[{"Title":"参考文献","UniqueID":"v89545686_zh","Index":1.0,"Summary":"

1.Centers for Disease Control and Prevention: Sickle cell disease (SCD): Data and statistics on sickle cell disease.May 15, 2024.Accessed February 6, 2026.

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在血红蛋白S中，β链第6个氨基酸上的谷氨酸被缬氨酸所取代。脱氧血红蛋白S 的溶解度比氧合血红蛋白A 低得多；在氧分压(PO2)较低的地方，它会聚合并形成一种半固体的凝胶，使红细胞(RBCs)变形成镰刀状。扭曲、缺乏弹性的红细胞粘附于血管内皮，引起炎症和血管收缩，堵塞小动脉和毛细血管，导致微血管和大血管梗塞（例如，引起中风）。血管阻塞也会引起内皮损伤，从而导致炎症并可导致血栓形成。由于镰状红细胞比较脆弱，循环中的机械损伤可引发溶血（见溶血性贫血概述）。慢性代偿性骨髓功能亢进和缺血性损伤会导致骨骼变形。

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多数症状源于：

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DNA检测（产前诊断，或在诊断不明时于出生后进行检测）

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DNA检测（产前诊断，或在诊断不明时于出生后进行检测）

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试验类型的选择取决于患者的年龄。DNA检测可用于胎儿期诊断或者明确镰状细胞病的基因类型。新生儿筛查包括血红蛋白电泳。儿童和成人筛查包括外周血涂片检查、血红蛋白溶解度试验，以及血红蛋白电泳或高效液相色谱法（HPLC）。

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","HeadType":"G_Head","SchemeItemProp":"","Children":[]},{"Title":"新生儿筛查","UniqueID":"v109277216_zh","Index":2.0,"Summary":"

","HeadType":"G_Head","SchemeItemProp":"","Children":[]},{"Title":"儿童和成人的筛查","UniqueID":"v109277219_zh","Index":3.0,"Summary":"

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广谱抗生素（针对感染）

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广谱抗生素（针对感染）

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广谱抗生素（针对感染）

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广谱抗生素（针对感染）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"吸氧","UniqueID":"v67165677_zh","Index":4.0,"Summary":"

广谱抗生素（针对感染）

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广谱抗生素（针对感染）

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广谱抗生素（针对感染）

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广谱抗生素（针对感染）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"持续管理","UniqueID":"v109277250_zh","Index":8.0,"Summary":"

广谱抗生素（针对感染）

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广谱抗生素（针对感染）

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纯合子患者的寿命已可稳定提高到 >50岁 ( 1 , 2 )。常见的死亡原因有急性胸部综合征、并发感染、肺栓塞、重要器官出现梗死、肺动脉高压以及慢性肾脏疾病。

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血红蛋白S（Hb S）纯合子患者的β链异常，导致当血红蛋白S聚合时，红细胞变得脆弱且相对僵硬；红细胞可粘附于血管内皮，引起血管收缩和炎症，导致组织梗死，并且容易发生溶血，从而引起贫血。

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