Wollmann H A, Schultz U, Grauer M L, Ranke M B
University Children's Hospital, Growth Research Center, Tübingen, Germany.
Eur J Pediatr. 1998 Aug;157(8):634-42. doi: 10.1007/s004310050901.
The spontaneous growth of 315 patients (109 girls and 208 boys) with Prader-Willi syndrome (PWS) was analysed in a mixed longitudinal and cross-sectional manner. 33 patients were seen in the department between 1970 and 1994; height and weight of 76 patients from Germany were evaluated by means of a questionnaire with detailed measuring instructions, and 206 definite cases were added from the literature. Mean ( SD) length of newborn babies with PWS was 50.2+/-2.8 cm (145 boys) and 48.9 3.3 cm (79 girls). Mean weight at birth was 2945 570 g in boys and 2782+/-594 g in girls. During the 1st year, the children's growth was nearly normal, thereafter short stature was present in approximately 50% of PWS patients. Between 3 and 13 years of age, the 50th percentile for height in PWS is roughly identical with the 3rd percentile in healthy controls. Body weight was normal for all boys and girls during the first 2 years. Thereafter, a rapid weight gain occurred; after an age of 10 years weight-for-height index in nearly all patients exceeded the normal range. The extent of pubertal growth was reduced for the group. Mean adult height was 161.6+/-8.1 cm (23 males) and 150.2+/-5.5 cm (21 females). Head circumference for age was normal for boys and girls.
Reference data on spontaneous development of growth and weight gain of children with Prader-Willi syndrome are described allowing a better counselling of patients and parents.
采用纵向和横断面混合的方式分析了315例普拉德-威利综合征（PWS）患者（109名女孩和208名男孩）的自然生长情况。1970年至1994年期间，该科室诊治了33例患者；通过一份附有详细测量说明的问卷对76名德国患者的身高和体重进行了评估，并从文献中补充了206例确诊病例。患有PWS的新生儿平均（标准差）身长为50.2±2.8厘米（145名男孩）和48.9±3.3厘米（79名女孩）。男孩出生时的平均体重为2945±570克，女孩为2782±594克。在第一年，患儿的生长情况基本正常，此后约50%的PWS患者出现身材矮小。在3至13岁之间，PWS患者身高的第50百分位数大致与健康对照者的第3百分位数相同。所有男孩和女孩在头两年体重均正常。此后，体重迅速增加；10岁以后，几乎所有患者的身高体重指数都超过了正常范围。该组青春期生长的幅度减小。成年男性的平均身高为161.6±8.1厘米（23例），成年女性为150.2±5.5厘米（21例）。男孩和女孩的年龄别头围均正常。
描述了普拉德-威利综合征患儿生长和体重增加的自然发育参考数据，有助于更好地为患者及其家长提供咨询。
