Tuchman M, Stoeckeler J S
Department of Pediatrics, University of Minnesota, Minneapolis 55455.
Pediatr Res. 1988 Jun;23(6):576-9. doi: 10.1203/00006450-198806000-00009.
Random urine samples were obtained from 31 patients with neuroblastoma (newborn to 8 yr of age) and from 100 children without this tumor (newborn to 10 yr). The urine samples were studied for the presence of sulfate and glucuronide conjugates of homovanillic (HVA), dihydroxyphenylacetic, vanilmandelic, and vanillactic acids. The urinary concentrations of these acids were determined by capillary gas-chromatography before and after enzymatic treatment with glucuronidase and sulfatase. Concentrations of the "free" fraction and "total" urinary content of these acids were determined using the results from untreated and treated urines respectively. Age-related reference values were established for children without neuroblastoma. Fractions of the total content of urinary HVA (18-39%) and dihydroxyphenylacetic acid (36-66%) were excreted as glucuronides and/or sulfates by the control group, with the highest conjugated fractions found in the urine of young infants (0-3 months). Vanilmandelic was excreted mainly as "free" acid (unconjugated), whereas vanillactic acid was undetectable in almost all control samples. Patients with neuroblastoma also excreted a fraction of these acids as glucuronide and/or sulfate conjugates, (25% of urinary HVA, 39% of dihydroxyphenylacetic acid and 45% of vanillactic acid) whereas vanilmandelic acid was excreted only as "free" in controls. Determination of "total" rather than "free" urinary HVA was diagnostic in one neuroblastoma patient with borderline "free" HVA levels, whereas determination of "free" or "total" dihydroxyphenylacetic acid and vanillactic acid did not improve the diagnostic sensitivity in the cases examined. We conclude that it may be clinically useful to determine "total" urinary HVA in patients with borderline "free" HVA levels who are suspected of having neuroblastoma.
从31例神经母细胞瘤患者（年龄从新生儿至8岁）和100名无此肿瘤的儿童（年龄从新生儿至10岁）中采集随机尿样。研究尿样中高香草酸（HVA）、二羟基苯乙酸、香草扁桃酸和香草乳酸的硫酸盐和葡糖醛酸共轭物。在用葡糖醛酸酶和硫酸酯酶进行酶处理前后，通过毛细管气相色谱法测定这些酸的尿浓度。分别使用未处理和处理后尿液的结果来确定这些酸的“游离”部分和尿液“总量”中的含量。为无神经母细胞瘤的儿童建立了与年龄相关的参考值。对照组中，尿HVA总量的18% - 39%和二羟基苯乙酸总量的36% - 66%以葡糖醛酸和/或硫酸盐形式排泄，在小婴儿（0 - 3个月）尿液中发现的共轭部分最高。香草扁桃酸主要以“游离”酸（未共轭）形式排泄，而在几乎所有对照样本中未检测到香草乳酸。神经母细胞瘤患者也将这些酸的一部分以葡糖醛酸和/或硫酸盐共轭物形式排泄（尿HVA的25%、二羟基苯乙酸的39%和香草乳酸的45%），而在对照中香草扁桃酸仅以“游离”形式排泄。在一名“游离”HVA水平处于临界值的神经母细胞瘤患者中，测定尿液“总量”而非“游离”HVA具有诊断价值，而在检查的病例中，测定“游离”或“总量”二羟基苯乙酸和香草乳酸并未提高诊断敏感性。我们得出结论，对于怀疑患有神经母细胞瘤且“游离”HVA水平处于临界值的患者，测定尿液“总量”HVA可能具有临床意义。
