位数。92.9%（66/71）的患者在2至3岁时体重指数（BMI）超过同龄人第97百分位数，早期肥胖明显。女性（n = 7）的最终平均身高为149.3±11.2厘米，男性（n = 6）为146.2±9.8厘米，分别比中国正常女孩和男孩的平均身高低11.3厘米和26.6厘米。中国大陆PWS的生长模式与白种人相似。考虑到生长迟缓与肥胖的早期发生，有必要对重组人生长激素进行早期干预。
