慢性腹膜透析患者和血液透析患者類似，常因長期透析而導致肉質素失調(dialysis related carnitine disorder, DCD)，血漿中游離型左旋肉質素(free L-carnitine)下降，醯化肉質素(acyl-carnitine)/游離型肉質素的比值上升，而長期透析導致肉質素失調通常會伴隨尿毒症肌肉病變(uremic myopathy)，尿毒症肌肉病變會限制透析患者運動耐力及持久力(endurance)，其臨床表現為肌肉痙攣/疼痛或肌肉無力/疲勞，已有報告顯示，血液透析患者每天口服補充低劑量左旋肉質素(500mg)，可降低肌肉症狀，且降低醯化肉質素/游離型肉質素比值。
　　我們進行六個月前瞻性開放式平行試驗，評估腹膜透析患者每天口服600mg左旋肉質素其肌肉症狀之變化，在進入研究前及第三、六個月，以標準無刻度100 mm視覺類比評量尺(visual analog scale, VAS)讓病患作肌肉症狀自覺評估，評估項目包括：肌肉痙攣/疼痛、肌肉無力/疲勞以及非肌肉痙攣引起之肌肉酸痛，另外，評估病患生活品質之變化，於研究前後使用36項生活品質評估表(36-item short-form health survey, SF-36)進行評估，並監測病患紅血球生成素(epoietin, rhuEPO)之治療反應、心臟射出分率（EF值）和心衰竭之症狀。
　　本研究中，一共有45位尿毒症肌肉病變之腹膜透析患者，分為實驗組(n=22)和對照組(n=23)，先以100mm的視覺類比評量尺評估肌肉痙攣/疼痛症狀，在基準期兩組間並無差異，經過六個月治療後，實驗組較對照組有改善症狀之趨勢(overall P = 0.05)；肌肉無力/疲勞症狀方面，在基準值兩組間無差異，經六個月實驗組較對照組呈現明顯改善(overall P = 0.02)；兩者症狀的改善與完整型副甲狀腺素、鐵質狀態、全身肌肉量並無相關。使用SF-36進行生活品質評估，經過六個月，實驗組較對照組在身體功能、身體角色、一般健康、和情緒角色四個面項有明顯改善(P < 0.05)，對於紅血球生成素之治療反應、心臟射出分率和心衰竭之症狀，服用左旋肉質素並無觀察到明顯反應。
　　因此由本實驗發現慢性腹膜透析患者口服補充左旋肉質素，耐受性不錯，未出現嚴重副作用，且服用六個月後可明顯改善肌肉症狀和增加生活品質。

　　Uremic patients receiving chronic peritoneal dialysis (PD), similar to hemodialysis patients, were associated with a reduction in plasma and tissue free carnitine concentration and high ratio of acyl-carnitine to free carnitine (AC/FC). The disturbed L-carnitine concentrations were referred to as dialysis–related carnitine deficiency which was associated with symptoms of uremic myopathy. Uremic myopathy frequently manifested with muscle weakness/fatigue, cramps/pain, and limited exercise tolerance and endurance. Oral low-dose supplement with L-carnitine had been shown benefits in alleviating skeletal muscle symptoms and increased physical activity by reducing AC/FC ratio in hemodialysis patients.
　　We performed a prospective open-label parallel controlled study to evaluate the effects of oral L-carnitine (600 mg/day) supplementation for six months on muscle symptoms and quality of life. Three components of muscle symptoms (muscle weakness/fatigue, muscle cramps/pain, and aching excluding pain during cramps) of PD patients were assessed via three standard un-graded 100-mm visual analog scales (VAS) separately at baseline, the 3rd month and the 6th month.The quality of life was measured by 36-item short-form health survey (SF-36) at baseline and the 6th month. Recombinant human erythropoietin (epoietin, rhuEPO) responsiveness, cardiac function and symptoms of heart failure were also monitored.
　　Forty-five patients with uremic myopathy were recruited into carnitine group (n=22) and control group (n=23). The 100mm VAS of muscle weakness/fatigue did not differ at entry. The carnitine group demonstrated an improvement as comparison to the control group (repeated measures ANOVA, overall P = 0.05). The 100-mm VAS of muscle cramps/pain of both was similar at baseline. After six months supplementation, carnitine group had lesser muscle cramps/pain than control group (overall P = 0.02). L-carnitine therapy also had positive effects on physical functioning, physical role, general health and emotional role concepts of SF-36 (P < 0.05). The epoietin responsiveness, ejection fraction of left ventricle and symptoms of heart failure did not have significant changes. No severe adverse effect was reported.
　　The oral low-dose supplementation of L-carnitine is well tolerated.L-carnitine therapy demonstrates significant alleviation of skeletal muscle symptoms and improves quality of life in chronic PD patients.

第一篇 慢性腹膜透析患者口服補充左旋肉質素之臨床效應
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