Aswani N, Taylor C J, McGaw J, Pickering M, Rigby A S
Academic Unit of Child Health, University of Sheffield Division of Clinical Sciences (South), Sheffield, UK.
Acta Paediatr. 2003 Sep;92(9):1029-32.
Normal growth patterns are seen throughout the first decade in children with cystic fibrosis (CF). Growth in the second decade is, however, less satisfactory and may reflect pubertal delay. This study was performed to assess the extent of pubertal delay, to examine factors that influence the timing and magnitude of the pubertal growth spurt, and to establish whether the final height for most CF patients differed significantly from the normal population.
Thirty subjects (16 male) attending a single centre were studied. Peak height velocity (PHV), final height and ages when achieved were compared with population norms. Outcome data were correlated with disease severity using Shwachman and Chrispin-Norman scores and forced expiratory volume in 1 s.
PHV was significantly later in both genders in this CF population compared with Tanner and Whitehouse standards: boys 14.6 y (95% confidence interval (95% CI) 12.4-16.8, p < 0.01) and girls 12.6 y (95% CI 10.5-14.7, p < 0.01). Mean PHV was also lower in both genders (boys 7.7 cm y(-1) and girls 6.4 cm y(-1), both p<0.001). However, final heights did not differ significantly from Freeman standards (height standard deviation scores: males--1.2, females--0.1); 52% of final heights equalled or exceeded the mid-parental centile.
CF patients showed suboptimal PHVs with a later pubertal growth spurt influenced by disease severity, but eventually achieved a normal final height.
囊性纤维化（CF）患儿在第一个十年内生长模式正常。然而，第二个十年的生长情况不太理想，可能反映青春期延迟。本研究旨在评估青春期延迟的程度，研究影响青春期生长突增时间和幅度的因素，并确定大多数CF患者的最终身高与正常人群是否存在显著差异。
对来自单一中心的30名受试者（16名男性）进行研究。将身高增长峰值速度（PHV）、最终身高及达到这些指标时的年龄与人群标准进行比较。使用Shwachman和Chrispin-Norman评分以及第1秒用力呼气量将结果数据与疾病严重程度进行关联。
与Tanner和Whitehouse标准相比，该CF人群中男女的PHV均显著延迟：男孩为14.6岁（95%置信区间（95%CI）12.4 - 16.8，p < 0.01），女孩为12.6岁（95%CI 10.5 - 14.7，p < 0.01）。男女的平均PHV也较低（男孩7.7 cm/年，女孩6.4 cm/年，均p < 0.001）。然而，最终身高与Freeman标准无显著差异（身高标准差评分：男性为 - 1.2，女性为 - 0.1）；52%的最终身高等于或超过父母身高的中位数百分位。
CF患者的PHV不理想，青春期生长突增延迟，受疾病严重程度影响，但最终达到正常的最终身高。
