ter Borg Pieter C J, Fekkes Durk, Vrolijk Jan Maarten, van Buuren Henk R
Department of Gastroenterology and Hepatology, Erasmus MC, Rotterdam, The Netherlands.
BMC Gastroenterol. 2005 Mar 24;5:11. doi: 10.1186/1471-230X-5-11.
In primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) fatigue is a major clinical problem. Abnormal amino acid (AA) patterns have been implicated in the development of fatigue in several non-hepatological conditions but for PBC and PSC no data are available. This study aimed to identify abnormalities in AA patterns and to define their relation with fatigue.
Plasma concentrations of tyrosine, tryptophan, phenylalanine, valine, leucine and isoleucine were determined in plasma of patients with PBC (n = 45), PSC (n = 27), chronic hepatitis C (n = 22) and healthy controls (n = 73). Fatigue and quality of life were quantified using the Fisk fatigue severity scale, a visual analogue scale and the SF-36.
Valine, isoleucine, leucine were significantly decreased in PBC and PSC. Tyrosine and phenylalanine were increased (p < 0.0002) and tryptophan decreased (p < 0.0001) in PBC. In PBC, but not in PSC, a significant inverse relation between tyrosine concentrations and fatigue and quality of life was found. Patients without fatigue and with good quality of life had increased tyrosine concentrations compared to fatigued patients. Multivariate analysis indicated that this relation was independent from disease activity or severity or presence of cirrhosis.
In patients with PBC and PSC, marked abnormalities in plasma AA patterns occur. Normal tyrosine concentrations, compared to increased concentrations, may be associated with fatigue and diminished quality of life.
在原发性胆汁性肝硬化(PBC)和原发性硬化性胆管炎(PSC)中,疲劳是一个主要的临床问题。在几种非肝脏疾病中,异常的氨基酸(AA)模式与疲劳的发生有关,但关于PBC和PSC的相关数据尚不可得。本研究旨在确定AA模式的异常情况,并明确其与疲劳的关系。
测定了PBC患者(n = 45)、PSC患者(n = 27)、慢性丙型肝炎患者(n = 22)和健康对照者(n = 73)血浆中酪氨酸、色氨酸、苯丙氨酸、缬氨酸、亮氨酸和异亮氨酸的浓度。使用菲斯克疲劳严重程度量表、视觉模拟量表和SF-36对疲劳和生活质量进行量化。
PBC和PSC患者的缬氨酸、异亮氨酸、亮氨酸显著降低。PBC患者的酪氨酸和苯丙氨酸升高(p < 0.0002),色氨酸降低(p < 0.0001)。在PBC患者中,而非PSC患者中,发现酪氨酸浓度与疲劳及生活质量之间存在显著的负相关。与疲劳患者相比,无疲劳且生活质量良好的患者酪氨酸浓度升高。多变量分析表明,这种关系独立于疾病活动度、严重程度或肝硬化的存在。
PBC和PSC患者血浆AA模式存在明显异常。与升高的浓度相比,正常的酪氨酸浓度可能与疲劳及生活质量下降有关。