Ghosal S, Taylor C J, Pickering M, McGaw J
Department of Paediatrics, University of Sheffield.
Arch Dis Child. 1996 Sep;75(3):191-3. doi: 10.1136/adc.75.3.191.
Growth in length, weight gain, and head circumference were recorded from 3 months to 4 years of age in 25 children with cystic fibrosis diagnosed by neonatal screening. Mean standard deviation (SD) scores at 3 months for length, weight, and head circumference were -0.78, -0.91, and -0.52 respectively. Over the first 2 years length SD scores showed a consistent improvement and stabilised at 0.1 SD below mean from 2 to 4 years. Weight SD scores remained essentially unaltered throughout, approximately 1 SD below the mean. Head circumference, however, after an initial increase from -0.52 at 3 months to -0.25 at 18 months, slowed and fell to 1 SD below the mean at 4 years. The data suggest that head growth continues to lag behind somatic growth in children with cystic fibrosis despite early diagnosis and good nutritional management in early infancy. These data also support functional expression of cystic fibrosis transmembrane conductance regulator in the brain.
对25名通过新生儿筛查确诊为囊性纤维化的儿童,记录了其3个月至4岁期间的身长增长、体重增加和头围情况。3个月时身长、体重和头围的平均标准差(SD)评分分别为-0.78、-0.91和-0.52。在最初2年,身长SD评分持续改善,并在2至4岁时稳定在低于均值0.1个标准差。体重SD评分在整个期间基本保持不变,约低于均值1个标准差。然而,头围在3个月时从-0.52最初增加到18个月时的-0.25后,增长放缓,并在4岁时降至低于均值1个标准差。数据表明,尽管在婴儿早期进行了早期诊断和良好的营养管理,但囊性纤维化患儿的头部生长仍继续落后于身体生长。这些数据也支持囊性纤维化跨膜传导调节因子在大脑中的功能性表达。