Guy's, King's & St Thomas' Medical School, King's College London, London, UK.
Int J Infect Dis. 2010 Jan;14(1):e2-e12. doi: 10.1016/j.ijid.2009.03.010. Epub 2009 Jun 3.
Infection is a significant contributor to morbidity and mortality in sickle cell disease (SCD). The sickle gene confers an increased susceptibility to infection, especially to certain bacterial pathogens, and at the same time infection provokes a cascade of SCD-specific pathophysiological changes. Historically, infection is a major cause of mortality in SCD, particularly in children, and it was implicated in 20-50% of deaths in prospective cohort studies over the last 20 years. Worldwide, it remains the leading cause of death, particularly in less developed nations. In developed countries, measures to prevent and effectively treat infection have made a substantial contribution to improvements in survival and quality of life, and are continually being developed and extended. However, progress continues to lag in less developed countries where the patterns of morbidity and mortality are less well defined and implementation of preventive care is poor. This review provides an overview of how SCD increases susceptibility to infections, the underlying mechanisms for susceptibility to specific pathogens, and how infection modifies the outcome of SCD. It also highlights the challenges in reducing the global burden of mortality in SCD.
感染是导致镰状细胞病（SCD）发病率和死亡率升高的重要因素。镰状基因使患者更容易感染，尤其是某些细菌病原体，同时感染会引发一系列 SCD 特异性的病理生理变化。在历史上，感染是 SCD 患者，尤其是儿童患者死亡的主要原因，在过去 20 年的前瞻性队列研究中，感染导致了 20-50%的死亡。在全球范围内，感染仍然是导致死亡的主要原因，尤其是在欠发达国家。在发达国家，预防和有效治疗感染的措施为提高生存率和生活质量做出了巨大贡献，并且还在不断发展和推广。然而，在欠发达国家，进展仍然滞后，这些国家的发病率和死亡率模式不太明确，预防保健的实施情况较差。本文综述了 SCD 如何增加感染易感性、对特定病原体易感性的潜在机制，以及感染如何改变 SCD 的结局，并强调了降低 SCD 全球死亡率负担所面临的挑战。
