Phebus C K, Maciak B J, Gloninger M F, Paul H S
Division of Hematology/Oncology, University of Pittsburgh School of Medicine, Graduate School of Public Health, University of Pittsburgh, PA.
Am J Hematol. 1988 Oct;29(2):67-73. doi: 10.1002/ajh.2830290203.
We examined the zinc status of 80 children with sickle cell disease (SCD) and 44 disease-free sibling controls aged 3 to 18 years. For both patients and controls, variations in serum zinc by age, type of hemoglobinopathy, and growth status were measured. The mean serum zinc concentration of patients was significantly lower than for controls (77.8 +/- 9.9 vs. 82.2 +/- 9.8 micrograms/dl, mean +/- 1SD, P less than .05). Serum levels of alkaline phosphatase (AP) and retinol-binding protein (RBP), two zinc-dependent proteins, were also lower among patients (AP: 171 +/- 66 vs. 243 +/- 97 IU/L, P less than .001; RBP: 1.92 +/- .9 vs. 2.77 +/- .9 mg/dl, P less than .001). Patients greater than or equal to 12 years of age (n = 34) had significantly lower zinc levels than those less than 12 years (74.5 +/- 8.4 vs. 80.3 +/- 10.3 micrograms/dl, P less than .01), and children with homozygous SCD (Hb SS, n = 55) had a more pronounced deficiency than those with a variant hemoglobinopathy (76.3 +/- 8.9 vs. 81.5 +/- 11.5, micrograms/dl, P less than .05). Patients classified as having "poor" growth (height-for-age less than 5th percentile, n = 24) had a lower serum zinc level than those with "normal" growth (72.8 +/- 8.0 vs. 79.8 +/- 10.0 micrograms/dl, P less than .01). Dietary intake data, body mass index, and serum total protein and albumin levels were similar for patients and controls, suggesting that zinc deficiency in SCD does not relate to inadequate dietary intake. The origin of low serum zinc levels in children with SCD is more likely to relate to factors such as increased urinary zinc excretion, chronic intravascular hemolysis, and/or zinc malabsorption.
我们对80名患有镰状细胞病(SCD)的儿童以及44名年龄在3至18岁、未患该病的同胞对照者的锌状态进行了检查。对于患者和对照者,均测量了血清锌随年龄、血红蛋白病类型及生长状态的变化情况。患者的平均血清锌浓度显著低于对照者(分别为77.8±9.9与82.2±9.8微克/分升,均值±1标准差,P<0.05)。两种锌依赖性蛋白——碱性磷酸酶(AP)和视黄醇结合蛋白(RBP)的血清水平在患者中也较低(AP:分别为171±66与243±97国际单位/升,P<0.001;RBP:分别为1.92±0.9与2.77±0.9毫克/分升,P<0.001)。年龄大于或等于12岁的患者(n = 34)的锌水平显著低于12岁以下的患者(分别为74.5±8.4与80.3±10.3微克/分升,P<0.01),并且纯合SCD(Hb SS,n = 55)患儿的锌缺乏比那些患有变异血红蛋白病的患儿更为明显(分别为76.3±8.9与81.5±11.5微克/分升,P<0.05)。被归类为“生长不良”(年龄别身高低于第5百分位数,n = 24)的患者血清锌水平低于“生长正常”的患者(分别为72.8±8.0与79.8±10.0微克/分升,P<0.01)。患者和对照者的饮食摄入数据、体重指数以及血清总蛋白和白蛋白水平相似,这表明SCD中的锌缺乏与饮食摄入不足无关。SCD患儿血清锌水平低的原因更可能与尿锌排泄增加、慢性血管内溶血和/或锌吸收不良等因素有关。