[先天性假性胆碱酯酶缺乏症]

[Congenital pseudocholinesterase deficiency].

作者信息

Gelo Remigiusz, Jurek-Gelo Anna, Wardaszko Ryszard, Kański Andrzej

机构信息

II Klinika Anestezjologii i Intensywnej Terapii, Warszawski Uniwersytet Medyczny.

出版信息

Anestezjol Intens Ter. 2011 Jan-Mar;43(1):33-5.

PMID:21786528

Abstract

BACKGROUND

Congenital pseudocholinesterase (pChe) deficiency is a rare genetic abnormality which may lead to prolonged duration of action of muscle relaxants that are hydrolysed by pChe. We describe two cases in which mivacurium resulted in neuromuscular block lasting several hours.

CASE REPORTS

Two non-related male patients, aged 26 and 7 years, scheduled for elective ENT surgery, received propofol, desflurane, remifentanil and mivacurium. At the end of the surgery it was not possible to reverse the neuromuscular blockade, and there were no responses to TOF or post-tetanic stimulation. Neuromuscular transmission returned spontaneously after 7, and 4 h, respectively. Postoperative assay revealed severe pChe deficiency in both patients, with values of 3393 UL(-1)and 2558 UL(-1), respectively (normal range 5100-11700 UL(-1). Family screening confirmed the presence of pChe deficiency in both cases.

CONCLUSION

In any case of unexpected prolonged muscle relaxation after mivacurium, pChe deficiency should be considered and its activity measured.When confirmed, careful family screening is mandatory.

摘要

背景

先天性假性胆碱酯酶（pChe）缺乏是一种罕见的遗传异常，可导致由pChe水解的肌肉松弛剂作用时间延长。我们描述了两例米库氯铵导致神经肌肉阻滞持续数小时的病例。

病例报告

两名非亲属男性患者，年龄分别为26岁和7岁，计划进行择期耳鼻喉科手术，接受了丙泊酚、地氟醚、瑞芬太尼和米库氯铵。手术结束时，无法逆转神经肌肉阻滞，对四个成串刺激（TOF）或强直后刺激无反应。神经肌肉传递分别在7小时和4小时后自发恢复。术后检测显示两名患者均存在严重的pChe缺乏，其值分别为3393 U/L（-1）和2558 U/L（-1）（正常范围5100 - 11700 U/L（-1））。家族筛查证实两例均存在pChe缺乏。