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瑞典遗传性转甲状腺素蛋白淀粉样变性(ATTR)患者肝移植后心肌病的发生。

Development of cardiomyopathy after liver transplantation in Swedish hereditary transthyretin amyloidosis (ATTR) patients.

机构信息

Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.

出版信息

Amyloid. 2011 Dec;18(4):200-5. doi: 10.3109/13506129.2011.615872.

Abstract

BACKGROUND

Recent studies of liver transplanted (LTx) familial amyloidotic polyneuropathy (FAP) patients have shown a progression of cardiomyopathy in some patients after LTx, but knowledge of the underlying factors remains limited.

METHODS

Seventy-five patients, who had undergone LTx from 1996 to 2008, were included. They had all been examined by echocardiography 1-16 months before LTx. Fifty-four had been re-examined 7-34 months, and forty-two 36-137 months after LTx.

RESULTS

A significant increase in interventricular septum (IVS) thickness occurred after LTx (p < 0.01), particularly in males (p = 0.002) and late onset patients (p = 0.003). The development of post-LTx cardiomyopathy was related to patient's age at onset of the disease, male gender and pre-LTx IVS thickness. On multivariate regression analysis, however, age at onset was the only significant predictor for the development of cardiomyopathy (odds ratio = 1.14, 95% confident interval 1.01-1.30, p = 0.04).

CONCLUSION

An increase of IVS thickness can be observed in FAP patients after LTx. Age at onset of the disease is the main predictor for increased IVS thickness and for the development of cardiomyopathy after liver transplantation.

摘要

背景

最近对肝移植(LTx)家族性淀粉样变性多发性神经病(FAP)患者的研究表明,一些患者在 LTx 后会出现心肌病进展,但对潜在因素的了解仍然有限。

方法

纳入了 1996 年至 2008 年间接受 LTx 的 75 例患者。他们在 LTx 前 1-16 个月均接受了超声心动图检查。54 例在 LTx 后 7-34 个月时复查,42 例在 36-137 个月时复查。

结果

LTx 后室间隔(IVS)厚度显著增加(p<0.01),尤其是男性(p=0.002)和发病晚的患者(p=0.003)。LTx 后发生心肌病与患者发病年龄、性别和 LTx 前 IVS 厚度有关。然而,多变量回归分析显示,发病年龄是发生心肌病的唯一显著预测因素(优势比=1.14,95%置信区间 1.01-1.30,p=0.04)。

结论

FAP 患者在 LTx 后可观察到 IVS 厚度增加。发病年龄是 IVS 厚度增加和 LTx 后发生心肌病的主要预测因素。

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