SERUM DICARBOXYLIC-ACIDS IN PATIENTS WITH REYE SYNDROME
SERUM DICARBOXYLIC-ACIDS IN PATIENTS WITH REYE SYNDROME
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DOI：
10.1016/s0022-3476(86)80114-7
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发表时间：
1986-09-01
影响因子：
5.1
通讯作者：
TONSGARD, JH
中科院分区：
文献类型：
--
作者：
TONSGARD, JH
Reye综合征类似于脂肪酸代谢紊乱。对18名Reye综合征患者的血清游离脂肪酸的分析显示，二羧酸占患者总游离脂肪酸的55%（范围4%至55%）;在任何对照样品中均未发现中链（6至12个碳长度）和长链（14至18个碳长度）二羧酸，而86%。10例Reye综合征昏迷3 ~ 4级患者血清二羧酸中长链占4%，长链占31%。8%的患者病情较轻。血清二羧酸浓度与临床状态相关（P < 0.001），与血氨浓度升高相关（r ~ 2 = 0.8767）。尿液中未发现长链二羧酸。Reye综合征中的二羧酸血症可能继发于一般线粒体功能障碍，或者可能表明脂肪酸代谢受损或ω-氧化刺激在疾病的发病机制中很重要。测量血清二羧酸，特别是长链，在评估Reye综合征中可能是重要的，并可能被证明有助于将其与其他疾病区分开来。
Reye syndrome resembles disorders of fatty acid metabolism. Analysis of serum free fatty acids from 18 patients with Reye syndrome revealed that dicarboxylic acids comprise as much as 55% (range 4% to 55%) of the patients'' total free fatty acids; both medium- (6 to 12 carbon lengths) and long-chain (14 to 18 carbon lengths) dicarboxylic acids were not found in any control samples, whereas 86% .+-. 4% of the serum dicarboxylic acids were long chain in 10 patients with Reye syndrome in state 3 to 4 coma and 31% .+-. 8% in eight patients with a milder illness. The serum concentration of dicarboxylic acids correlated with the clinical state (P < 0.001) and with the elevation in blood ammonia concentration (r2 = 0.8767). No long-chain dicarboxylic acids were found in the urine. The dicarboxylic acidemia in Reye syndrome may be secondary to the general mitochondrial dysfunction or could indicate that an insult to fatty acid metabolism or the stimulation of omega-oxidation is important in the pathogenesis of the illness. Measurement of serum dicarboxylic acids, especially long chain, may be important in assessing Reye syndrome and may prove useful in distinguishing this from other diseases.