West M S, Wethers D, Smith J, Steinberg M
Department of Medicine, Baylor College of Medicine, Houston, TX.
J Clin Epidemiol. 1992 Aug;45(8):893-909. doi: 10.1016/0895-4356(92)90073-v.
We have collected steady-state laboratory data for over 2600 patients, age 2 years and over, with sickle cell anemia (HbSS), HbSC disease, and HbS-beta(+)-thalassemia. The packed cell volume (PCV) is lower in males than in females until 17 or 18 years of age in HbSS and ages 13 to 15 in HbSC, but then becomes consistently higher in males. After age 40, the PCV falls in HbSS. The steady-state leukocyte count in HbSS is higher than that in normals, blunting the utility of this measurement in the assessment of infection. In HbSC and HbS-beta(+)-thalassemia, the leukocyte counts are more often within the range of normal. Platelet counts in HbSS are often found to be above normal and show a downward trend with age. There is a progressive rise in creatinine with age. In HbSS, this rise begins at age 14 and may be accounted for by the increased muscle mass that occurs with puberty. The further deterioration of renal function in patients over 20 may be a result of the known adverse effects of sickle cell disease upon the kidney. Our data provide a basis to compare perturbations caused by intercurrent complications and new therapies, as well as to contrast with similar information from other populations of patients with sickle cell disease.
我们收集了2600多名2岁及以上镰状细胞贫血（HbSS）、HbSC病和HbS-β（+）-地中海贫血患者的稳态实验室数据。在HbSS中，直到17或18岁，男性的红细胞压积（PCV）低于女性，在HbSC中则是13至15岁，但之后男性的PCV持续更高。40岁以后，HbSS中的PCV下降。HbSS中的稳态白细胞计数高于正常人，这削弱了该指标在评估感染方面的作用。在HbSC和HbS-β（+）-地中海贫血中，白细胞计数更常在正常范围内。HbSS中的血小板计数常高于正常，并随年龄呈下降趋势。肌酐水平随年龄逐渐升高。在HbSS中，这种升高始于14岁，可能是青春期肌肉量增加所致。20岁以上患者肾功能的进一步恶化可能是镰状细胞病对肾脏已知的不良影响的结果。我们的数据为比较并发并发症和新疗法引起的扰动提供了基础，也为与其他镰状细胞病患者群体的类似信息进行对比提供了基础。
