Roizen N J, Amarose A P
Department of Pediatrics, University of Chicago Pritzker School of Medicine, Wyler Children's Hospital, La Rabida Children's Hospital and Research Center, Chicago, Illinois.
Am J Med Genet. 1993 Jun 15;46(5):510-2. doi: 10.1002/ajmg.1320460509.
We compared the hematologic parameters of 18 otherwise healthy children with Down syndrome (DS) in the age range of 2-6 years to those of 18 healthy non-DS controls matched for age and gender. The children with DS had MCVs and hematocrits increased significantly compared to controls and decreased WBCs compared to controls; 66% of the children with DS compared to 11% of non-DS controls had MCVs greater than the 97th percentile for age (P < 0.0001); the mean MCVs were 86.9 and 80.6, respectively. Although hematocrits were within normal limits for age for all DS and non-DS subjects, the DS patients had significantly higher hematocrits (39.1% vs. 36.9%, P < 0.014). We also found that 33% of the children had WBCs < 5% for age compared to 6% of controls. To determine whether folate deficiency contributed to these observations, we measured serum and RBC folate concentrations: these were not significantly different between the 2 groups. We conclude that macrocytosis and leukopenia are common in children with DS.
我们将18名年龄在2至6岁、无其他健康问题的唐氏综合征（DS）患儿的血液学参数与18名年龄和性别相匹配的健康非DS对照儿童的参数进行了比较。与对照组相比，DS患儿的平均红细胞体积（MCV）和血细胞比容显著升高，白细胞计数（WBC）降低；66%的DS患儿的MCV高于年龄的第97百分位数，而非DS对照组中这一比例为11%（P<0.0001）；平均MCV分别为86.9和80.6。尽管所有DS和非DS受试者的血细胞比容在年龄正常范围内，但DS患者的血细胞比容显著更高（39.1%对36.9%，P<0.014）。我们还发现，33%的患儿的WBC低于年龄的第5百分位数，而对照组中这一比例为6%。为了确定叶酸缺乏是否导致了这些观察结果，我们测量了血清和红细胞叶酸浓度：两组之间无显著差异。我们得出结论，大细胞性贫血和白细胞减少在DS患儿中很常见。
