Nickavar Azar, Qmarsi Azadeh, Ansari Shahla, Zarei Elham
Department of Pediatric Nephrology, Iran University of Medical Sciences, Tehran, Iran.
Iran J Kidney Dis. 2017 Mar;11(2):132-137.
Renal involvement is a rare complication of β-thalassemia. Both tubular and glomerular dysfunction might occur in these patients. The aim of this study was to evaluate and compare kidney function in the major, intermedia, and minor variants of β-thalassemia.
Renal tubular and glomerular function of 72 patients with β-thalassemia (25 major, 23 intermedia, and 24 minor) were evaluated. Patients older than 40 years and those with chronic kidney disease, diabetes mellitus, congestive heart failure, associated infections, congenital anomalies of the kidney and urinary tract were excluded. Blood and urine samples were collected electrolytes and markers of kidney function.
Mean age at the time of study was significantly higher in the minor group. The majority of patients with thalassemia major were males. Hematuria and pyuria occurred in 4% to 8% of the patients. Serum level of all variables were within normal limits, with no significant difference between the three groups. Glomerular filtration rate was nonsignificantly higher in the major and intermedia groups, compared to the minor variant. A significantly lower urine phosphorus and uric acid excretion was noted with the minor variant. Urine phosphorus and uric acid excretion increased more frequently in the major and intermedia groups.
Tubular and glomerular functions appear to be well preserved in all variants of β-thalassemia.
肾脏受累是β地中海贫血的一种罕见并发症。这些患者可能会出现肾小管和肾小球功能障碍。本研究的目的是评估和比较β地中海贫血的重型、中间型和轻型变异型患者的肾功能。
对72例β地中海贫血患者（25例重型、23例中间型和24例轻型）的肾小管和肾小球功能进行了评估。排除年龄超过40岁以及患有慢性肾脏病、糖尿病、充血性心力衰竭、合并感染、肾脏和尿路先天性异常的患者。采集血液和尿液样本检测电解质和肾功能指标。
轻型组研究时的平均年龄显著更高。大多数重型地中海贫血患者为男性。4%至8%的患者出现血尿和脓尿。所有变量的血清水平均在正常范围内，三组之间无显著差异。与轻型变异型相比，重型和中间型组的肾小球滤过率略高，但无统计学意义。轻型变异型患者的尿磷和尿酸排泄显著降低。重型和中间型组的尿磷和尿酸排泄更频繁增加。
β地中海贫血的所有变异型患者的肾小管和肾小球功能似乎均保存良好。
