Faraj B A, Newman S L, Caplan D B, Ali F M, Camp V M, Ahmann P A
Pediatrics. 1979 Jul;64(1):76-80.
Utilizing a specific and sensitive radioimmunoassay, palsma and urine tyramine were measured in 14 consecutive patients with liver biopsy-proven Reye's syndrome. Plasma tyrosine was measured in 11 of these patients. The results revealed significant (P less than .003) elevation in plasma (3.4 +/- .52 ng/ml) (mean +/- SEM) and urine (1.00 +/- .26 mg/24 hr) tyramine as well as plasma tyrosine (204 +/- 52.5 mumole/liter) at the onset of the disease when compared to the levels of tyramine and tyrosine in a group of hospitalized patients without hepatic disorders. Furthermore, there was a positive correlation between plasma tyramine and days in coma (r = .86; P less than .001), and between plasma tyramine and tyrosine (r = 0.80; P less than .001). These data suggest that there is s substantial disturbance of tyrosine metabolism in Reye's syndrome and that the accumulation of this amino acid and its metabolite, tyramine, may contribute to the encephalopathy of this disease.
利用一种特异且灵敏的放射免疫分析法,对14例经肝活检证实为瑞氏综合征的连续患者测定了血浆和尿中的酪胺。其中11例患者测定了血浆酪氨酸。结果显示,与一组无肝脏疾病的住院患者的酪胺和酪氨酸水平相比,在疾病发作时,血浆(3.4±0.52纳克/毫升)(均值±标准误)和尿(1.00±0.26毫克/24小时)中的酪胺以及血浆酪氨酸(204±52.5微摩尔/升)显著升高(P<0.003)。此外,血浆酪胺与昏迷天数之间存在正相关(r = 0.86;P<0.001),血浆酪胺与酪氨酸之间也存在正相关(r = 0.80;P<0.001)。这些数据表明,瑞氏综合征中酪氨酸代谢存在严重紊乱,这种氨基酸及其代谢产物酪胺的积累可能导致该疾病的脑病。