Department of Pediatrics, Groene Hart Ziekenhuis, PO Box 1098, Gouda, The Netherlands.
Eur J Pediatr. 2011 Feb;170(2):207-11. doi: 10.1007/s00431-010-1287-3. Epub 2010 Sep 10.
Ellis-van Creveld (EvC) syndrome is a congenital malformation syndrome with marked growth retardation. In this study, specific growth charts for EvC patients were derived to allow better follow-up of growth and earlier detection of growth patterns unusual for EvC. With the use of 235 observations of 101 EvC patients (49 males, 52 females), growth charts for males and females from 0 to 20 years of age were derived. Longitudinal and cross-sectional data were collected from an earlier review of growth data in EvC, a database of EvC patients, and from recent literature. To model the growth charts, the GAMLSS package for the R statistical program was used. Height of EvC patients was compared to healthy children using Dutch growth charts. Data are presented both on a scale for age and on a scale for the square root of age. Compared to healthy Dutch children, mean height standard deviation score values for male and female EvC patients were -3.1 and -3.0, respectively. The present growth charts should be useful in the follow-up of EvC patients. Most importantly, early detection of growth hormone deficiency, known to occur in EvC, will be facilitated.
Ellis-van Creveld (EvC) 综合征是一种先天性畸形综合征，具有明显的生长迟缓。在这项研究中，得出了特定的 EvC 患者生长图表，以更好地跟踪生长情况，并更早地发现 EvC 特有的生长模式。使用 101 名 EvC 患者（49 名男性，52 名女性）的 235 次观察结果，得出了 0 至 20 岁男性和女性的生长图表。纵向和横断面数据来自对 EvC 生长数据的早期回顾、EvC 患者数据库以及最近的文献。为了构建生长图表，使用了 R 统计程序中的 GAMLSS 包。将 EvC 患者的身高与荷兰健康儿童的身高进行了比较。数据分别在年龄尺度和年龄平方根尺度上呈现。与荷兰健康儿童相比，男性和女性 EvC 患者的身高标准差评分值分别为-3.1 和-3.0。本研究中的生长图表在 EvC 患者的随访中应该是有用的。最重要的是，将促进对已知在 EvC 中发生的生长激素缺乏的早期检测。
