Trauner D, Sweetman L, Holm J, Kulovich S, Nyhan W L
Ann Neurol. 1977 Sep;2(3):238-41. doi: 10.1002/ana.410020310.
Eight patients with Reye's syndrome were followed through the course of their disease with serial measurements of serum concentrations of ammonia, short-chain organic acids, and amino acids. Correlations were made between clinical status and biochemical alterations. Elevated short-chain fatty acids, hyperammonemia, and hyperaminoacidemia were found in all patients studied. Clinical improvement appeared to correlate most closely with clearance of short-chain fatty acids from the serum. These observations suggest that the clinical symptoms are at least in part related to organic acidemia and that treatment directed toward the rapid clearance of these compounds from the system is reasonable.
对8例瑞氏综合征患者在疾病过程中进行了随访,连续测定血清氨、短链有机酸和氨基酸的浓度。对临床状况与生化改变之间进行了相关性分析。在所有研究患者中均发现短链脂肪酸升高、高氨血症和高氨基酸血症。临床改善似乎与血清中短链脂肪酸的清除最为密切相关。这些观察结果表明,临床症状至少部分与有机酸血症有关,并且针对这些化合物从体内快速清除的治疗是合理的。