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目的了解不同初治年龄对先天性肾上腺皮质增生症(CAH)患儿身高、骨龄、性早熟等方面的影响。方法将1982～2004年在上海新华医院和上海市儿科医学研究所内分泌、遗传代谢病专科诊治的32例CAH患儿(年龄:女≥8岁,男≥9岁),按初治年龄分为≤3岁组(14例)和>3岁组(18例),观察两组间末次复诊时骨龄与身高龄之差、性早熟例数及男女患儿发生性早熟的不同。结果14例初治年龄≤3岁患儿末次复诊时骨龄与身高龄之差[(3.0±2.0)岁]与18例>3岁组[(4.6±1.6)岁]比较差异有显著性(P<0.05),初治年龄>3岁组发生真性性早熟(9例)与≤3岁组(2例)比较差异有显著性(χ2=4.453,P<0.05)。男性患儿发生真性性早熟(9例)与女性患儿(2例)比较差异有显著性(χ2=4.794,P<0.05)。结论CAH患儿≤3岁得到诊治者其预测终身高较>3岁方诊治者明显改善,其性早熟发生率明显减少,男性CAH患儿较女性CAH患儿更易发生性早熟。
Objective To investigate the effect of different initial treatment on the height, bone age and precocious puberty in children with congenital adrenal hyperplasia (CAH). Methods Thirty-two children with CAH (Age≥8 years old, male≥9 years) diagnosed and treated by endocrine and genetic metabolic diseases in Shanghai Xinhua Hospital and Shanghai Institute of Pediatric Medicine from 1982 to 2004 were divided into ≤3 years old group (14 cases) and> 3 years old group (18 cases). The differences of skeletal age and seniority, the number of precocious puberty cases and the precocious puberty between the two groups were observed. Results The difference of skeletal age and height at the last follow-up in 14 patients ≤3 years of age [(3.0 ± 2.0) years] was significantly different from that in 18 patients> 3 years (4.6 ± 1.6) years (P (P <0.05). There was significant difference between the age of onset> 3 years old group (9 cases) and the age≤3 years group (2 cases) (χ2 = 4.453, P <0.05). There were significant differences between males (9 cases) and female females (χ2 = 4.794, P <0.05). Conclusion The children with CAH ≤3 years old have higher life expectancy than those aged> 3 years old. The incidence of precocious puberty in CAH children is obviously reduced. The incidence of precocious puberty in CAH children is higher than that in female children with CAH.