Developmental pattern of splenic dysfunction in sickle cell disorders.
Developmental pattern of splenic dysfunction in sickle cell disorders.
复制标题
镰状细胞病中脾功能障碍的发育模式。
DOI：
10.1542/peds.76.3.392
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发表时间：
1985
期刊：
影响因子：
8
通讯作者：
A. Ritchey
中科院分区：
文献类型：
--
作者：
Howard A. Pearson;Diane Gallagher;Robert R. Chilcote;Edmund Sullivan;Judith A. Wilimas;M. Espeland;A. Ritchey
评估镰状血红蛋白病综合征的脾功能，以确定脾功能障碍的发育模式。使用锝-99亚稳态（99mTc）脾脏扫描显示脾脏不可见与袋状（囊状）红细胞大于或等于3.5%密切相关。对来自2086例患者的口袋红细胞数据的横断面分析显示，几种疾病之间脾功能障碍的发展模式存在差异。在血红蛋白SS疾病（镰状细胞性贫血）和血红蛋白S β(0)地中海贫血中，脾功能障碍（大于或等于3.5%的红细胞袋化）通常发生在生命的前6至12个月。在血红蛋白S β(+)地中海贫血中，脾功能障碍发生的频率较低，时间较晚。血红蛋白SC病（镰状细胞-血红蛋白C）的脾功能障碍为中度。红细胞袋化水平与胎儿血红蛋白呈负相关（P < 0.05）。007)，并与年龄直接相关（P <或等于0.001）。这些脾功能障碍的模式反映了已知的溶血和血管内镰状病变的严重程度，并且与这些疾病中严重的细菌性脑膜炎和败血症的流行病学一致。连续测量袋状红细胞可以确定脾功能障碍的发生和对严重细菌感染的易感性增加的时间。
Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs greater than or equal to 3.5%. Cross-sectional analysis of pocked RBC data from 2,086 patients showed differences in the developmental pattern of splenic dysfunction between several disorders. In hemoglobin SS disease (sickle cell anemia) and hemoglobin S beta(0) thalassemia, splenic dysfunction (greater than or equal to 3.5% pocked RBCs) often occurred in the first 6 to 12 months of life. In hemoglobin S beta(+) thalassemia, splenic dysfunction occurred less frequently and later. Splenic dysfunction in hemoglobin SC disease (sickle cell-hemoglobin C) was intermediate. The level of pocked RBCs was inversely associated with fetal hemoglobin (P less than .007) and directly associated with age (P less than or equal to .001). These patterns of splenic dysfunction reflect the known severity of hemolysis and intravascular sickling and are consistent with the epidemiology of severe bacterial meningitis and sepsis in these diseases. Serial measurement of pocked RBCs permits determination of the onset of splenic dysfunction and the time of increased susceptibility to severe bacterial infections.