Feinstein S, Becker-Cohen R, Algur N, Raveh D, Shalev H, Shvil Y, Frishberg Y
Division of Pediatric Nephrology, Biochemistry Laboratory, and Infectious Diseases, Shaare Zedek Medical Center and Hebrew University-Hadassah School of Medicine, Jerusalem, Israel.
Am J Kidney Dis. 2001 Apr;37(4):736-42. doi: 10.1016/s0272-6386(01)80122-0.
Anemia in persistent nephrotic syndrome (NS) has been described in a few case reports but has not been studied systematically. We present a group of 19 children with NS who developed anemia before the deterioration of kidney function. The aim of our study is to determine whether erythropoietin (EPO) and/or iron deficiency are causative factors and to evaluate the effect of EPO replacement therapy. Serum EPO levels, iron status, and vitamin B(12) concentrations were measured in nephrotic patients with anemia (NS-A) and compared with those of nephrotic children with normal hemoglobin (Hb) levels (NS-NHb; n = 13). Two control groups consisted of age-matched patients without kidney disease or hypoxemia with either iron deficiency anemia (IDA; n = 19) or normal Hb concentrations (NHb; n = 16). Most NS-A patients experienced persistent steroid-resistant NS, whereas most NS-NHb children had steroid-responsive NS. Although serum iron, ferritin, and B(12) levels were significantly lower in NS-A children, appropriate replacement therapy that resulted in normalization of ferritin and/or cobalamin levels did not lead to correction of the anemia. NS-A patients had greater EPO levels than those without anemia (21.6 +/- 3.3 versus 5.5 +/- 0.8 IU/L; P: < 0.001), but their response to anemia was inappropriately low compared with IDA children (EPO, 94.6 +/- 15.1 IU/L) despite similar Hb concentrations. EPO therapy for 4 to 9 months in 6 NS-A children with Hb levels less than 9 g/dL led to resolution of the anemia. In conclusion, anemia is a common feature of persistent NS that develops before the deterioration of kidney function. Depletion of iron stores may contribute to the development of anemia, but iron replacement therapy is ineffective. Nephrotic patients have EPO deficiency with a blunted response to anemia. The EPO deficiency is amenable to EPO therapy, which is recommended for this group of patients.
少数病例报告中描述了持续性肾病综合征（NS）中的贫血情况，但尚未进行系统研究。我们报告一组19例在肾功能恶化前就已出现贫血的NS患儿。我们研究的目的是确定促红细胞生成素（EPO）和/或铁缺乏是否为致病因素，并评估EPO替代治疗的效果。对贫血的肾病患者（NS-A）测定血清EPO水平、铁状态和维生素B12浓度，并与血红蛋白（Hb）水平正常的肾病患儿（NS-NHb；n = 13）进行比较。两个对照组由年龄匹配的无肾脏疾病或低氧血症的患者组成，分别为缺铁性贫血（IDA；n = 19）或Hb浓度正常（NHb；n = 16）。大多数NS-A患者经历持续性激素抵抗性NS，而大多数NS-NHb患儿有激素反应性NS。虽然NS-A患儿的血清铁、铁蛋白和B12水平显著较低，但使铁蛋白和/或钴胺素水平恢复正常的适当替代治疗并未纠正贫血。NS-A患者的EPO水平高于无贫血者（21.6±3.3对5.5±0.8 IU/L；P：<0.001），但尽管Hb浓度相似，与IDA患儿相比，他们对贫血的反应却异常低（EPO，94.6±15.1 IU/L）。对6例Hb水平低于9 g/dL的NS-A患儿进行4至9个月的EPO治疗后贫血得到缓解。总之，贫血是持续性NS在肾功能恶化前就出现的常见特征。铁储备耗竭可能导致贫血的发生，但铁替代治疗无效。肾病患者存在EPO缺乏且对贫血反应迟钝。EPO缺乏适合EPO治疗，建议对该组患者进行EPO治疗。
