Department of Haematology, College of Graduate Studies and Scientific Research, Karary University, Khartoum, Sudan.
Delta Scientific Consultation Center Alexendria, Alexandria, Egypt.
BMC Nephrol. 2024 Oct 26;25(1):380. doi: 10.1186/s12882-024-03806-5.
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.
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.
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).
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.
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。
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)。
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)。
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。