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通过网织红细胞参数和 β-球蛋白 mRNA 表达评估苏丹儿科终末期肾病血液透析患者的骨髓反应和铁状态。

Assessment of myeloid response and iron status among Sudanese pediatric ESKD on hemodialysis through reticulocyte parameters and β-globin mRNA expression.

机构信息

Department of Haematology, College of Graduate Studies and Scientific Research, Karary University, Khartoum, Sudan.

Delta Scientific Consultation Center Alexendria, Alexandria, Egypt.

出版信息

BMC Nephrol. 2024 Oct 26;25(1):380. doi: 10.1186/s12882-024-03806-5.

Abstract

BACKGROUND

Sudanese children with End-Stage Kidney Disease (ESKD) often show limited improvement in hemoglobin levels despite treatment with recombinant human erythropoietin (rHuEPO). This study aims to assess the response to rHuEPO therapy by analyzing β-globin mRNA expression and reticulocyte parameters. Additionally, it classifies anemia among Sudanese pediatric patients based on iron status, considering age and gender as biological markers for evaluating treatment response.

METHODS

A prospective observational cohort study was conducted from January 2019 to February 2020 in Khartoum, Sudan, involving 45 anemic children aged 2 to 15 years diagnosed with ESKD. The treatment protocol included rHuEPO injections and maintenance hemodialysis. Laboratory assessments consisted of complete blood count (CBC), absolute reticulocyte count, ferritin, and transferrin measurements. β-globin mRNA expression was quantified using reverse transcription polymerase chain reaction (RT-PCR), and reticulocyte parameters, including Reticulocyte Hemoglobin Content (CHr), percentage of hypochromic reticulocytes (HYPO%), and Immature Reticulocyte Fraction (IRF), were measured via flow cytometry.

RESULTS

Significant variations in hemoglobin levels were observed across different age groups (p = 0.011). Gender analysis revealed a significant association with IRF, showing a lower IRF in male patients (p = 0.017). However, there were no significant differences in hemoglobin levels between genders (p = 0.999). β-globin mRNA expression showed considerable variability, with a strong positive correlation with hemoglobin levels (r = 0.875, p < 0.0001).

CONCLUSION

Age and gender significantly influence treatment responses in children with ESKD, highlighting the need to consider growth physiology in anemia management. This study underscores the variability in β-globin mRNA expression and its association with Flow Cytometry parameters, demonstrating their effectiveness in evaluating iron status and guiding rHuEPO dosage.

摘要

背景

苏丹终末期肾病(ESKD)患儿在接受重组人促红细胞生成素(rHuEPO)治疗后,血红蛋白水平的改善往往有限。本研究旨在通过分析β-球蛋白 mRNA 表达和网织红细胞参数来评估 rHuEPO 治疗的反应。此外,根据铁状态对苏丹儿科患者的贫血进行分类,同时考虑年龄和性别作为评估治疗反应的生物学标志物。

方法

本研究是一项 2019 年 1 月至 2020 年 2 月在苏丹喀土穆进行的前瞻性观察队列研究,共纳入 45 名年龄在 2 至 15 岁、诊断为 ESKD 的贫血儿童。治疗方案包括 rHuEPO 注射和维持性血液透析。实验室评估包括全血细胞计数(CBC)、绝对网织红细胞计数、铁蛋白和转铁蛋白测定。采用逆转录聚合酶链反应(RT-PCR)定量β-球蛋白 mRNA 表达,通过流式细胞术测量网织红细胞参数,包括网织红细胞血红蛋白含量(CHr)、低色素网织红细胞百分比(HYPO%)和未成熟网织红细胞分数(IRF)。

结果

不同年龄组的血红蛋白水平存在显著差异(p=0.011)。性别分析显示与 IRF 显著相关,男性患者的 IRF 较低(p=0.017)。然而,性别间血红蛋白水平无显著差异(p=0.999)。β-球蛋白 mRNA 表达变化较大,与血红蛋白水平呈强正相关(r=0.875,p<0.0001)。

结论

年龄和性别对 ESKD 患儿的治疗反应有显著影响,提示在贫血管理中需要考虑生长生理学。本研究强调了β-球蛋白 mRNA 表达的可变性及其与流式细胞术参数的相关性,表明这些参数在评估铁状态和指导 rHuEPO 剂量方面具有有效性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/259b/11514810/7eecc742615d/12882_2024_3806_Fig1_HTML.jpg

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