Ware R E, Filston H C
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina.
Surg Clin North Am. 1992 Dec;72(6):1223-36. doi: 10.1016/s0039-6109(16)45878-0.
Sickle hemoglobinopathies include sickle cell disease, sickle-C disease, and sickle-beta thalassemia. Patients with these disorders commonly suffer a multitude of destructive events to vital organs, especially to the central nervous system, the spleen, the kidney, the lung, and the heart as a result of microvascular plugging by the sickled erythrocytes. Thoughtful preparation for anesthesia and operation, especially when directed by experienced individuals, can greatly reduce the hazard of inducing the sickle crises that formerly plagued individuals with sickle hemoglobinopathies who faced major operations under general anesthesia. The patient must be free of any acute illness, especially one involving the respiratory system. Adequate hydration preoperatively combined with avoiding perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will go far toward avoiding sickle-induced complications. Modern transfusion therapy, consisting of multiple small transfusions of Hb A erythrocytes administered over several weeks prior to the operation, not only corrects the chronic anemia but suppresses erythropoiesis of cells containing Hb S in the patient's bone marrow and leaves him or her with a majority of cells containing Hb A. This provides a safety net in case a sickle-inducing insult occurs despite the best efforts to avoid one. Individuals with sickle hemoglobinopathies may require any of the operations common to all children, for example, herniorrhaphy, appendectomy, tonsillectomy, and circumcision, but a significant number will develop calcium bilirubinate cholelithiasis and possibly cholecystitis as a result of the continual increased load of bile salts resulting from the shortened lifespan of the cells containing Hb S. Also, although most individuals with Hb S will gradually suffer splenic infarction by late childhood, a significant number of infants will experience acute splenic sequestration crisis, a life-threatening entity, the recurrence of which is prevented by splenectomy. Several publications have demonstrated that such surgical procedures can be performed in large numbers of patients with sickle hemoglobinopathies without deaths and with minimal morbidity.
镰状血红蛋白病包括镰状细胞病、镰状-C病和镰状-β地中海贫血。患有这些疾病的患者通常会因镰状红细胞导致的微血管阻塞而使重要器官遭受多种破坏性病变，尤其是中枢神经系统、脾脏、肾脏、肺和心脏。精心准备麻醉和手术，尤其是在经验丰富的人员指导下进行，可大大降低诱发镰状细胞危象的风险，以前患有镰状血红蛋白病的患者在全身麻醉下接受大手术时曾深受镰状细胞危象的困扰。患者必须没有任何急性疾病，尤其是涉及呼吸系统的疾病。术前充分补液并避免围手术期缺氧、体温过低和酸中毒（这些是镰状化的触发因素），将大大有助于避免镰状细胞诱发的并发症。现代输血疗法是在手术前数周内多次少量输注血红蛋白A红细胞，这不仅能纠正慢性贫血，还能抑制患者骨髓中含血红蛋白S细胞的红细胞生成，使患者体内大多数细胞含有血红蛋白A。这提供了一个安全保障，以防尽管已尽力避免，但仍发生诱发镰状化的损伤。患有镰状血红蛋白病的个体可能需要进行所有儿童常见的任何手术，例如疝修补术、阑尾切除术、扁桃体切除术和包皮环切术，但由于含血红蛋白S细胞寿命缩短导致胆汁盐负荷持续增加，相当一部分患者会发生胆红素钙胆结石，甚至可能发展为胆囊炎。此外，尽管大多数血红蛋白S患者在童年后期会逐渐出现脾梗死，但相当多的婴儿会经历急性脾滞留危象，这是一种危及生命的情况，脾切除可预防其复发。多篇出版物表明，大量患有镰状血红蛋白病的患者可以进行此类手术，且无死亡病例，发病率极低。
