Woelfle Joachim, Lindberg Anders, Aydin Ferah, Ong Ken K, Camacho-Hubner Cecilia, Gohlke Bettina
Pediatric Endocrinology Division, Children's Hospital, University of Bonn, Bonn, Germany.
Endocrine Care, Pfizer Health AB, Sollentuna, Sweden.
Front Endocrinol (Lausanne). 2018 Feb 28;9:54. doi: 10.3389/fendo.2018.00054. eCollection 2018.
Whether children with chromosomal disorders of growth and puberty are affected by secular trends (STs) as observed in the general population remains unanswered, but this question has relevance for expectations of spontaneous development and treatment responses.
The aim of the study was to evaluate STs in birth parameters, growth, and pubertal development in girls with Turner syndrome (TS).
Retrospective analysis of KIGS data (Pfizer International Growth Database). We included all TS patients who entered KIGS between 1987 and 2012 and were born from 1975 to 2004, who were prepubertal and growth treatment naïve at first entry (total number: 7,219). Pretreatment height and ages at the start of treatment were compared across 5-year birth year groups, with subgroup analyses stratified by induced or spontaneous puberty start.
We observed significant STs across the birth year groups for birth weight [+0.18 SD score (SDS), < 0.001], pretreatment height at mean age 8 years (+0.73 SDS, < 0.001), height at the start of growth hormone (GH) therapy (+0.38 SDS, < 0.001) and start of puberty (+0.42 SDS, < 0.001). Spontaneous puberty onset increased from 15 to 30% ( < 0.001). Mean age at the start of GH treatment decreased from 10.8 to 7.4 years (-3.4 years; < 0.001), and substantial declines were seen in ages at onset of spontaneous and induced puberty (-2.0 years; < 0.001) and menarche (-2.1 years; < 0.001).
Environmental changes leading to increased height and earlier and also more common, spontaneous puberty are applicable in TS as in normal girls. In addition, greater awareness for TS may underlie trends to earlier start of GH therapy and induction of puberty at a more physiological age.
患有生长和青春期染色体疾病的儿童是否如普通人群中观察到的那样受到长期趋势(STs)的影响仍未得到解答,但这个问题对于自发发育的预期和治疗反应具有相关性。
本研究的目的是评估特纳综合征(TS)女孩出生参数、生长和青春期发育中的长期趋势。
对辉瑞国际生长数据库(KIGS)数据进行回顾性分析。我们纳入了1987年至2012年间进入KIGS且出生于1975年至2004年的所有TS患者,这些患者首次入组时未进入青春期且未接受过生长治疗(总数:7219例)。比较了5年出生年份组的治疗前身高和开始治疗时的年龄,并按青春期开始是自然发生还是诱导发生进行亚组分析。
我们观察到出生年份组之间出生体重[+0.18标准差评分(SDS),<0.001]、平均年龄8岁时的治疗前身高(+0.73 SDS,<0.001)、生长激素(GH)治疗开始时的身高(+0.38 SDS,<0.001)和青春期开始时的身高(+0.42 SDS,<0.001)存在显著的长期趋势。自然青春期开始率从15%增加到30%(<0.001)。GH治疗开始时的平均年龄从10.8岁降至7.4岁(-3.4岁;<0.001),自然青春期和诱导青春期开始的年龄(-2.0岁;<0.001)以及初潮年龄(-2.1岁;<0.001)都有大幅下降。
导致身高增加、青春期更早且更普遍自然发生的环境变化在TS女孩中与正常女孩一样适用。此外,对TS认识的提高可能是GH治疗更早开始以及在更接近生理年龄诱导青春期这一趋势的原因。