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杜氏肌营养不良症中的转氨酶升高

Transaminitis in Duchenne's muscular dystrophy.

作者信息

Tay S K, Ong H T, Low P S

机构信息

Department of Paediatrics, National University Hospital, 5 Lower Kent Ridge Road, Singapore 119074.

出版信息

Ann Acad Med Singap. 2000 Nov;29(6):719-22.

Abstract

BACKGROUND

Persistently raised transaminase levels often prompt the clinician to investigate for liver pathology. Previously, some of our patients with Duchenne's muscular dystrophy have had investigations to look for liver disorders when the alanine transaminases (ALTs) were found incidentally to be high.

AIM

The objective of the study was to ascertain the levels of the transaminases in patients with Duchenne's muscular dystrophy and to see if the levels of transaminases correlated with muscle enzymes such as creatine kinase (CK).

MATERIALS AND METHODS

This is a case series of 19 patients with Duchenne's muscular dystrophy. Alanine and aspartate transaminase (AST) levels and CK levels were measured in the serum of the patients.

RESULTS

In this series, ALT and AST levels were all found to be raised significantly in patients with biopsy-proven Duchenne's muscular dystrophy and Becker's muscular dystrophy. Alanine transaminase, in particular, was raised to a mean of 356 mmol/L, 9 times above the mean for normal. There was also good correlation between ALT and CK levels with a correlation coefficient of r = 0.80 and P value 0.01. Similar to CK, the transaminase levels were inversely proportional to mobility and to age.

CONCLUSION

The presence of hypertransaminasemia in patients with muscular dystrophy should be attributed to muscle breakdown rather than to liver pathology and such patients should not be over-investigated for liver disease. In the absence of liver pathology, raised transaminases may be an early sign of occult muscular dystrophy and such patients should have CK levels checked to look for evidence of muscular dystrophy.

摘要

背景

转氨酶水平持续升高常常促使临床医生对肝脏病变进行检查。此前,我们的一些杜氏肌营养不良症患者在偶然发现丙氨酸转氨酶(ALT)升高时,接受了肝脏疾病方面的检查。

目的

本研究的目的是确定杜氏肌营养不良症患者的转氨酶水平,并观察转氨酶水平是否与肌酸激酶(CK)等肌肉酶相关。

材料与方法

这是一个包含19例杜氏肌营养不良症患者的病例系列。检测了患者血清中的丙氨酸转氨酶、天冬氨酸转氨酶(AST)水平以及CK水平。

结果

在这个系列中,经活检证实为杜氏肌营养不良症和贝克肌营养不良症的患者,其ALT和AST水平均显著升高。尤其是丙氨酸转氨酶,平均升高至356 mmol/L,是正常均值的9倍。ALT与CK水平之间也存在良好的相关性,相关系数r = 0.80,P值为0.01。与CK相似,转氨酶水平与活动能力和年龄呈反比。

结论

肌营养不良症患者出现高转氨酶血症应归因于肌肉分解,而非肝脏病变,此类患者不应过度接受肝脏疾病方面的检查。在没有肝脏病变的情况下,转氨酶升高可能是隐匿性肌营养不良症的早期迹象,此类患者应检查CK水平以寻找肌营养不良症的证据。

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