Germain-Lee Emily L, Brennen Feng-Shu, Stern Diana, Kantipuly Aditi, Melvin Pamela, Terkowitz Mia S, Shapiro Jay R
Bone and Osteogenesis Imperfecta Department, Kennedy Krieger Institute, Baltimore, Maryland.
Department of Pediatrics, Division of Pediatric Endocrinology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Pediatr Res. 2016 Mar;79(3):489-95. doi: 10.1038/pr.2015.230. Epub 2015 Nov 5.
There is strikingly limited information on linear growth and weight in the different types of osteogenesis imperfecta (OI). Here, we define growth patterns further with the intent of implementing appropriate adaptations proactively.
We report cross-sectional anthropometric data for 343 subjects with different OI types (144 children, 199 adults). Longitudinal height data for 36 children (18 girls, 18 boys) with OI type I and 10 children (8 girls, 2 boys) with OI type III were obtained.
In all cases, the height Z-scores were negatively impacted, and final height Z-scores were impacted the most. In type I, the growth velocities taper near puberty, and there is a blunted pubertal growth spurt. The growth velocities of children with type III decelerate before age 5 y; poor growth continues without an obvious pubertal growth spurt. Obesity is a concern for all patients with OI, with type III patients being the most affected.
The linear growth patterns, in addition to the marked increase in weight over time, indicate a need for lifestyle modifications early in childhood, especially a need for weight control. Further definition of the anthropometric measures in OI enables patients to begin modifications as early as possible.
关于不同类型成骨不全症（OI）患者的线性生长和体重情况，现有信息极为有限。在此，我们进一步明确生长模式，以便积极采取适当的适应性措施。
我们报告了343例不同类型OI患者（144名儿童，199名成人）的横断面人体测量数据。获取了36例I型OI儿童（18名女孩，18名男孩）和10例III型OI儿童（8名女孩，2名男孩）的纵向身高数据。
在所有病例中，身高Z评分均受到负面影响，其中最终身高Z评分受影响最大。在I型中，生长速度在青春期临近时逐渐减缓，青春期生长突增不明显。III型儿童在5岁前生长速度就开始减慢；生长缓慢持续存在，且无明显的青春期生长突增。肥胖是所有OI患者都需关注的问题，其中III型患者受影响最大。
线性生长模式以及体重随时间的显著增加表明，儿童早期就需要调整生活方式，尤其是需要控制体重。对OI患者人体测量指标的进一步明确，能使患者尽早开始调整。
