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特纳综合征女孩的生长情况。

Growth in girls with Turner syndrome.

机构信息

Department of Pediatrics, Toranomon Hospital, Tokyo, Japan.

Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.

出版信息

Front Endocrinol (Lausanne). 2023 Jan 12;13:1068128. doi: 10.3389/fendo.2022.1068128. eCollection 2022.

Abstract

Turner syndrome (TS) is a chromosomal disorder affecting females characterized by short stature and gonadal dysgenesis. Untreated girls with TS reportedly are approximately 20-cm shorter than normal girls within their respective populations. The growth patterns of girls with TS also differ from those of the general population. They are born a little smaller than the normal population possibly due to a mild developmental delay in the uterus. After birth, their growth velocity declines sharply until 2 years of age, then continues to decline gradually until the pubertal age of normal children and then drops drastically around the pubertal period of normal children because of the lack of a pubertal spurt. After puberty, their growth velocity increases a little because of the lack of epiphyseal closure. A secular trend in height growth has been observed in girls with TS so growth in excess of the secular trend should be used wherever available in evaluating the growth in these girls. Growth hormone (GH) has been used to accelerate growth and is known to increase adult height. Estrogen replacement treatment is also necessary for most girls with TS because of hypergonadotropic hypogonadism. Therefore, both GH therapy and estrogen replacement treatment are essential in girls with TS. An optimal treatment should be determined considering both GH treatment and age-appropriate induction of puberty. In this review, we discuss the growth in girls with TS, including overall growth, pubertal growth, the secular trend, growth-promoting treatment, and sex hormone replacement treatment.

摘要

特纳综合征(TS)是一种影响女性的染色体疾病,其特征为身材矮小和性腺发育不良。未经治疗的 TS 女孩据称比其所在人群中的正常女孩矮约 20 厘米。TS 女孩的生长模式也与普通人群不同。她们出生时比正常人群略小,可能是由于子宫内发育迟缓。出生后,她们的生长速度急剧下降,直到 2 岁,然后逐渐下降,直到正常儿童的青春期,然后在正常儿童的青春期前后急剧下降,因为缺乏青春期突增。青春期后,由于骨骺闭合,她们的生长速度略有增加。在 TS 女孩中观察到身高生长的长期趋势,因此在评估这些女孩的生长时,应尽可能使用超过长期趋势的生长。生长激素(GH)已被用于加速生长,已知可增加成人身高。由于促性腺激素性性腺功能减退,大多数 TS 女孩还需要雌激素替代治疗。因此,GH 治疗和雌激素替代治疗对 TS 女孩都是必不可少的。在考虑 GH 治疗和适当的青春期诱导的情况下,应确定最佳治疗方案。在这篇综述中,我们讨论了 TS 女孩的生长,包括总体生长、青春期生长、长期趋势、促进生长治疗和性激素替代治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6d96/9877326/e3925690cdaf/fendo-13-1068128-g001.jpg

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