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目的了解不同的用药方式对先天性肾上腺皮质增生症(CAH)患儿生长速率及骨龄进展的影响,为选择一种较好的用药方式提供依据。方法对2005—2009年在武汉同济医院儿科遗传代谢内分泌专科门诊就诊的18例CAH患儿(男9例,女9例)进行回顾性分析。根据氢化可的松用药方式不同分为A组(自2007年5月开始用药,用药方式为新方式,即每天用药3次,早8时和下午4时均服用全天总量的1/4,晚10时左右服用全天总量的1/2。)和B组(用药为2007年5月前用旧方式而2007年5月后改用新方式,旧方式即全天总量分2次或1次服用),B组又根据前后用药方式的改变分为B1组(旧方式组)和B2组(新方式组),分别比较A组和B1组、B1组和B2组年生长速率(GV)、身高年龄增长(ΔHA)、ΔHA与骨龄增长(ΔBA)间的关系(ΔHA/ΔBA)以及骨龄变化与实际年龄变化的比值(ΔBA/ΔCA)的差异。结果 A组和B1组、B2组和B1组相比,△BA/△CA的值均显著降低(P<0.05),ΔHA与ΔHA/ΔBA的值差异均无统计学意义(P>0.05)。A组和B1组的GV差异无统计学意义(P>0.05),但B2组和B1组相比,GV显著降低(P<0.05)。结论患儿以新方式用药在抑制骨龄进展和保持正常生长方面明显优于旧方式,新方式用药可增加患儿身高增长空间,提高患儿终身高。
Objective To understand the effects of different modes of administration on the growth rate and bone age of children with congenital adrenal hyperplasia (CAH), and to provide a basis for choosing a better mode of treatment. Methods A retrospective analysis was performed on 18 CAH children (9 males and 9 females) who were treated in Department of Genetics, Metabolism and Endocrinology, Tongji Hospital, Wuhan University from 2005 to 2009. According to hydrocortisone medication is divided into different groups A (from May 2007 started medication, medication is a new way, that is, three times daily medication, as early as 8 o’clock and 4 o’clock taking the total amount of 1/4 , Taking about 1/2 of the total amount of the day after 10 o’clock in the evening) and Group B (medication was used before May 2007 instead of May 2007 and new method was adopted after May 2007, B group was divided into B1 group (old mode group) and B2 group (new mode group) according to the changes of before and after treatment mode, and compared the annual growth rate of group A and group B1, group B1 and group B2 (ΔV A) and bone age (Δ HA / Δ BA), and the ratio of changes in bone age to actual age (ΔBA / ΔCA). Results The values of △ BA / △ CA in group A and group B1, group B2 were significantly lower than those in group B1 (P <0.05). There was no significant difference in the values of ΔHA and ΔHA / ΔBA (P> 0.05). There was no significant difference in GV between group A and group B1 (P> 0.05), but GV was significantly lower in group B2 compared with group B1 (P <0.05). Conclusion The new drug treatment in children is superior to the old one in inhibiting the progress of bone age and maintaining normal growth. The new method can increase the height growth of children and increase the lifelong height of children.