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骨骼肌标本的组织形态学检查无法区分恶性高热易感患者和正常患者。

Histomorphologic examination of skeletal muscle preparations does not differentiate between malignant hyperthermia-susceptible and -normal patients.

作者信息

von Breunig Franziska, Wappler Frank, Hagel Christian, von Richthofen Verena, Fiege Marko, Weisshorn Ralf, Stavrou Dimitrios, Schulte am Esch Jochen

机构信息

Department of Anesthesiology, University Hospital Hamburg-Eppendorf, Hamburg, Germany.

出版信息

Anesthesiology. 2004 Apr;100(4):789-94. doi: 10.1097/00000542-200404000-00007.

Abstract

BACKGROUND

It has been suggested that malignant hyperthermia (MH) can be diagnosed by specific myopathologic alterations. The purpose of this study was to investigate whether there are characteristic myopathologic changes in skeletal muscles of MH-susceptible (MHS) compared with MH-normal (MHN) patients.

METHODS

Four hundred forty patients with clinical suspicion of MH were classified as MHN, MH equivocal (MHE), or MHS by the in vitro contracture test with halothane and caffeine. In addition, a small muscle sample excised from each patient was analyzed by histologic, histochemical, immunohistochemical, and computer-aided morphometric methods.

RESULTS

MHN was diagnosed in 243 patients, MHE was diagnosed in 65, and MHS was diagnosed in 132. No myopathologic abnormalities were found in 53.5% of the MHN, 53.9% of the MHE, and 56.1% of the MHS patients. Thirty-five percent of all patients showed one, 9.8% showed two, and only 0.9% showed three different pathologic findings within skeletal muscle preparations. The frequency of pathologic findings did not differ between the MHN and the MHS patients; only fiber type I predominance was observed more often in MHN. MHE patients could not be assigned to a diagnostic group by detection of myopathologic alterations. In six clinically unaffected patients, a former unrecognized myopathy, such as central core disease, was diagnosed. This disease is characterized by a specific alteration (cores).

CONCLUSIONS

Histologic differences between MHS and MHN statuses could not be demonstrated in this study. Histopathologic examinations can neither improve the diagnosis of MH nor contribute to a better definition of the MH status. However, histopathologic examinations might be useful to detect formerly unrecognized specific myopathies.

摘要

背景

有人提出恶性高热(MH)可通过特定的肌病理改变来诊断。本研究的目的是调查与MH正常(MHN)患者相比,MH易感性(MHS)患者的骨骼肌是否存在特征性的肌病理变化。

方法

440例临床怀疑患有MH的患者通过氟烷和咖啡因的体外挛缩试验被分类为MHN、MH疑似(MHE)或MHS。此外,对从每位患者切除的小块肌肉样本进行组织学、组织化学、免疫组织化学和计算机辅助形态计量学分析。

结果

243例患者被诊断为MHN,65例为MHE,132例为MHS。在53.5%的MHN患者、53.9%的MHE患者和56.1%的MHS患者中未发现肌病理异常。所有患者中有35%在骨骼肌标本中表现出一种病理发现,9.8%表现出两种,只有0.9%表现出三种不同的病理发现。MHN和MHS患者之间病理发现的频率没有差异;仅在MHN患者中更常观察到I型纤维优势。通过检测肌病理改变,无法将MHE患者归入诊断组。在6例临床未受影响的患者中,诊断出一种以前未被认识的肌病,如中央轴空病。这种疾病的特征是特定的改变(轴空)。

结论

本研究未证实MHS和MHN状态之间的组织学差异。组织病理学检查既不能改善MH的诊断,也无助于更好地定义MH状态。然而,组织病理学检查可能有助于检测以前未被认识的特定肌病。

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