Hussain M A, Davis L R, Laulicht M, Hoffbrand A V
Arch Dis Child. 1978 Apr;53(4):319-21. doi: 10.1136/adc.53.4.319.
In a group of 35 children with sickle cell anaemia serum ferritin concentration ranged from 70 to 2460 microgram/l (mean 367, median 180 microgram/l). This was significantly higher than the ferritin levels (range 8-101, mean 34, median 30 microgram/l) in a group of 63 normal control children of the same age group. 30 (86%) of the sickle cell children showed serum ferritin levels greater than 101 microgram/l, and 2 (6%) levels greater than 1000 microgram/l. 7 of the patients had not been transfused before this study. Their serum ferritin levels were all raised and showed a significant correlation with age but not with haemoglobin level. In the remainder of the patients the serum ferritin bore no significant correlation with age, haemoglobin level, or number of units of blood transfused. 2 children with HbSC disease had levels within the control range. Since patients with sickle cell anaemia have an increasing chance of long survival, we suggest that serial estimations of their iron status be made by means of serum ferritin assay in order to determine which patients are accumulating excessive iron.
在一组35名镰状细胞贫血患儿中,血清铁蛋白浓度范围为70至2460微克/升(平均367,中位数180微克/升)。这显著高于同一年龄组的63名正常对照儿童的铁蛋白水平(范围8 - 101,平均34,中位数30微克/升)。30名(86%)镰状细胞贫血患儿的血清铁蛋白水平高于101微克/升,2名(6%)高于1000微克/升。7名患者在本研究之前未接受过输血。他们的血清铁蛋白水平均升高,且与年龄呈显著相关,但与血红蛋白水平无关。在其余患者中,血清铁蛋白与年龄、血红蛋白水平或输血单位数均无显著相关性。2名HbSC病患儿的铁蛋白水平在对照范围内。由于镰状细胞贫血患者长期存活的机会越来越大,我们建议通过血清铁蛋白测定对他们的铁状态进行系列评估,以确定哪些患者正在蓄积过量的铁。