Nagai T, Matsuo N, Kayanuma Y, Tonoki H, Fukushima Y, Ohashi H, Murai T, Hasegawa T, Kuroki Y, Niikawa N
Department of Pediatrics, Dokkyo University School of Medicine Koshigaya Hospital, Saitama, Japan.
Am J Med Genet. 2000 Nov 13;95(2):130-4. doi: 10.1002/1096-8628(20001113)95:2<130::aid-ajmg7>3.0.co;2-r.
We constructed the standard growth (length/height and weight) curves for Japanese individuals with Prader-Willi syndrome (PWS). Crude height and weight data were collected from 153 males and 99 females with the syndrome, and the collected data were arranged by a mathematical method to construct the curves. Height growth patterns were quite different between PWS and normal children. Mean height of individuals with the syndrome by puberty is -2 SD for normal children, and it drops off far below -2 SD value after puberty. Final mean height is 141.2 +/- 4.8 cm for females (n = 13) and 147.7 +/- 7.7 cm for males (n = 17), showing 15.8 and 21.9 cm below the average height for normal Japanese girls and boys, respectively. Thus, the degree of shortness is more pronounced in male than in female patients. There was no difference in height between those with chromosome 15q deletion and those without. Mean weight at birth of girls (n = 88) and boys (n = 131) were 2.70 +/- 0.45 Kg and 2.62 +/- 0.47 Kg, respectively. These values were smaller than those for normal neonates (P < 0.05, t-test). The weight of PWS children was under the mean value for normal infants by age 2 years, but gradually increase above the mean values for normal children around ages 2-4 years. Overweight in both males and females becomes obvious during prepuberty. Growth patterns are not different between Japanese and Caucasian children with the syndrome. Short stature is more prominent in boys of both ethnic groups, whereas the degree of overweight appears much more severe in Caucasians.
我们构建了日本普拉德-威利综合征（PWS）患者的标准生长（身高/身长和体重）曲线。收集了153名男性和99名女性该综合征患者的原始身高和体重数据，并通过数学方法对收集到的数据进行整理以构建曲线。PWS患者与正常儿童的身高增长模式有很大不同。该综合征患者青春期时的平均身高比正常儿童低2个标准差，青春期后则远低于-2个标准差。女性（n = 13）的最终平均身高为141.2±4.8厘米，男性（n = 17）为147.7±7.7厘米，分别比正常日本女孩和男孩的平均身高低15.8厘米和21.9厘米。因此，男性患者的矮小程度比女性更明显。15号染色体q臂缺失的患者与未缺失的患者在身高上没有差异。女孩（n = 88）和男孩（n = 131）出生时的平均体重分别为2.70±0.45千克和2.62±0.47千克。这些值低于正常新生儿（P < 0.05，t检验）。PWS儿童在2岁时体重低于正常婴儿的平均值，但在2至4岁左右逐渐超过正常儿童的平均值。青春期前，男性和女性的超重情况都很明显。日本和白种人患有该综合征的儿童生长模式没有差异。两个种族的男孩身材矮小更为突出，而白种人的超重程度似乎更为严重。
