Rafia S, García-Peña J J, López-Pisón J, Aguirre-Rodríguez J, Ramos-Lizana J, García-Pérez A, Martínez-Granero M A, Sans A, Campistol J, Peña-Segura J L, Espino-Hernández M
Servicio de Neurología Pediátrica, Hospital Infantil Universitario La Paz, Madrid, Spain.
Rev Neurol. 2004;38(11):1009-12.
To make the charts of stature and head circumference of Spanish pediatric patients affected with neurofibromatosis type 1 (NF1), to compare them with the ones from a healthy population and to review the possible causes of its alterations.
We obtained the data from 251 Spanish patients (122 women and 129 men) with NF1 seen in seven hospitals between the years 2000 and 2002, with ages between 1 month and 18 years old. The calculation of the 50th centile or median was done using the method of mobile variables, and the 3rd and 97th centiles where calculated from this median.
Girls with NF1 have a shorter stature than the healthy ones, which becomes evident after 10 years of age. We have not found differences in the stature between boys with the disease and those without it. In regards to head circumference, in boys and girls we have observed that it is bigger throughout life in patients affected with NF1, and that the 50th centile of healthy people correlates with the 3rd centile of those affected, the 97th centile of the healthy ones with the 50th centile of those affected, being the 3rd centile of the healthy people well below the 3rd centile of the affected ones, and the 97th centile of the affected ones well above the 97th of the healthy people.
The short stature in postpuberal girls and the macrocephaly in both sexes at all ages, are primary characteristics in the NF1 and they are not usually related with other alterations, although they are not specific for this illness.
绘制1型神经纤维瘤病(NF1)西班牙儿科患者的身高和头围图表,将其与健康人群的图表进行比较,并探讨其变化的可能原因。
我们收集了2000年至2002年间在七家医院就诊的251例西班牙NF1患者(122名女性和129名男性)的数据,年龄在1个月至18岁之间。使用移动变量法计算第50百分位数或中位数,并据此计算第3和第97百分位数。
患有NF1的女孩身高比健康女孩矮,这在10岁以后变得明显。我们未发现患病男孩与未患病男孩在身高上存在差异。关于头围,我们观察到,患有NF1的患者无论男女,其头围在一生中都较大,健康人群的第50百分位数与患病者的第3百分位数相关,健康人群的第97百分位数与患病者的第50百分位数相关,健康人群的第3百分位数远低于患病者的第3百分位数,患病者的第97百分位数远高于健康人群的第97百分位数。
青春期后女孩身材矮小以及各年龄段男女头围增大是NF1的主要特征,它们通常与其他改变无关,尽管并非该疾病所特有。