Griffin T C, McIntire D, Buchanan G R
Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas 75235-9063.
N Engl J Med. 1994 Mar 17;330(11):733-7. doi: 10.1056/NEJM199403173301101.
The management of episodes of severe pain in patients with sickle cell disease is a difficult clinical problem. We studied 36 children and adolescents with sickle cell disease who had 56 acute episodes of severe pain (44 in 27 patients with sickle cell anemia, 8 in 7 patients with sickle cell-hemoglobin C disease, and 4 in 2 patients with sickle cell-beta (+)-thalassemia). The patients were randomly assigned in double-blind fashion to receive an intravenous infusion of either saline placebo or high-dose methylprednisolone (15 mg per kilogram of body weight, to a maximum of 1000 mg) on their admission to the hospital and again 24 hours later. All the patients received intravenous morphine sulfate until severe pain abated and were then given acetaminophen with codeine.
For all episodes of pain, the duration of inpatient analgesic therapy (intravenous and oral) was significantly shorter for the patients who received methylprednisolone than for those given placebo (mean, 41.3 vs 71.3 hours; P = 0.030). The difference was still significant (31.0 vs. 62.5 hours; P = 0.010) when we excluded seven episodes that were complicated by the chest syndrome (three in the methylprednisolone group and four in the placebo group). The patients who received methylprednisolone had recurrent episodes of pain shortly after the discontinuation of therapy more often than did the patients receiving placebo. No adverse effects of methylprednisolone were observed.
A short course of high-dose methylprednisolone decreased the duration of severe pain in children and adolescents with sickle cell disease, but patients who received methylprednisolone had more rebound attacks after therapy was discontinued. On balance, corticosteroids are promising as an adjunct to supportive therapy for painful episodes in children and adolescents with sickle cell disease.
镰状细胞病患者严重疼痛发作的管理是一个棘手的临床问题。我们研究了36例患有镰状细胞病的儿童和青少年，他们共经历了56次严重疼痛急性发作（27例镰状细胞贫血患者中有44次，7例镰状细胞-血红蛋白C病患者中有8次，2例镰状细胞-β（+）-地中海贫血患者中有4次）。患者入院时及24小时后以双盲方式随机分配接受静脉输注生理盐水安慰剂或高剂量甲基强的松龙（15毫克/千克体重，最大剂量1000毫克）。所有患者均接受静脉注射硫酸吗啡直至严重疼痛缓解，随后给予对乙酰氨基酚加可待因。
对于所有疼痛发作，接受甲基强的松龙治疗的患者住院镇痛治疗（静脉和口服）的持续时间明显短于接受安慰剂的患者（平均分别为41.3小时和71.3小时；P = 0.030）。当我们排除7例并发胸部综合征的发作（甲基强的松龙组3例，安慰剂组4例）时，差异仍然显著（分别为31.0小时和62.5小时；P = 0.010）。接受甲基强的松龙治疗的患者在治疗中断后不久疼痛复发的频率高于接受安慰剂的患者。未观察到甲基强的松龙的不良反应。
短期高剂量甲基强的松龙可缩短镰状细胞病儿童和青少年严重疼痛的持续时间，但接受甲基强的松龙治疗的患者在治疗中断后有更多的反弹发作。总体而言，皮质类固醇有望作为镰状细胞病儿童和青少年疼痛发作支持治疗的辅助药物。
