An analysis of acid-labile subunit (ALS) levels in children with short stature born with normal weight.

Tomasz Jackowski, Anita Horodnicka-Józwa, Ewa Berus, Mieczysław Walczak, Elżbieta Petriczko
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Abstract

Introduction: The acid-labile subunit (ALS) is a protein best known for its function in stabilising the insulin like growth factor-1/2-insulin-like growth factor-1 binding protein 3/5 (IGF-1/2-IGFBP3/5) binary complex by creating the ternary complex and in consequence regulating the biological activity of IGF-1. The aim of the study was to assess ALS concentrations in a chosen population of children with short stature taking into account their clinical diagnosis.

Material and methods: A total of 109 prepubertal children were involved in the study - 85 children in the study group and 24 in controls. In all the children IGF-1, IGFBP3, and ALS were measured. The study group was divided according to diagnosis into groups: growth hormone deficiency (GHD), constitutional delay of growth and puberty (CDGP), idiopathic short stature (ISS), and familial short stature (FSS).

Results: In the control group the ALS concentration ranged from 4.81 to 13.66 μg/mL. In the whole study group the ALS concentration ranged from 2.73 to 15.81 μg/mL. The difference between both groups was statistically significant (p < 0.0001, R = 0.39). A strong, statistically significant correlation between ALS levels and age was observed, but only in the study group (p < 0.0001, r = 0.59). The ALS standard deviation score (SDS) was not significantly different between the control and CDGP children (p = 0.0644). The ALS concentration was significantly lower in children with short stature. There was, however, no difference between the subgroups of the study group.

Conclusion: There was no significant difference in ALS SDS between the control group and children with constitutional delay of growth and development. The usefulness of ALS in routine short stature diagnostics is uncertain, but it might play a role in the diagnosis of children with ISS and CDGP in the future.

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分析出生时体重正常的矮身材儿童体内的酸亲和素亚基(ALS)水平。
导言:嗜酸亚基(ALS)是一种蛋白质,其最著名的功能是通过创建三元复合物稳定胰岛素样生长因子-1/2-胰岛素样生长因子-1结合蛋白3/5(IGF-1/2-IGFBP3/5)二元复合物,从而调节IGF-1的生物活性。本研究的目的是根据临床诊断结果,对所选身材矮小儿童群体中的 ALS 浓度进行评估:共有 109 名青春期前儿童参与研究,其中研究组 85 名,对照组 24 名。对所有儿童的 IGF-1、IGFBP3 和 ALS 进行了测量。研究组根据诊断分为以下几组:生长激素缺乏症(GHD)、生长和青春期发育迟缓(CDGP)、特发性矮身材(ISS)和家族性矮身材(FSS):在对照组中,ALS 的浓度介于 4.81 至 13.66 μg/mL 之间。整个研究组的 ALS 浓度介于 2.73 至 15.81 μg/mL 之间。两组之间的差异具有统计学意义(P < 0.0001,R = 0.39)。ALS 水平与年龄之间存在密切的统计学意义上的相关性,但仅在研究组中存在(p < 0.0001,r = 0.59)。对照组和 CDGP 儿童的 ALS 标准差评分(SDS)无明显差异(p = 0.0644)。身材矮小儿童的 ALS 浓度明显较低。然而,研究组中各分组之间没有差异:结论:对照组和生长发育迟缓儿童的 ALS SDS 没有明显差异。ALS在常规矮身材诊断中的作用尚不确定,但它将来可能会在ISS和CDGP患儿的诊断中发挥作用。
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