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核心技术专利：CN118964589B侵权必究

核心技术专利：CN118964589B侵权必究

Paxton J W, Moore M R, Beattie A D, Goldberg A

Clin Sci Mol Med. 1975 Nov;49(5):441-4. doi: 10.1042/cs0490441.

Urinary 17-oxosteroid conjugates were measured by gas-liquid chromatography in five patients with hereditary coproporphyria. 2. Three patients were in an acute attack and showed significantly increased excretion of sulphate or glucuronide conjugates of aetiocholanolone. There was increased excretion of several other related steroids but no consistent pattern was apparent. 3. In the two patients in remission, excretion of urinary 17-oxosteroids was not increased. 4. The ratio of total urinary aetiocholanolone to androsterone (5beta:5alpha) was found to be significantly elevated for the three patients in an acute attack. Serial measurements were made in two of these patients and showed a highly significant linear correaltion between this ratio and the urinary content of delta-aminolaevulic acid and porphobilinogen. 5. These observations suggest the involvement of the 17-oxosteroids, espically aetiocholanolone, in the pathogenesis of hereditary coproporphyria.

采用气液色谱法对5例遗传性粪卟啉病患者的尿17 - 氧类固醇结合物进行了测定。2. 3例患者处于急性发作期，表现为本胆烷醇酮的硫酸盐或葡糖醛酸结合物排泄显著增加。其他几种相关类固醇的排泄也有所增加，但未呈现出一致的模式。3. 2例缓解期患者的尿17 - 氧类固醇排泄未增加。4. 发现3例急性发作期患者尿中总本胆烷醇酮与雄酮的比值（5β:5α）显著升高。对其中2例患者进行了连续测量，结果显示该比值与δ-氨基乙酰丙酸和卟啉胆色素原的尿含量之间存在高度显著的线性相关性。5. 这些观察结果提示17 - 氧类固醇，尤其是本胆烷醇酮，参与了遗传性粪卟啉病的发病机制。

Paxton J W, Moore M R, Beattie A D, Goldberg A

Clin Sci Mol Med. 1975 Nov;49(5):441-4. doi: 10.1042/cs0490441.

Urinary 17-oxosteroid conjugates were measured by gas-liquid chromatography in five patients with hereditary coproporphyria. 2. Three patients were in an acute attack and showed significantly increased excretion of sulphate or glucuronide conjugates of aetiocholanolone. There was increased excretion of several other related steroids but no consistent pattern was apparent. 3. In the two patients in remission, excretion of urinary 17-oxosteroids was not increased. 4. The ratio of total urinary aetiocholanolone to androsterone (5beta:5alpha) was found to be significantly elevated for the three patients in an acute attack. Serial measurements were made in two of these patients and showed a highly significant linear correaltion between this ratio and the urinary content of delta-aminolaevulic acid and porphobilinogen. 5. These observations suggest the involvement of the 17-oxosteroids, espically aetiocholanolone, in the pathogenesis of hereditary coproporphyria.

采用气液色谱法对5例遗传性粪卟啉病患者的尿17 - 氧类固醇结合物进行了测定。2. 3例患者处于急性发作期，表现为本胆烷醇酮的硫酸盐或葡糖醛酸结合物排泄显著增加。其他几种相关类固醇的排泄也有所增加，但未呈现出一致的模式。3. 2例缓解期患者的尿17 - 氧类固醇排泄未增加。4. 发现3例急性发作期患者尿中总本胆烷醇酮与雄酮的比值（5β:5α）显著升高。对其中2例患者进行了连续测量，结果显示该比值与δ-氨基乙酰丙酸和卟啉胆色素原的尿含量之间存在高度显著的线性相关性。5. 这些观察结果提示17 - 氧类固醇，尤其是本胆烷醇酮，参与了遗传性粪卟啉病的发病机制。