REFERENCE VALUES AND HEMATOLOGIC CHANGES FROM BIRTH TO 5 YEARS IN PATIENTS WITH SICKLE-CELL-DISEASE
REFERENCE VALUES AND HEMATOLOGIC CHANGES FROM BIRTH TO 5 YEARS IN PATIENTS WITH SICKLE-CELL-DISEASE
复制标题
DOI：
10.1001/archpedi.1994.02170080026005
复制
发表时间：
1994-08-01
影响因子：
--
通讯作者：
WACLAWIW, MA
中科院分区：
文献类型：
--
作者：
BROWN, AK;SLEEPER, LA;WACLAWIW, MA
目的：探讨新生儿和儿童镰状细胞病从出生到5岁的血液学变化，建立血液学参考值。研究设计：前瞻性自然史研究。背景：美国19个儿童镰状细胞中心。患者：694名患有镰状细胞病（镰状细胞性贫血、镰状细胞-血红蛋白C病和镰状-地中海贫血）的婴儿，他们在6个月以下参加了镰状细胞病的合作研究。5岁的中位随访时间为4.1年。测量和结果：我们对总血红蛋白浓度、胎儿血红蛋白百分比值、平均红细胞体积、总胆红素浓度、红细胞（RBC）、“口袋”红细胞、白细胞、血小板和网织红细胞计数进行了纵向分析。镰状细胞性贫血婴儿（SS婴儿）在出生10周时出现明显的贫血。这种贫血与网状红细胞计数上升有关，与溶血过程一致。SS患儿网织红细胞计数稳定上升，5岁时超过12%。SS患儿的胎儿血红蛋白浓度下降较镰状细胞血红蛋白C病患儿（SC患儿）慢。6个月后，袋红细胞计数急剧上升，到1岁时，28%的SS婴儿计数异常，高于3.5%，表明脾功能不佳。3岁时，78%的SS患者和32%的SC患者有异常的红细胞袋化计数。并发α -地中海贫血的SS患者在6个月后和整个儿童早期，平均总血红蛋白浓度略高于无α -地中海贫血的SS患者，平均口袋红细胞和网状红细胞计数略低于无α -地中海贫血的SS患者。SC婴儿的血液学特征与正常黑人婴儿更接近，但在儿童早期有轻度贫血（10.5 g/dL），网状红细胞（3%）和胎儿血红蛋白（3%）的平均值略有升高。
Objective: To examine hematologic changes from birth to 5 years of age and establish hematologic reference values for infants and children with sickle cell disease.Research Design: Prospective natural history study.Setting: Nineteen pediatric sickle cell centers across the United States.Patients: Six hundred ninety-four infants with sickle cell disease (sickle cell anemia, sickle cell-hemoglobin C disease, and sickle-beta-thalassemia) who were enrolled in the Cooperative Study of Sickle Cell Disease at younger than 6 months of age. Median follow-up time through 5 years of age was 4.1 years.Measurements and Results: We present longitudinal analyses of total hemoglobin concentration, percent fetal hemoglobin values, mean corpuscular volumes, total bilirubin concentration, and red blood cell (RBC), ''pocked'' RBC, white blood cell, platelet, and reticulocyte counts. Anemia was apparent by 10 weeks of life in infants with sickle cell anemia (SS infants). This anemia was associated with a rising reticulocyte count consistent with a hemolytic process. The reticulocyte count of SS infants increased steadily, exceeding 12% at 5 years of age. The fetal hemoglobin concentration of SS infants declined more slowly than that of infants with sickle cell hemo globin C disease (SC infants). Pocked RBC counts rose sharply after 6 months of age, and by 1 year, 28% of SS infants had abnormal counts, above 3.5%, indicating poor splenic function. At 3 years of age, 78% of SS patients and 32% of SC patients had abnormal pocked RBC counts. The SS patients with concurrent alpha-thalassemia had, after 6 months of age and throughout early childhood, a slightly higher mean total hemoglobin concentration and lower mean pocked RBC and reticulocyte counts than SS patients without alpha-thalassemia. The hematologic profile of SC infants more closely resembled that of normal black infants, but there was mild anemia (10.5 g/dL) and slightly elevated mean values for reticulocytes (3%) and fetal hemoglobin (3%) during early childhood.