Sagi Varun, Song-Naba Waogwende L, Benson Barbara A, Joshi Sonal S, Gupta Kalpna
Vascular Biology Center, Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota.
Curr Protoc Neurosci. 2018 Oct;85(1):e54. doi: 10.1002/cpns.54. Epub 2018 Sep 28.
Sickle cell disease (SCD) is a genetic blood disorder that impacts millions of individuals worldwide. SCD is characterized by debilitating pain that can begin during infancy and may continue to increase throughout life. This pain can be both acute and chronic. A characteristic feature specific to acute pain in SCD occurs during vaso-occlusive crisis (VOC) due to the blockade of capillaries with sickle red blood cells. The acute pain of VOC is intense, unpredictable, and requires hospitalization. Chronic pain occurs in a significant population with SCD. Treatment options for sickle pain are limited and primarily involve the use of opioids. However, long-term opioid use is associated with numerous side effects. Thus, pain management in SCD remains a major challenge. Humanized transgenic mice expressing exclusively human sickle hemoglobin show features of pain and pathobiology similar to that in patients with SCD. Therefore, these mice offer the potential for investigating the mechanisms of pain in SCD and allow for development of novel targeted analgesic therapies. © 2018 by John Wiley & Sons, Inc.
镰状细胞病（SCD）是一种遗传性血液疾病，影响着全球数百万人。SCD的特点是使人衰弱的疼痛，这种疼痛可能在婴儿期就开始出现，并可能在一生中持续加剧。这种疼痛可以是急性的，也可以是慢性的。SCD急性疼痛的一个特征性表现发生在血管阻塞性危机（VOC）期间，此时镰状红细胞会阻塞毛细血管。VOC的急性疼痛剧烈、不可预测，需要住院治疗。慢性疼痛在大量SCD患者中出现。镰状疼痛的治疗选择有限，主要涉及使用阿片类药物。然而，长期使用阿片类药物会带来许多副作用。因此，SCD的疼痛管理仍然是一个重大挑战。仅表达人类镰状血红蛋白的人源化转基因小鼠表现出与SCD患者相似的疼痛和病理生物学特征。因此，这些小鼠为研究SCD疼痛机制提供了潜力，并有助于开发新型靶向镇痛疗法。© 2018约翰威立国际出版公司
