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重型β地中海贫血患儿葡萄糖-胰岛素内环境稳态的演变:一项从儿童早期到青年期的长达 20 年的回顾性 ICET-A 观察性分析。

The evolution of glucose-insulin homeostasis in children with β-thalassemia major (β -TM): A twenty-year retrospective ICET- A observational analysis from early childhood to young adulthood.

机构信息

Quisisana Hospital, Ferrara.

Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Sultanate of Oman.

出版信息

Acta Biomed. 2022 Jul 1;93(3):e2022243. doi: 10.23750/abm.v93i3.12643.

Abstract

BACKGROUND

Thalassemia guidelines recommend oral glucose tolerance test (OGTT), starting from the age of 10 years, or earlier in the presence of iron overload.

OBJECTIVE

The aim of this retrospective study was to review and document the changes of glucose-insulin homeostasis from early childhood to young adulthood in β-thalassemia major (β -TM) patients with impaired fasting glucose (IFG) and normal OGTT.

METHODS

All data of the clinical patients' records of 18 β -TM patients' from September 1983 to September 2021 were included in the study. Annual or biennial OGTT results, for a duration of 15-20 years, were available for all patients.

RESULTS

The main findings are: a) IFG in children with β -TM represents a risk factor for the development of glucose dysregulation (GD) at later age; b) fluctuations of glucose homeostasis during follow-up were observed mainly in β-TM patients with IFG at baseline; and c) the primary defect of GD appears to be a low degree insulin resistance (IR), as estimated by HOMA-IR, followed by an insulin secretion defect.

CONCLUSION

These results are noteworthy as they revealed that firstly, the baseline IFG predicts future development of GD, and secondly, that almost half of patients with IFG at the outset had normal glucose handling 15 years later. Understanding the sequence of abnormalities in the progression from normal glucose homeostasis to GD and identifying the risk factors for the glycometabolic defects in thalassemic patients might help in the formulation of interventions.

摘要

背景

地中海贫血症指南建议从 10 岁开始,或在铁过载的情况下更早进行口服葡萄糖耐量试验(OGTT)。

目的

本回顾性研究旨在回顾和记录从早期儿童到成年早期β-地中海贫血症(β-TM)患者中,空腹血糖受损(IFG)和正常 OGTT 患者的葡萄糖-胰岛素稳态的变化。

方法

研究纳入了 1983 年 9 月至 2021 年 9 月期间 18 名β-TM 患者的临床病历数据。所有患者的 OGTT 结果均为年度或两年一次,持续 15-20 年。

结果

主要发现为:a)β-TM 儿童的 IFG 是日后发生血糖调节异常(GD)的危险因素;b)在随访期间,血糖稳态波动主要发生在基线 IFG 的β-TM 患者中;c)GD 的主要缺陷似乎是低程度的胰岛素抵抗(IR),这是通过 HOMA-IR 估计的,其次是胰岛素分泌缺陷。

结论

这些结果值得注意,因为它们表明,首先,基线 IFG 预测未来 GD 的发展,其次,几乎一半的 IFG 患者在 15 年后葡萄糖处理正常。了解从正常血糖稳态到 GD 进展的异常顺序,并识别地中海贫血症患者糖代谢缺陷的危险因素,可能有助于制定干预措施。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d46e/9335438/b84c0488c94f/ACTA-93-243-g001.jpg

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