ACUTE MULTIORGAN FAILURE SYNDROME - A POTENTIALLY CATASTROPHIC COMPLICATION OF SEVERE SICKLE-CELL PAIN EPISODES
ACUTE MULTIORGAN FAILURE SYNDROME - A POTENTIALLY CATASTROPHIC COMPLICATION OF SEVERE SICKLE-CELL PAIN EPISODES
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DOI：
10.1016/0002-9343(94)90136-8
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发表时间：
1994-02-01
影响因子：
5.9
通讯作者：
LANE, PA
中科院分区：
文献类型：
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作者：
HASSELL, KL;ECKMAN, JR;LANE, PA
本报告的目的是描述急性多器官衰竭综合征的特点，它使某些镰状疼痛发作复杂化。采用回顾性病历审查来确定镰状疼痛发作并发三个器官（肺、肝或肾）中至少两个的急性衰竭。器官功能衰竭的定义标准，并记录符合标准的发作的临床特征，实验室值，治疗方法和结局。14例患者中有17次急性多器官功能衰竭发作，其中10例为镰状细胞贫血，4例为血红蛋白SC病。大多数事件发生在患者异常严重的疼痛事件期间。器官衰竭的发生与发热、血红蛋白水平和血小板计数的快速下降、非局灶性脑病和横纹肌溶解有关。除4次发作外，细菌培养均为阴性。除一次发作外，积极的输血治疗与生存率和器官功能的快速恢复有关。急性多器官衰竭综合征是一种严重的、危及生命的并发症，在患有其他轻度镰状细胞病的患者中出现疼痛发作。该综合征似乎可以通过及时、积极的输血治疗逆转。高基线血红蛋白水平可能是一个诱发因素。
The purpose of this report is to characterize the acute multiorgan failure syndrome that complicates some episodes of sickle pain. A retrospective chart review was used to identify episodes of sickle pain complicated by the acute failure of at least two of three organs: lung, liver, or kidney. The defining criteria of organ failure were established, and the clinical characteristics, laboratory values, treatment methods, and outcomes were noted in episodes that met the criteria.Seventeen episodes of acute multiorgan failure were identified in 14 patients, 10 with sickle cell anemia and 4 with hemoglobin SC disease. Most episodes occurred during a pain event that was unusually severe for the patient. The onset of organ failure was associated with fever, rapid fall in hemoglobin level and platelet count, nonfocal encephalopathy, and rhabdomyolysis. Bacterial cultures were negative in all but four episodes. Aggressive transfusion therapy was associated with survival and with rapid recovery of organ function in all but one episode. The syndrome developed in patients who had previously exhibited relatively mild disease with little evidence of chronic organ damage and relatively high hemoglobin values in steady state,Acute multiorgan failure syndrome is a severe, life-threatening complication of pain episodes in patients with otherwise mild sickle cell disease, The syndrome appears to be reversed with prompt, aggressive transfusion therapy. High baseline hemoglobin levels may represent a predisposing factor.