【摘要】 目的 探討基因重組人生長激素（recombinant human growth hormone，rhGH）及雌/孕激素（estrogen/progestogem，E/P）治療對Turner綜合征（Turner syndrome，TS）患兒身高及性征發育的影響。 方法 2005年1月—2009年6月四川大學華西第二醫院門診就診TS患兒22例，12例患兒接受rhGH治療，年齡（13.58±2.23）歲，劑量0.15 U/（kg?d），睡前皮下注射，療程4~24個月。16例年齡≥13歲、骨齡≥11歲的患兒接受E/P治療，療程3~30個月。 結果 rhGH治療后患兒身高、身高的標準差積分提高，生長速率達（9.33±2.39）cm/年；E/P治療可促進患兒乳房發育及規律月經出現。 結論 rhGH和E/P治療對TS患兒身高增長及性征發育有明顯療效。【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:探討基因重組人生長激素(recombinant human growth hormone, rhGH)對特發性矮小兒童促身高增長的療效。方法:ISS兒童60例,每晚睡前接受rhGH治療0.15～0.18 IU/(kg·d),療程3～9個月,并對其療效進行觀察。結果:ISS患兒經生長激素治療后,生長速率明顯增快,由治療前4.21±0.36 cm/年提高到治療后8.29±4.72 cm/年,差異有顯著性(Plt;0.05)。而骨齡和體重無明顯變化,差異不顯著(Pgt;0.05)。治療期間除少數肝功能輕度異常,注射部位輕度反應外,未發現明顯副作用。結論:rhGH對ISS兒童有增快生長速度作用。
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.