【摘要】 目的 探讨基因重组人生长激素（recombinant human growth hormone，rhGH）及雌/孕激素（estrogen/progestogem，E/P）治疗对Turner综合征（Turner syndrome，TS）患儿身高及性征发育的影响。 方法 2005年1月—2009年6月四川大学华西第二医院门诊就诊TS患儿22例，12例患儿接受rhGH治疗，年龄（13.58±2.23）岁，剂量0.15 U/（kg•d），睡前皮下注射，疗程4~24个月。16例年龄≥13岁、骨龄≥11岁的患儿接受E/P治疗，疗程3~30个月。 结果 rhGH治疗后患儿身高、身高的标准差积分提高，生长速率达（9.33±2.39）cm/年；E/P治疗可促进患儿乳房发育及规律月经出现。 结论 rhGH和E/P治疗对TS患儿身高增长及性征发育有明显疗效。【Abstract】 Objective To explore the therapeutic effects of recombinant human growth hormone (rhGH) and sex hormone on the stature and sex feature of children with Turner syndrome (TS). Methods A total of 22 children with TS were selected in the outpatients department of West China Second Hospital between January 2005 and June 2009. Twelve children with TS the average age of (13.58±2.23) years received rhGH [0.15 U/(kg•d)] every night before sleep for 4-24 months . Sixteen children with TS (age≥14 years old, bone age≥11 years old) underwent estrogen and progestogen (E/P) treatment for 3-30 months. Results The height and height standard deviation score increased significantly in children with rhGH therapy (Plt;0.01). The height velocity was (9.33±2.39) cm/year after the treatment. The treatment of estrogen and progestogen could promote the development of breast and establish menstrual cycle in children with TS. Conclusion rhGH and E/P can play a significant role in treatment of TS in children.
目的:探讨基因重组人生长激素(recombinant human growth hormone, rhGH)对特发性矮小儿童促身高增长的疗效。方法:ISS儿童60例,每晚睡前接受rhGH治疗0.15～0.18 IU/(kg·d),疗程3～9个月,并对其疗效进行观察。结果:ISS患儿经生长激素治疗后,生长速率明显增快,由治疗前4.21±0.36 cm/年提高到治疗后8.29±4.72 cm/年,差异有显著性(Plt;0.05)。而骨龄和体重无明显变化,差异不显著(Pgt;0.05)。治疗期间除少数肝功能轻度异常,注射部位轻度反应外,未发现明显副作用。结论:rhGH对ISS儿童有增快生长速度作用。
Objective To explore the accuracy and practicability of bone age assessment for the diagnosis of idiopathic precocious puberty (IPP). Methods According to the “Gold Standard”, we selected 55 girls with IPP for the study group, and 83 normal girls for the control group. We retrospectively analyzed the first left hand-wrist radiographs at the first visit. Bone ages were assessed by using a single-blind method according to the RUS (Radius Ulna and Short bones), carpale and 20 bones method (TW2). Each had 5 decision thresholds (gt;97th percentile, gt;90th percentile, gt;75th percentile, gt;50th percentile and ≤50th percentile). The diagnostic values from RUS, carpale and 20 bones methods assessing bone age were analyzed to identify the best decision threshold. Results ① Both sensitivity and specificity of the four decision thresholds were relatively higher, including gt;90th percentile of RUS (sensitivity 0.836, specificity 0.916), gt;90th percentile of carpale (sensitivity 0.746, specificity 0.916), gt;90th and gt;75th percentile of 20 bone (sensitivity 0.746, specificity 0.964 and sensitivity 0.982, specificity 0.783, respectively). ② Area under receiver operator characteristic curve (AUR): AUR of RUS 0.939 ± 0.019 (95%CI 0.902 to 0.977), AUR of carpale 0.899 ± 0.028 (95%CI 0.845 to 0.954), AUR of 20 bone 0.958 ± 0.014 (95%CI 0.930 to 0.986). No significant difference was found (F=2.03, P=0.13). ③ Agreement assessment within-observer reliability was 89.28%, and between-observer reliability was 80.3% (Kappa 0.68, u=6.87, P＜0.01). Conclusions RUS and 20 bones methods have high accuracy for the diagnosis of idiopathic precocious puberty. Considering sensitivity and specificity, we think that ＞90th percentile of RUS is the best decision threshold.