肌无力危机Vs胆碱能危机:没有血浆置换或静脉注射免疫球蛋白(IVIG)的危机管理的挑战

Lila Tri Harjana, Hardiono Hardiono
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引用次数: 1

摘要

简介:重症肌无力(Myasthenia gravity, MG)是一种获得性自身免疫性疾病,临床表现为骨骼肌无力和运动疲劳,发病率高达2-7 / 10000,女性发病频率高于男性(~3:2)。超过12-16%的广泛性MG患者一生中经历过一次危象。重症肌无力的一个严重并发症是呼吸衰竭。这可能是继发于重症肌无力加重(重症肌无力危象)或过量使用胆碱酯酶抑制剂(胆碱能危象)治疗。病例报告:32岁女性患者在重症监护室治疗9天后,因重症肌无力危象从私立医院转至急诊科接受进一步治疗。患者已插管机械通气,有大剂量多种抗胆碱酯酶药物和类固醇治疗史,无血浆置换或免疫球蛋白静脉注射。入院时出现腹泻,无胃肠道感染迹象。入院第三天,患者进行了自主呼吸试验,成功后拔管。拔管2天后,患者出现呼吸衰竭,需要机械通气。行抗胆碱酯酶试验,临床症状无改善,诊断为胆碱能危象。经重新调整低剂量抗胆碱酯酶药物后,临床呼吸情况好转,入院第10天成功拔管。入院第12天,患者出院。讨论:重症肌无力和胆碱能危象是一种严重的危及生命的疾病,其特征是全身性肌肉无力,呼吸系统受损,需要通气支持。胆碱能危象时可出现呼吸衰竭,但无胆碱能症状(贫血、腹泻、尿失禁、心动过缓、呕吐、流泪或流涎)。重症肌无力危象患者最重要的管理方面是对重症肌无力与胆碱能危象的认识和治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Myasthenia Crisis Vs Cholinergic Crisis: Challenges in Crisis Management Without Plasmapheresis or Intravenous Immunoglobulin (IVIG)
Introduction: Myasthenia gravis (MG) is an acquired autoimmune disorder clinically characterized by skeletal muscle weakness & fatigability on exertion with prevalence as high as 2–7 in 10,000 and women are affected more frequently than men (~3:2). Over 12-16% of generalized MG patients experience crisis once in their lifetime. A serious complication of myasthenia gravis is respiratory failure. This may be secondary to an exacerbation of myasthenia (myasthenia crisis) or to treatment with excess doses of a cholinesterase inhibitor (cholinergic crisis). Case Report: Thirty-two years old woman refereed from a private hospital to ED for further treatment with myasthenia in crisis, after nine days of treatment in the previous ICU. Patient already in intubation with mechanical ventilation and history of the treatment of a high dose of multiple anticholinesterase drugs and steroids without plasmapheresis or immunoglobulin intravenous. During admission, diarrhea was present, with no sign of GI infection. On the third day of admission, the patient performed a Spontaneous Breathing Trial and was a success then extubated. Then two day after extubation, the patient falls to respiratory failure and need mechanical ventilation. Anticholinesterase test was performed, and it shows no improvement in clinical signs, and diagnose as Cholinergic Crisis. After re-adjustment of anticholinesterase drug with a lower dose, clinically, the respiratory condition improved, and on the 10th day of admission, the patient was succeed extubated. At 12nd days of ICU admission, patient discharge from ICU. Discussion: Myasthenia and Cholinergic Crisis is a severe and life-threatening condition characterized by generalized muscle weakness with a respiratory compromise that requires ventilatory support. Respiratory failure may be present in the cholinergic crisis without cholinergic symptoms (miosis, diarrhea, urinary incontinence, bradycardia, emesis, lacrimation, or salivation). The most important management aspect of Myasthenia patients in crisis is the recognition and treatment of myasthenia vs cholinergic crisis.
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