Shaalan Mohammad G, Hassan Meaad K, Al-Shanoof Hamid J, Al Naama Lamia M
Department of Pediatrics, Basrah Maternity and Child Hospital, Basrah, IRQ.
Department of Pediatrics, College of Medicine, University of Basra, Basrah, IRQ.
Cureus. 2022 Sep 15;14(9):e29183. doi: 10.7759/cureus.29183. eCollection 2022 Sep.
Background With optimum transfusion and chelation therapy, the survival of β-thalassemia patients and the incidence of various complications, including renal complications, have improved. Objectives To investigate renal involvement in β-thalassemia patients using serum and urinary biochemical markers of glomerular and tubular dysfunction. Methods This case-control study included 69 β-thalassemia major (β-TM) patients, 23 β-thalassemia intermedia (β-TI) patients, and 100 healthy controls, all ranging from 1 to 16 years in age. Blood urea nitrogen (BUN), serum ferritin, serum and urinary levels of creatinine (Cr), uric acid (UA), calcium (Ca), phosphorus (Ph), magnesium (Mg), sodium (Na), and potassium (K), and the urinary albumin/creatinine ratio were evaluated. Results The BUN level and the urinary Na/Cr, K/Cr, Ca/Cr, Mg/Cr, Ph/Cr, albumin/Cr, and UA/Cr ratios were significantly higher in the β-thalassemia patients than in the controls. In contrast, the serum Na, K, Ca, and Mg levels were significantly lower in the patients (P<0.05). An elevated urinary UA/Cr ratio was found in 61.9% of β-thalassemia patients, and an elevated urinary Ca/Cr, and urinary albumin/Cr ratio was found in 53.2%. An elevated Na/Cr ratio was found in 41.3%. The serum and urinary renal markers showed no significant differences between patients with β-TM and β-TI, except for microscopic hematuria, which was significantly higher in β-TI patients (34.8%) than in β-TM patients (13%), P>0.02. At an older age, high serum ferritin levels and deferoxamine therapy were associated with significant tubular and glomerular dysfunction in β-thalassemia patients. Conclusions Pediatric patients with β-thalassemia have significantly abnormal tubular and glomerular functions, necessitating early detection and monitoring to prevent/reverse renal function deterioration.
背景 通过优化输血和螯合疗法，β地中海贫血患者的生存率以及包括肾脏并发症在内的各种并发症的发生率均有所改善。目的 使用肾小球和肾小管功能障碍的血清和尿液生化标志物研究β地中海贫血患者的肾脏受累情况。方法 本病例对照研究纳入了69例重型β地中海贫血（β-TM）患者、23例中间型β地中海贫血（β-TI）患者和100名健康对照者，年龄均在1至16岁之间。评估了血尿素氮（BUN）、血清铁蛋白、血清和尿液中的肌酐（Cr）、尿酸（UA）、钙（Ca）、磷（Ph）、镁（Mg）、钠（Na）和钾（K）水平，以及尿白蛋白/肌酐比值。结果 β地中海贫血患者的BUN水平以及尿Na/Cr、K/Cr、Ca/Cr、Mg/Cr、Ph/Cr、白蛋白/Cr和UA/Cr比值均显著高于对照组。相比之下，患者的血清Na、K、Ca和Mg水平显著较低（P<0.05）。61.9%的β地中海贫血患者尿UA/Cr比值升高，53.2%的患者尿Ca/Cr和尿白蛋白/Cr比值升高。41.3%的患者Na/Cr比值升高。除镜下血尿外，β-TM和β-TI患者的血清和尿液肾脏标志物无显著差异，β-TI患者的镜下血尿（34.8%）显著高于β-TM患者（13%），P>0.02。在年龄较大时，高血清铁蛋白水平和去铁胺治疗与β地中海贫血患者显著的肾小管和肾小球功能障碍有关。结论 小儿β地中海贫血患者的肾小管和肾小球功能明显异常，需要早期检测和监测以预防/逆转肾功能恶化。
