Khatib Rana, Rabah Raja, Sarnaik Sharada A
Children's Hospital of Michigan, Carmen and Ann Adams Department of Pediatrics, Wayne State University School of Medicine, 3901 Beaubien Blvd., Detroit, MI 48201, USA.
Pediatr Radiol. 2009 Jan;39(1):17-22. doi: 10.1007/s00247-008-1049-9. Epub 2008 Nov 11.
In early life, patients with sickle cell disease (SCD) can have acute, life-threatening emergencies related to splenic hypofunction (overwhelming bacterial sepsis), as well as anemic crises from acute splenic sequestration because of sudden pooling of blood in the spleen. The landmark penicillin prophylaxis study in 1985 showed a remarkable decrease in mortality from sepsis in young children with SCD who were treated with oral penicillin prophylaxis compared to placebo. Since that study, newborns are screened for SCD and placed on oral penicillin prophylaxis in nearly all of the United States, as well as in other countries where the disease is highly prevalent. The previously described permanent, complete and nearly universal "autosplenectomy" emerging by late childhood or early adulthood is now challenged by recent findings of reversibility of splenic dysfunction by the antisickling drug hydroxyurea or by successful allogeneic stem cell transplantation, even in older patients. Imaging techniques for hypofunction of the spleen are the most commonly used modalities to guide the clinician in decisions regarding medical or surgical management.
在生命早期，镰状细胞病（SCD）患者可能会出现与脾功能减退相关的急性、危及生命的紧急情况（暴发性细菌败血症），以及由于脾脏突然充血导致急性脾滞留引起的贫血危象。1985年具有里程碑意义的青霉素预防性研究表明，与安慰剂相比，接受口服青霉素预防性治疗的SCD幼儿败血症死亡率显著降低。自该研究以来，美国几乎所有地区以及其他该疾病高发国家的新生儿都接受了SCD筛查并开始口服青霉素预防性治疗。先前描述的在儿童晚期或成年早期出现的永久性、完全性且几乎普遍存在的“自体脾切除”，现在受到了抗镰状细胞药物羟基脲或成功的异基因干细胞移植可使脾功能障碍逆转这一最新发现的挑战，即使是老年患者也是如此。脾脏功能减退的成像技术是指导临床医生进行药物或手术治疗决策时最常用的方法。
