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库欣综合征中肌病的持续存在:德国库欣登记处的评估

Persistence of myopathy in Cushing's syndrome: evaluation of the German Cushing's Registry.

作者信息

Berr Christina M, Stieg Mareike R, Deutschbein Timo, Quinkler Marcus, Schmidmaier Ralf, Osswald Andrea, Reisch Nicole, Ritzel Katrin, Dimopoulou Christina, Fazel Julia, Hahner Stefanie, Stalla Günter K, Beuschlein Felix, Reincke Martin

机构信息

Medizinische Klinik und Poliklinik IVKlinikum der Ludwig-Maximilians-Universität München, Munich, Germany.

RG NeuroendocrinologyMax Planck Institute of Psychiatry, Munich, Germany.

出版信息

Eur J Endocrinol. 2017 Jun;176(6):737-746. doi: 10.1530/EJE-16-0689. Epub 2017 Mar 21.

DOI:10.1530/EJE-16-0689
PMID:28325824
Abstract

BACKGROUND

Cushing's syndrome (CS) is characterized by an excessive secretion of glucocorticoids that results in a characteristic clinical phenotype. One feature of clinical hypercortisolism is breakdown of protein metabolism translating into clinical consequences including glucocorticoid-induced myopathy. While surgery is effective in control of cortisol excess, the effect of biochemical remission on muscular function is yet unclear.

METHODS

In a cross-sectional study we analyzed 47 patients with CS during the florid phase (ActiveCS). 149 additional patients were studied 2-53 years (mean: 13 years) after surgery in biochemical long-term remission (RemissionCS). Also, 93 rule-out CS patients were used as controls (CON). All subjects were assessed for grip strength using a hand grip dynamometer and underwent the chair rising test (CRT).

RESULTS

Hand grip strength (85% vs 97% of norm,  = 0.002) and the CRT performance (9.5 s vs 7.1 s,  = 0.001) were significantly lower in ActiveCS compared to the CON group. Six months after treatment grip strength further decreased in CS ( = 0.002) and CRT performance remained impaired. The RemissionCS group (mean follow-up 13 years) had reduced hand grip strength (92% compared to normal reference values for dominant hand,  < 0.001). The chair rising test performance was at 9.0 s and not significantly different from the ActiveCS group ( = 0.45).

CONCLUSION

CS affects muscle strength in the acute phase, but functional impairment remains detectable also during long-term follow-up despite biochemical remission.

摘要

背景

库欣综合征(CS)的特征是糖皮质激素分泌过多,导致典型的临床表型。临床皮质醇增多症的一个特征是蛋白质代谢分解,产生包括糖皮质激素诱导的肌病在内的临床后果。虽然手术对控制皮质醇过多有效,但生化缓解对肌肉功能的影响尚不清楚。

方法

在一项横断面研究中,我们分析了47例处于急性期的CS患者(活动期CS)。另外149例患者在生化长期缓解(缓解期CS)的手术后2至53年(平均13年)接受了研究。此外,93例排除CS的患者用作对照(CON)。所有受试者均使用握力计评估握力,并进行坐椅起立试验(CRT)。

结果

与CON组相比,活动期CS患者的握力(为正常的85% 对97%,P = 0.002)和CRT表现(9.5秒对7.1秒,P = 0.001)显著降低。治疗6个月后,CS患者的握力进一步下降(P = 0.002),CRT表现仍受损。缓解期CS组(平均随访13年)的握力降低(优势手与正常参考值相比为92%,P < 0.001)。坐椅起立试验表现为9.0秒,与活动期CS组无显著差异(P = 0.45)。

结论

CS在急性期会影响肌肉力量,但尽管生化缓解,在长期随访中仍可检测到功能损害。

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