Byard P J
Department of Pediatrics, Case Western Reserve University, Ohio.
Ann Hum Biol. 1994 May-Jun;21(3):229-40. doi: 10.1080/03014469400003242.
The adolescent spurt in 230 children with cystic fibrosis (CF) treated at the Cleveland CF centre in northeastern Ohio was compared to that found in normal children from the Fels Longitudinal Growth Study in southwestern Ohio. The Preece-Baines Model 1 (PB1) growth equation was applied to longitudinal height data from both samples to describe a large number of data points for each child in terms of a few biologically meaningful parameters, such as age, height, and velocity at the take-off and peak of the adolescent growth spurt. The growth spurt is delayed by an average of 0.8 years and is about 1 cm/year slower at its peak in CF patients compared to the normal controls. This delay should be considered when comparing clinical growth measurements of adolescent CF patients with normal standards. Peak velocity is lower than expected, even for late-maturing normal children, and height at take-off, peak velocity, and adulthood is significantly reduced, especially in boys. These findings are consistent with the clinical impression that the growth spurt is delayed and attenuated in CF patients, particularly those with poor pulmonary function. Girls homozygous for the delta F508 mutation have significantly more growth retardation than those with other CF mutations.
对俄亥俄州东北部克利夫兰囊性纤维化(CF)中心治疗的230名患有囊性纤维化的儿童的青春期发育突增情况,与俄亥俄州西南部费尔斯纵向生长研究中的正常儿童进行了比较。将普里斯-贝恩斯模型1(PB1)生长方程应用于两个样本的纵向身高数据,以便根据一些具有生物学意义的参数(如年龄、身高以及青春期发育突增开始和峰值时的速度)来描述每个儿童的大量数据点。与正常对照组相比,CF患者的青春期发育突增平均延迟0.8年,且在峰值时每年约慢1厘米。在将青春期CF患者的临床生长测量结果与正常标准进行比较时,应考虑到这种延迟。即使对于发育较晚的正常儿童,峰值速度也低于预期,且开始时的身高、峰值速度和成年时的身高均显著降低,尤其是男孩。这些发现与临床印象一致,即CF患者,尤其是那些肺功能较差的患者,青春期发育突增延迟且减弱。携带ΔF508突变的纯合子女孩比携带其他CF突变的女孩有更明显的生长发育迟缓。