Improved growth velocity during thyroid hormone therapy in children with Fanconi anemia and borderline thyroid function
Improved growth velocity during thyroid hormone therapy in children with Fanconi anemia and borderline thyroid function
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DOI：
10.1002/pbc.21673
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发表时间：
2008-11-01
影响因子：
3.2
通讯作者：
Rose, Susan R.
中科院分区：
文献类型：
--
作者：
Eyal, Ori;Blum, Samantha;Rose, Susan R.
背景范可尼贫血（FA）的儿童往往身材矮小，促甲状腺素（TSH）轻度升高，游离甲状腺素（FT 4）临界低。目的是检查甲状腺激素治疗是否改善。FA和临界甲状腺功能试验的儿童线性生长程序。对63例FA患儿进行甲状腺功能检查。受试者参加了随机顺序，双盲，交叉治疗7个月的evothyroxine和7个月的安慰剂。监测包括每个阶段1、4和7个月时的生长测量和实验室测定。从每个治疗阶段的分析中排除1个月导入/洗脱期。结果大多数（63%）FA儿童的甲状腺功能检查结果为临界值。入组治疗研究的所有8例FA受试者的FT 4均在正常范围的最低三分之一范围内，即0.8-1.8 ng/dL（10.3-23.2 pmol/L）[FT 4 0.9 +/- 0.1 ng/dL（平均值+/- SD），范围0.8-1.2 ng/dL（10.3-15. 4 pmol/L）]。8名受试者中有6名（4.0 +/- 1.5 mU/L，1.9-7.3 mU/L）的TSH（最佳范围0.5-3 mU/L）处于临界升高状态。基线时生长速度缓慢，与安慰剂治疗期相比，甲状腺治疗期生长速度显著改善（2.1 +/- 1.2 cm/年vs. 5.4 +/- 1.7 cm/年，P < 0.05）。结论.甲状腺激素治疗是安全的，可以改善甲状腺功能处于临界状态的FA患儿的线性生长速度。轻微的甲状腺功能减退对儿童的生长发育有重要意义。甲状腺激素治疗是否能改善这些儿童的成年身高仍有待阐明。
Background. Children with Fanconi anemia (FA) tend to have short stature, mild thyrotropin (TSH) elevation, and borderline low free thyroxine (FT4). Objective was to examine whether thyroid hormone therapy improves. linear growth in children with FA and borderline thyroid function tests Procedure. Thyroid function tests were performed in 63 children with FA. Fight subjects participated in a random order, double-blind, cross-over treatment for 7 months with evothyroxine and for 7 months with placebo. Monitoring included growth measurements and laboratory assays at 1, 4 and 7 months of each phase. A 1 month lead in/wash out period was excluded from analysis of each treatment phase. Results. The majority (63%) of FA children had borderline thyroid function tests. All eight FA subjects enrolled in the treatment study had FT4 in the lowest third of the normal range of 0.8-1.8 ng/dL (10.3-23.2 pmol/L) [FT4 0.9 +/- 0.1 ng/dL (mean +/- SD), range 0.8-1.2 ng/dL (10.3-15. 4 pmol/L)]. TSH (optimal range 0.5-3 mU/L) was borderline elevated in six of eight subjects (4.0 +/- 1.5 mU/L, 1.9-7.3 mU/L). Growth velocity was slow at baseline and improved significantly during the thyroid phase compared to the placebo phase (2.1 +/- 1.2 cm/year vs. 5.4 +/- 1.7 cm/year, P < 0.05). Conclusions. Thyroid hormone therapy is safe and may improve linear growth velocity ill children with FA who have borderline thyroid function. Subtle hypothyroidism has importance for growth in children. Whether thyroid hormone treatment improves adult height in these children remains to be elucidated.